Dermatology

Question 1

Aplasia cutis congenita

• Congenital absence of skin (epidermis, dermis, and occasionally subcutaneous tissue) most commonly presenting as a solitary, vertex scalp lesion without hair.
• If it occurs in multiple places on the scalp, look for trisomy 13.
• Midline scalp lesions that are encircled by thicker, darker hair (“hair collar sign”) suggest cranial dysraphism. MRI is needed in this situation to rule out underlying pathology.

Answer 1

Aplasia cutis congenita

• Congenital absence of skin (epidermis, dermis, and occasionally subcutaneous tissue) most commonly presenting as a solitary, vertex scalp lesion without hair.
• If it occurs in multiple places on the scalp, look for trisomy 13.
• Midline scalp lesions that are encircled by thicker, darker hair (“hair collar sign”) suggest cranial dysraphism. MRI is needed in this situation to rule out underlying pathology.

Question 2

Sebaceous Hyperplasia
- Pt: Multiple pinpoint white-to-yellow papules, usually on or around the nose and upper lip. These lesions are more yellow than milia

Answer 2

Sebaceous Hyperplasia
- Pt: Multiple pinpoint white-to-yellow papules, usually on or around the nose and upper lip. These lesions are more yellow than milia

Question 3

Erythema toxicum neonatorum

• Multiple evanescent blotchy erythematous macules and overlying 1-3mm white-yellow papules
• Dx: A biopsy would show numerous _____.

Answer 3

Erythema toxicum neonatorum

• Multiple evanescent blotchy erythematous macules and overlying 1-3mm white-yellow papules
• Dx: Eosinophils. A biopsy would show numerous eosinophils.

Question 4

Transient Neonatal Pustular Melanosis (TNPM)

• Vesicle → pustule ruptures → scaly hyperpigmented macules (often surrounded by rim or collarette of scale).
• Contrasts with ETN since lesions lack surrounding erythema
• Dx: Clinical. Tzanck / Wright stain will reveal largely ______.

Answer 4

Transient Neonatal Pustular Melanosis (TNPM)

• Vesicle → pustule ruptures → scaly hyperpigmented macules (often surrounded by rim or collarette of scale).
• Contrasts with ETN since lesions lack surrounding erythema
• Dx: Clinical. Tzanck / Wright stain will reveal largely neutrophils.

Question 5

Neonatal Acne / Neonatal Cephalic Pustulosis

• Generally apparent within the first 2-4 weeks of life
• Treatment: Daily cleansing with soap/water, avoid oils/lotions. Resolves within the first 1-2 months of life without scarring.

Answer 5

Neonatal Acne / Neonatal Cephalic Pustulosis

• Generally apparent within the first 2-4 weeks of life
• Treatment: Daily cleansing with soap/water, avoid oils/lotions. Resolves within the first 1-2 months of life without scarring.

Question 6

Infantile Acne

• Pt: Typically presents around 2-4 months.
• Tx: Usually resolves over 6-12 months.
• Because it is potentially persistent and severe cases cause scarring, tx with topical medications (retinoids, benzoyl peroxide, antibiotics) is helpful.
• In severe or recalcitrant cases, oral erythromycin is an option

Answer 6

Infantile Acne

• Pt: Typically presents around 2-4 months.
• Tx: Usually resolves over 6-12 months.
• Because it is potentially persistent and severe cases cause scarring, tx with topical medications (retinoids, benzoyl peroxide, antibiotics) is helpful.
• In severe or recalcitrant cases, oral erythromycin is an option

Question 7

Infantile Hemangioma

• PROLIFERATION AGE 0-1. Bright red, raised nodule
• Involution: age 1-9. Deeper red/violet, regression in size.
  
  • 50% will involute without treatment by 5yo, 70% by 7yo.
• Tx:
  
  • Indications for treatment of a hemangioma include the following:
    
    • 1) Location interferes with physiologic functioning
    • 2) Recurrent bleeding or ulceration
    • 3) High output cardiac failure
    • 4) Kasabach-Merritt syndrome
    • 5) Rapidly growing or disfiguring
  • Pediatric Dermatology or IH specialist
    
    • Periorbital
    • Ear
    • Nasal tip
    • Lip
    • Beard area
    • Large, segmental facial hemangiomas occur with _____ syndrome:
      
      • P___
      • H___
      • A___
      • C___
      • E____
      • S___/S___
    • Midline lumbosacral: Increased risk of underlying spinal dysraphism
    • Multiple cutaneous hemangiomas ( >5): increased risk of internal hemangiomas, chiefly of the liver and GI tract.
    • Kasabach-Merritt syndrome is characterized by ___ and ______.
  • Topical ___ BID for 7-10 days
  • ____ is the most effective agent
    
    • Adverse effects of B-blocking drugs include hypotension, bradycardia, conduction delays, bronchospasm, and hypoglycemia.

Answer 7

Infantile Hemangioma

• PROLIFERATION AGE 0-1. Bright red, raised nodule
• Involution: age 1-9. Deeper red/violet, regression in size.
  
  • 50% will involute without treatment by 5yo, 70% by 7yo.
• Tx:
  
  • Indications for treatment of a hemangioma include the following:
    
    • 1) Location interferes with physiologic functioning
    • 2) Recurrent bleeding or ulceration
    • 3) High output cardiac failure
    • 4) Kasabach-Merritt syndrome
    • 5) Rapidly growing or disfiguring
  • Pediatric Dermatology or IH specialist
    
    • Periorbital
    • Ear
    • Nasal tip
    • Lip
    • Beard area
    • Large, segmental facial hemangiomas occur with PHACE(S) syndrome:
      
      • Posterior fossa abnormalities (Dandy-Walker syndrome, cerebellar hypoplasia or dysplasia)
      • Hemangioma
      • Arterial anomalies (typically, intracerebral arterial anomalies)
      • Cardiac defects, esp coarctation of the aorta
      • Eye abnormalities (variable: microphthalmia, may involve either posterior or anterior segments)
      • Sternal defects/Supraumbilical raphe
    • Midline lumbosacral: Increased risk of underlying spinal dysraphism
    • Multiple cutaneous hemangiomas ( >5): increased risk of internal hemangiomas, chiefly of the liver and GI tract.
    • Kasabach-Merritt syndrome is characterized by giant hemangiomas and severe thrombocytopenia.
  • Topical timolol BID for 7-10 days
  • Beta blockers/ propranolol is the most effective agent
    
    • Adverse effects of B-blocking drugs include hypotension, bradycardia, conduction delays, bronchospasm, and hypoglycemia.

Question 8

Port-wine stain (PWS) (Nevus flammeus)

• Pt: They appear as flat pink to dark red patches that are present at birth and may become lighten, darken, or thicken over time.
  
  • Unlike hemangiomas, they do not proliferate.
• When present on the face, PWS raises concern for Sturge-Weber syndrome and may be disfiguring.
  
  • Occasionally it may be associated with underlying AV malformations and bone and soft tissue overgrowth (Parkes Weber syndrome) or venous varicosities accompanied by bone and soft tissue hypertrophy (Klippel-Trenaunay syndrome).
    
    • _______ - presents with vascular malformations (often mixed capillary-venous-lymphatic derivation) of an extremity and soft tissue and/or limb overgrowth.
      
      • Port-wine stains of the LEs are seen in Klippel-Trenaunay syndrome (KTS).
      • Triad of _____, ____, and _____
    • Parkes Weber syndrome - features AV malformations that have a palpable thrill and can be visualized with US
• Management
  
  • Infants who have facial PWS (concerning for possible Sturge-weber syndrome) should undergo ophthalmologic consultation to assess intraocular pressure and radiologic imaging of the head.

Answer 8

Port-wine stain (PWS) (Nevus flammeus)

• Pt: They appear as flat pink to dark red patches that are present at birth and may become lighten, darken, or thicken over time.
  
  • Unlike hemangiomas, they do not proliferate.
• When present on the face, PWS raises concern for Sturge-Weber syndrome and may be disfiguring.
  
  • Occasionally it may be associated with underlying AV malformations and bone and soft tissue overgrowth (Parkes Weber syndrome) or venous varicosities accompanied by bone and soft tissue hypertrophy (Klippel-Trenaunay syndrome).
    
    • Klippel-Trenaunay syndrome (KTS) - presents with vascular malformations (often mixed capillary-venous-lymphatic derivation) of an extremity and soft tissue and/or limb overgrowth.
      
      • Port-wine stains of the LEs are seen in Klippel-Trenaunay syndrome (KTS).
      • Triad of cutaneous capillary malformation, lymphatic anomalies, and abnormal veins
    • Parkes Weber syndrome - features AV malformations that have a palpable thrill and can be visualized with US
• Management
  
  • Infants who have facial PWS (concerning for possible Sturge-weber syndrome) should undergo ophthalmologic consultation to assess intraocular pressure and radiologic imaging of the head.

Question 9

Nevus Sebaceus

• Yellow-pink, hairless plaque that often has a _______ texture.
• Through early childhood, the lesions often appear flat or as thin plaques; however, during puberty, the nevus sebaceous becomes thicker
• Biopsy of the lesion during the latter stage often shows an over-abundance of sebaceous glands with few or absent ______.
• Tx:
  
  • If there are no cosmetic concerns or growths that arise within nevus sebaceus, it is acceptable to simply monitor it.
  • Otherwise, definitive tx consists of full-thickness excision.

Answer 9

Nevus Sebaceus

• Yellow-pink, hairless plaque that often has a waxy or velvety texture.
• Through early childhood, the lesions often appear flat or as thin plaques; however, during puberty, the nevus sebaceous becomes thicker
• Biopsy of the lesion during the latter stage often shows an over-abundance of sebaceous glands with few or absent hair follicles.
• Tx:
  
  • If there are no cosmetic concerns or growths that arise within nevus sebaceus, it is acceptable to simply monitor it.
  • Otherwise, definitive tx consists of full-thickness excision.

Question 10

Nevus Spilus (speckled lentiginous nevus)
- Characteristically, the patches develop multiple, small dark macules and/or papules within and can resemble a \_\_\_\_\_\_

Answer 10

Nevus Spilus (speckled lentiginous nevus)
- Characteristically, the patches develop multiple, small dark macules and/or papules within and can resemble a chocolate chip cookie.

Question 11

Congenital Melanocytic Nevi

• Pt:
  
  • On exam, look for key words like “coat sleeve,” “stocking,” capelike,” “bathing trunk,” or “garment-type” bc these are often used to describe large congenital nevi.
  • Increased hair growth, slightly irregular borders, and uneven pigmentation are possible; thus observation for clinical changes is warranted.
• Neurocutaneous melanosis - Large lesions located over the scalp, midline neck, or spine can indicate melanocytes within the meninges or CNS.

Answer 11

Congenital Melanocytic Nevi

• Pt:
  
  • On exam, look for key words like “coat sleeve,” “stocking,” capelike,” “bathing trunk,” or “garment-type” bc these are often used to describe large congenital nevi.
  • Increased hair growth, slightly irregular borders, and uneven pigmentation are possible; thus observation for clinical changes is warranted.
• Neurocutaneous melanosis - Large lesions located over the scalp, midline neck, or spine can indicate melanocytes within the meninges or CNS.

Question 12

Nevus of Ota (Oculodermal melanocytosis)

• Pt:
  
  • Clinically, nevus of Ota is characterized by unilateral (rarely bilateral) blue-gray macules and patches on the face
    
    • Unlike Mongolian spots, nevus of Ota does not resolve with time.
    • Rarely, cutaneous and ocular melanoma can occur within these lesions; thus periodic ophthalmologic and skin exams are recommended.

Answer 12

Nevus of Ota (Oculodermal melanocytosis)

• Pt:
  
  • Clinically, nevus of Ota is characterized by unilateral (rarely bilateral) blue-gray macules and patches on the face
    
    • Unlike Mongolian spots, nevus of Ota does not resolve with time.
    • Rarely, cutaneous and ocular melanoma can occur within these lesions; thus periodic ophthalmologic and skin exams are recommended.

Question 13

Cafe-au-lait Macules (CALMs)

• Pt:
  
  • Presence of 1 or 2 lesions is normal, but large or many lesions suggest a neurocutaneous syndrome, such as in NF1 (See “neurocutaneous disorder”)
    
    • Among the major diagnostic criteria of NF1 are >____ cafe au lait spots (must be >___mm prepubertal and >___ mm postpubertal) and Crowe sign (small grouped freckle-like, cafe au lait macules 1-4mm clustered in the axilla or groin)

Answer 13

Cafe-au-lait Macules (CALMs)

• Pt:
  
  • Presence of 1 or 2 lesions is normal, but large or many lesions suggest a neurocutaneous syndrome, such as in NF1 (See “neurocutaneous disorder”)
    
    • Among the major diagnostic criteria of NF1 are >6 cafe au lait spots (must be >5mm prepubertal and >15 mm postpubertal) and Crowe sign (small grouped freckle-like, cafe au lait macules 1-4mm clustered in the axilla or groin)

Question 14

Dermoid cyst

• Pt: Congenital, skin-colored subcutaneous nodule (1-4cm) that is firm to palpation and located on the lateral forehead
  
  • Most often occur near the eyes, especially the lateral eyebrow
    
    • A dermal sinus tract may also occur. This appears as a pit, sometimes with a tuft of protruding hair
      
      • Sinuses involving the midline craniospinal axis, including the nasal bridge, occiput, and midline back, raise concern for connection with the underlying CNS and risk of meningitis.
      • CNS extension is more commonly seen with midline scalp or nasal lesions or when associated with a cutaneous dimple or sinus.
• Management:
  
  • All midline dermoid cysts or sinuses (glabella, occipital scalp, midline back) should undergo imaging (typically MRI but occasionally CT) to evaluate for possible intracranial extension.
  • Lesions located near the eyebrow or on the lateral forehead do not require imaging before excision.

Answer 14

Dermoid cyst

• Pt: Congenital, skin-colored subcutaneous nodule (1-4cm) that is firm to palpation and located on the lateral forehead
  
  • Most often occur near the eyes, especially the lateral eyebrow
    
    • A dermal sinus tract may also occur. This appears as a pit, sometimes with a tuft of protruding hair
      
      • Sinuses involving the midline craniospinal axis, including the nasal bridge, occiput, and midline back, raise concern for connection with the underlying CNS and risk of meningitis.
      • CNS extension is more commonly seen with midline scalp or nasal lesions or when associated with a cutaneous dimple or sinus.
• Management:
  
  • All midline dermoid cysts or sinuses (glabella, occipital scalp, midline back) should undergo imaging (typically MRI but occasionally CT) to evaluate for possible intracranial extension.
  • Lesions located near the eyebrow or on the lateral forehead do not require imaging before excision.

Question 15

Ichthyoses = Disorders of cornification. Abnormal keratinization.
Ichthyosis Vulgaris
- Most common ichthyosis.

• Path: Semi AD inheritance or recessive loss-of-function mutations in the ______ gene
• Pt:
  
  • Scaling is most prominent in the extensor surfaces of the extremities.
  • Affected persons have accentuated skin markings on the palms and soles / prominent creases (hyperlinearity)
• Tx:
  
  • ______

Answer 15

Ichthyoses = Disorders of cornification. Abnormal keratinization.
Ichthyosis Vulgaris
- Most common ichthyosis.

• Path: Semi AD inheritance or recessive loss-of-function mutations in the filaggrin gene
• Pt:
  
  • Scaling is most prominent in the extensor surfaces of the extremities.
  • Affected persons have accentuated skin markings on the palms and soles / prominent creases (hyperlinearity)
• Tx:
  
  • Direct at improving skin hydration through emollients containing alpha-hydroxy acid or urea

Question 16

X-Linked Recessive Ichthyosis

• Affected boys can have _______, with an associated increased risk of testicular cancer.
• If diagnosis suspected, pts need a thorough GU exam.

Answer 16

X-Linked Recessive Ichthyosis

• Affected boys can have cryptorchidism, with an associated increased risk of testicular cancer.
• If diagnosis suspected, pts need a thorough GU exam.

Question 17

Lamellar Ichthyosis and Congenital (nonbullous) Ichthyosiform Erythroderma
- Classically present as the “_________” Affected newborns appear with a taut, shiny covering over their body called a collodion membrane (similar to cellophane).

Answer 17

Lamellar Ichthyosis and Congenital (nonbullous) Ichthyosiform Erythroderma
- Classically present as the “collodion baby.” Affected newborns appear with a taut, shiny covering over their body called a collodion membrane (similar to cellophane).

Question 18

Gorlin Syndrome (basal cell nevus syndrome)
- \_\_\_\_\_ disorder

• Path: Mutations of the “patched” gene, a tumor suppressor that controls cell growth and patterning
• Children develop numerous _________ in childhood.
• Because this is a defect in tumor suppression, these pts are at risk of developing other tumors, including ovarian and cardiac fibromas, meningiomas, and medulloblastomas
• Pts benefit from vismodegib, which is a targeted therapy that inhibits hedgehog pathway

Answer 18

Gorlin Syndrome (basal cell nevus syndrome)
- AD disorder

• Path: Mutations of the “patched” gene, a tumor suppressor that controls cell growth and patterning
• Children develop numerous basal cell carcinomas in childhood.
• Because this is a defect in tumor suppression, these pts are at risk of developing other tumors, including ovarian and cardiac fibromas, meningiomas, and medulloblastomas
• Pts benefit from vismodegib, which is a targeted therapy that inhibits hedgehog pathway

Question 19

Progeria (Hutchinson-Gilform Syndrome)

- Rare, sporadic AR disorder characterized by premature aging.

Answer 19

Progeria (Hutchinson-Gilform Syndrome)

- Rare, sporadic AR disorder characterized by premature aging.

Question 20

Ectodermal dysplasia (ED)

• Most are X-linked, but there are AD and AR cases
• Heterogenous group of disorders characterized by problems with 2 or more of the following: teeth, skin, and appendageal structures (eg hair, nails, eccrine, and sebaceous glands)
• 3 main types:
  
  • 1. Hypohidrotic ED (HED) (aka Christ-Siemens-Touraine syndrome)
       - Characterized by hypohidrosis or anhidrosis, hypotrichosis, and hypodontia
       - Key features:
       
       • Characteristic facial features: Prominent frontal bone; periorbital hyperpigmentation; retruded (moved backward) midface
       • Hypotrichosis: Sparse, lightly pigmented, slow-growing scalp and body hair that also has increased fragility
       • Hypohidrosis: Reduced ability to sweat, which may lead to episodes of hyperthermia
       • Hypodontia: Teeth are reduced in number and often are smaller than expected and abnormally shaped (ie the anterior teeth often have a conical shape)
       • Eczema
         - Management:
       • Gentle hair care practices are encouraged to avoid breaking fragile hairs and wigs may be appropriate for some individuals
       • Evaluation by an experienced dentist
  • 2. Hypohidrotic ED with immunodeficiency (HED-IE)
       - These individuals have similar clinical findings to HED, but they have dysgammaglobulinemia and an increased risk of bronchiectasis, pneumonia, skin infections, osteomyelitis, and meningitis.
  • 3. Hidrotic ED (HED2) (aka Clouston syndrome)
       - Hyperkeratosis of the palms and soles, nail dystrophy, and abnormal hair. These pts are able to sweat normally and usually have normal teeth.

Answer 20

Ectodermal dysplasia (ED)

• Most are X-linked, but there are AD and AR cases
• Heterogenous group of disorders characterized by problems with 2 or more of the following: teeth, skin, and appendageal structures (eg hair, nails, eccrine, and sebaceous glands)
• 3 main types:
  
  • 1. Hypohidrotic ED (HED) (aka Christ-Siemens-Touraine syndrome)
       - Characterized by hypohidrosis or anhidrosis, hypotrichosis, and hypodontia
       - Key features:
       
       • Characteristic facial features: Prominent frontal bone; periorbital hyperpigmentation; retruded (moved backward) midface
       • Hypotrichosis: Sparse, lightly pigmented, slow-growing scalp and body hair that also has increased fragility
       • Hypohidrosis: Reduced ability to sweat, which may lead to episodes of hyperthermia
       • Hypodontia: Teeth are reduced in number and often are smaller than expected and abnormally shaped (ie the anterior teeth often have a conical shape)
       • Eczema
         - Management:
       • Gentle hair care practices are encouraged to avoid breaking fragile hairs and wigs may be appropriate for some individuals
       • Evaluation by an experienced dentist
  • 2. Hypohidrotic ED with immunodeficiency (HED-IE)
       - These individuals have similar clinical findings to HED, but they have dysgammaglobulinemia and an increased risk of bronchiectasis, pneumonia, skin infections, osteomyelitis, and meningitis.
  • 3. Hidrotic ED (HED2) (aka Clouston syndrome)
       - Hyperkeratosis of the palms and soles, nail dystrophy, and abnormal hair. These pts are able to sweat normally and usually have normal teeth.

Question 21

Epstein pearls

• Milia of the ____ in newborns
• Pt: Small, benign, whitish-yellow papules
• Tx: Spontaneously resolve without treatment

Answer 21

Epstein pearls

• Milia of the hard palate in newborns
• Pt: Small, benign, whitish-yellow papules
• Tx: Spontaneously resolve without treatment

Question 22

Bohn Nodules

• Benign intraoral inclusion cysts that are often observed on the alveolar ridges of newborns
• Frequently mistaken as neonatal teeth
• Generally resolve spontaneously over the first several months of life; harmless

Answer 22

Bohn Nodules

• Benign intraoral inclusion cysts that are often observed on the alveolar ridges of newborns
• Frequently mistaken as neonatal teeth
• Generally resolve spontaneously over the first several months of life; harmless

Question 23

Ranula

• Benign mass originating in the floor of the mouth; it is a sublingual dilatation of a salivary gland. It is characterized by a translucent-to-bluish, nonblanching, fluctuant swelling lateral to the midline of the lower mouth.
• Tx: Excision of the mass and the severed duct exteriorized

Answer 23

Ranula

• Benign mass originating in the floor of the mouth; it is a sublingual dilatation of a salivary gland. It is characterized by a translucent-to-bluish, nonblanching, fluctuant swelling lateral to the midline of the lower mouth.
• Tx: Excision of the mass and the severed duct exteriorized

Question 24

Natal teeth

- In general, if anything more than a simple pinch is required to remove the tooth, it should not be removed.

Answer 24

Natal teeth

- In general, if anything more than a simple pinch is required to remove the tooth, it should not be removed.

Question 25

Oropharyngeal Candidiasis / Thrush

• One of the most common presentations of Candida infection in infants <5mo. After 5mo, it is seen in children who are receiving antibiotics, are immunocompromised (think about AIDS or SCID), or are debilitated and malnourished.
  
  • Oral candidiasis in children >6 months is usually the result of an underlying RF
• RFs:
  
  • Primary or acquired immunodeficiencies such as HIV
  • Neutropenia or neutrophil functional defects
  • Extreme prematurity
  • Indwelling catheters
  • Medications that increase the risk for candidiasis include broad-spectrum antibiotics, inhaled steroid use, systemic corticosteroids, immunosuppressive medications, and cytotoxic chemotherapy.
  • Diabetes
  • Radiation
• Tx:
  
  • Use topical ______ in the immunocompetent and oral fluconazole in the immunocompromised pt.
  • For breastfeeding mother: _____ 150mg 1 tab (preferred) or nystatin cream onto nipples (place after feeds to keep on nipples)
• Ppx: Rinsing water after steroid inhalation

Answer 25

Oropharyngeal Candidiasis / Thrush

• One of the most common presentations of Candida infection in infants <5mo. After 5mo, it is seen in children who are receiving antibiotics, are immunocompromised (think about AIDS or SCID), or are debilitated and malnourished.
  
  • Oral candidiasis in children >6 months is usually the result of an underlying RF
• RFs:
  
  • Primary or acquired immunodeficiencies such as HIV
  • Neutropenia or neutrophil functional defects
  • Extreme prematurity
  • Indwelling catheters
  • Medications that increase the risk for candidiasis include broad-spectrum antibiotics, inhaled steroid use, systemic corticosteroids, immunosuppressive medications, and cytotoxic chemotherapy.
  • Diabetes
  • Radiation
• Tx:
  
  • Use topical nystatin or clotrimazole in the immunocompetent and oral fluconazole in the immunocompromised pt.
  • For breastfeeding mother: fluconazole 150mg 1 tab (preferred) or nystatin cream onto nipples (place after feeds to keep on nipples)
• Ppx: Rinsing water after steroid inhalation

Question 26

Acquired Melanocytic Nevi (Pigmented moles)

• Tx:
  
  • Excision/biopsy of acquired melanocytic nevi if they:
  • Become painful
  • Become pruritic
  • Ulcerate
  • Change significantly in size
  • Change in color
  • Change in shape
  • Are prone to trauma

Answer 26

Acquired Melanocytic Nevi (Pigmented moles)

• Tx:
  
  • Excision/biopsy of acquired melanocytic nevi if they:
  • Become painful
  • Become pruritic
  • Ulcerate
  • Change significantly in size
  • Change in color
  • Change in shape
  • Are prone to trauma

Question 27

Lichen Sclerosus

• Thought to be autoimmune process
• Pt:
  
  • Epidermal atrophy and inflammation with pain and pruritus.
  • Can occur anywhere on the body but classically involves skin of the vulva, perineum, and glans penis.
• Exam reveals white atrophic patches and plaques, often forming an hourglass configuration around the vulva and perianal skin.
• Tx: Potent topical corticosteroids are usually the 1st step of management.
  
  • Condition can be self-limited or progressive, with possible obliteration of anatomy and a small increased risk for squamous cell carcinoma of involved skin.

Answer 27

Lichen Sclerosus

• Thought to be autoimmune process
• Pt:
  
  • Epidermal atrophy and inflammation with pain and pruritus.
  • Can occur anywhere on the body but classically involves skin of the vulva, perineum, and glans penis.
• Exam reveals white atrophic patches and plaques, often forming an hourglass configuration around the vulva and perianal skin.
• Tx: Potent topical corticosteroids are usually the 1st step of management.
  
  • Condition can be self-limited or progressive, with possible obliteration of anatomy and a small increased risk for squamous cell carcinoma of involved skin.

Question 28

Acanthosis Nigricans
- Screening for diabetes, hyperlipidemia, and HTN must be considered, depending on the history and physical exam. Up to 30% of pts with PCOS have acanthosis nigricans. In adults, it can be associated with GI cancer.

• Tx underlying cause. For those who wanted accelerated improvement of skin lesions, tx with topical retinoids or topical vitamin D analog.

Answer 28

Acanthosis Nigricans
- Screening for diabetes, hyperlipidemia, and HTN must be considered, depending on the history and physical exam. Up to 30% of pts with PCOS have acanthosis nigricans. In adults, it can be associated with GI cancer.

• Tx underlying cause. For those who wanted accelerated improvement of skin lesions, tx with topical retinoids or topical vitamin D analog.

Question 29

Pityriasis Alba

• Form of postinflammatory hypopigmentation at sites of dry skin or mild eczema that often occurs in children who have a hx of atopic dermatitis.
• Poorly circumcised Hypopigmented macules that may have associated scale. Borders of lesions are indistinct, with a gradual transition from normal to abnormal pigmentation.
• Tx: _____. Some clinicians tx w short course of low-potency topical corticosteroid (hydrocortisone 1% or 2.5%) for 7 days. Regardless of the tx selected, the pt and family should be counseled that the return of normal pigmentation takes months.

Answer 29

Pityriasis Alba

• Form of postinflammatory hypopigmentation at sites of dry skin or mild eczema that often occurs in children who have a hx of atopic dermatitis.
• Poorly circumcised Hypopigmented macules that may have associated scale. Borders of lesions are indistinct, with a gradual transition from normal to abnormal pigmentation.
• Tx: Emollient. Some clinicians tx w short course of low-potency topical corticosteroid (hydrocortisone 1% or 2.5%) for 7 days. Regardless of the tx selected, the pt and family should be counseled that the return of normal pigmentation takes months.

Question 30

Tinea versicolor (pityriasis versicolor)

• Common superficial infection with yeasts of genus _______
• Pt: Hypopigmented, pink, or hyperpigmented scaly macules/patches, borders are well defined; scale may be present.
• Dx: Usually clinically. If uncertainty exists, a _____ preparation performed on scale from a lesion will demonstrate short hyphae and spores “________” and a Wood lamp exam will reveal ______ fluorescence of the affected areas.
• Tx:
  
  • 1st line tx usually with topical agent:
    
    • ______ lotion
    • Topical azole: ______ shampoo
  • 2nd line treatments might include oral agents
    
    • Options include itraconazole (400mg once or 200mg/day for 7 days) and fluconazole (400mg once).

Answer 30

Tinea versicolor (pityriasis versicolor)

• Common superficial infection with yeasts of genus Malassezia
• Pt: Hypopigmented, pink, or hyperpigmented scaly macules/patches, borders are well defined; scale may be present.
• Dx: Usually clinically. If uncertainty exists, a potassium hydroxide preparation performed on scale from a lesion will demonstrate short hyphae and spores “spaghetti and meatballs,” and a Wood lamp exam will reveal yellow-gold fluorescence of the affected areas.
• Tx:
  
  • 1st line tx usually with topical agent:
    
    • Selenium sulfide lotion
    • Topical azole: Ketoconazole shampoo
  • 2nd line treatments might include oral agents
    
    • Options include itraconazole (400mg once or 200mg/day for 7 days) and fluconazole (400mg once).

Question 31

Vitiligo

• Associated with other autoimmune conditions including type 1 diabetes, scleroderma, rheumatoid arthritis, Addison disease, alopecia areata, pernicious anemia, SLE. One of the most common associations is Hashimoto thyroiditis.
  
  • It would be appropriate to check a _____ level in a pt with vitiligo.
• Pt:
  
  • Vitiligo can appear in sites of skin trauma (Koebner phenomenon).
• Tx:
  
  • No known cure for vitiligo.
  • Makeup (foundation, coverup) is often recommended for mild disease or for those who have lighter skin.
  • ____ is a backbone of vitiligo therapy.
  • 1st line tx usually is with a topical ____ or a topical _____ inhibitor.
  • Other options include narrowband ultraviolet B phototherapy, photochemotherapy using psoralen and ultraviolet A, and excimer laser.

Answer 31

Vitiligo

• Associated with other autoimmune conditions including type 1 diabetes, scleroderma, rheumatoid arthritis, Addison disease, alopecia areata, pernicious anemia, SLE. One of the most common associations is Hashimoto thyroiditis.
  
  • It would be appropriate to check a TSH level in a pt with vitiligo.
• Pt:
  
  • Vitiligo can appear in sites of skin trauma (Koebner phenomenon).
• Tx:
  
  • No known cure for vitiligo.
  • Makeup (foundation, coverup) is often recommended for mild disease or for those who have lighter skin.
  • Sunscreen is a backbone of vitiligo therapy.
  • 1st line tx usually is with a topical corticosteroid or a topical calcineurin inhibitor.
  • Other options include narrowband ultraviolet B phototherapy, photochemotherapy using psoralen and ultraviolet A, and excimer laser.

Question 32

ATOPIC DERMATITIS / ECZEMA
- While the exact mechanisms are not fully understood, up to 50% of pts with severe atopic dermatitis have a genetic mutation in the _____ gene (FLG) family

• Pt:
  
  • Pruritus is hallmark “the itch that rashes.”
  • In infants (esp <4yo), atopic dermatitis most commonly presents on the cheeks and extensor surfaces of the extremities
  • Older children who are able to scratch more areas usually have disease in the antecubital fossae, popliteal fossae, and flexor aspect of the neck.
  • Other clues to diagnosis:
    
    • Double or triple creases under the lower eyelid (Dennie Morgan lines is associated)
    • Obvious sparing around the nose (“headlight sign”)
    • Small fissures at the base of the ear lobe
    • Increased skin markings on palms and soles
    • Dry skin (xerosis)
• Risks
  
  • Staph aureus colonization is common (as many as 25% are colonized).
  • Children with atopic dermatitis are also at increased risk of disseminated skin infection with molluscum contagiosum and herpes simplex (eczema herpeticum)
• Remember: The live smallpox vaccine is NOT given to those who have atopic dermatitis or those who are immunodeficient.
• Ppx:
  
  • Research studies have shown that breastfeeding for at least 4 months (in comparison to infants fed cow’s milk formula) prevents or delays the occurrence of atopic dermatitis.
• Tx:
  
  • Avoid heat and low humidity, keep skin hydrated with creams or ointments (not lotions). Recommend gentle skin care products, unscented soaps, baby detergent when wash clothes
  • Avoidance of factors that contribute to itching, such as excessively hot or dry environments and harsh soaps and detergents
  • 1st LEVEL: The most important aspect of tx is aggressive hydration of the skin
    
    • Children with atopic dermatitis ought to moisturize with bland emollients daily and minimize use of harsh soaps. Most pediatric dermatologists recommend daily baths followed by immediate application of a moisturizer.
    • Use pH neutral soap.
    • Daily baths to wash off bacteria. Creams placed after baths.
  • 2ND LEVEL
    
    • Hydrocortisone 1% ointment 2x/day. Use steroids on skin only for 7-10 days. Do NOT get in eyes bc can cause glaucoma
    • Topical immunomodulators/calcineurin inhibitors (tacrolimus ointment and pimecrolimus cream) are approved as 2nd line therapy for management of atopic dermatitis in children >2yo.
      
      • The topical immunomodulators have some advantages over topical steroids in that they do not cause thinning of the skin
      • Although this medication is safe with few side effects, long-term safety data is limited, and there is a controversial black box warning by FDA in 2006: “Although a causal relationship has not been established, rare cases of malignancy (skin and lymphoma) have been reported in pts treated with topical calcineurin inhibitors.”
  • 3RD LEVEL: Oral antihistamines
  • 4TH LEVEL: Wet wraps
  • 5TH LEVEL: Oral corticosteroids.
  • 6TH LEVEL: Oral antibiotics

Answer 32

ATOPIC DERMATITIS / ECZEMA
- While the exact mechanisms are not fully understood, up to 50% of pts with severe atopic dermatitis have a genetic mutation in the filaggrin gene (FLG) family

• Pt:
  
  • Pruritus is hallmark “the itch that rashes.”
  • In infants (esp <4yo), atopic dermatitis most commonly presents on the cheeks and extensor surfaces of the extremities
  • Older children who are able to scratch more areas usually have disease in the antecubital fossae, popliteal fossae, and flexor aspect of the neck.
  • Other clues to diagnosis:
    
    • Double or triple creases under the lower eyelid (Dennie Morgan lines is associated)
    • Obvious sparing around the nose (“headlight sign”)
    • Small fissures at the base of the ear lobe
    • Increased skin markings on palms and soles
    • Dry skin (xerosis)
• Risks
  
  • Staph aureus colonization is common (as many as 25% are colonized).
  • Children with atopic dermatitis are also at increased risk of disseminated skin infection with molluscum contagiosum and herpes simplex (eczema herpeticum)
• Remember: The live smallpox vaccine is NOT given to those who have atopic dermatitis or those who are immunodeficient.
• Ppx:
  
  • Research studies have shown that breastfeeding for at least 4 months (in comparison to infants fed cow’s milk formula) prevents or delays the occurrence of atopic dermatitis.
• Tx:
  
  • Avoid heat and low humidity, keep skin hydrated with creams or ointments (not lotions). Recommend gentle skin care products, unscented soaps, baby detergent when wash clothes
  • Avoidance of factors that contribute to itching, such as excessively hot or dry environments and harsh soaps and detergents
  • 1st LEVEL: The most important aspect of tx is aggressive hydration of the skin
    
    • Children with atopic dermatitis ought to moisturize with bland emollients daily and minimize use of harsh soaps. Most pediatric dermatologists recommend daily baths followed by immediate application of a moisturizer.
    • Use pH neutral soap.
    • Daily baths to wash off bacteria. Creams placed after baths.
  • 2ND LEVEL
    
    • Hydrocortisone 1% ointment 2x/day. Use steroids on skin only for 7-10 days. Do NOT get in eyes bc can cause glaucoma
    • Topical immunomodulators/calcineurin inhibitors (tacrolimus ointment and pimecrolimus cream) are approved as 2nd line therapy for management of atopic dermatitis in children >2yo.
      
      • The topical immunomodulators have some advantages over topical steroids in that they do not cause thinning of the skin
      • Although this medication is safe with few side effects, long-term safety data is limited, and there is a controversial black box warning by FDA in 2006: “Although a causal relationship has not been established, rare cases of malignancy (skin and lymphoma) have been reported in pts treated with topical calcineurin inhibitors.”
  • 3RD LEVEL: Oral antihistamines
  • 4TH LEVEL: Wet wraps
  • 5TH LEVEL: Oral corticosteroids.
  • 6TH LEVEL: Oral antibiotics

Question 33

Nummular (coinlike) eczema

- Weepy-to-lichenified round or oval plaques that exhibit crust (rather than scale) and are not elevated

Answer 33

Nummular (coinlike) eczema

- Weepy-to-lichenified round or oval plaques that exhibit crust (rather than scale) and are not elevated

Question 34

Dyshidrotic eczema (pompholyx)
- Affects the palms, soles, and sides of the digits. Characterized by small, firm, multilocular (tapioca-like) vesicles on the lateral edges of the fingers.

Answer 34

Dyshidrotic eczema (pompholyx)
- Affects the palms, soles, and sides of the digits. Characterized by small, firm, multilocular (tapioca-like) vesicles on the lateral edges of the fingers.

Question 35

Keratosis Pilaris

• ____inheritance? condition.
• Many ~1mm rough, skin-tone to pink-colored follicular papules distributed symmetrically
• Tx: Skin care with emollients and keratolytic agents (eg lactic acid, urea, topical retinoids/tretinoin)

Answer 35

Keratosis Pilaris

• AD condition.
• Many ~1mm rough, skin-tone to pink-colored follicular papules distributed symmetrically
• Tx: Skin care with emollients and keratolytic agents (eg lactic acid, urea, topical retinoids/tretinoin)

Question 36

Juvenile plantar dermatosis (moon boot foot)

• Pt: Redness, cracking, and fissuring of the weight-bearing part of the soles. It is aggravated by occlusive footwear (eg boots, sneakers) and can be confused with tinea pedis.
• Tx: Application of petrolatum for lubrication and an absorbent powder can help.

Answer 36

Juvenile plantar dermatosis (moon boot foot)

• Pt: Redness, cracking, and fissuring of the weight-bearing part of the soles. It is aggravated by occlusive footwear (eg boots, sneakers) and can be confused with tinea pedis.
• Tx: Application of petrolatum for lubrication and an absorbent powder can help.

Question 37

Seborrheic Dermatitis

• Path:
  
  • In infants is due to lingering effects of exogenous ______ on sebaceous glands, and it improves as these hormones dissipate
  • Although the cause is not fully understood, it may be the result of an inflammatory response to the yeasts of the genus _____ furfur
• Pt:
  
  • Scaling of the scalp (ie dandruff) or scaling and erythema of the eyebrows, eyelids, glabella, alar or retroauricular creases, beard or sideburn areas, or ear canals
• Tx:
  
  • Scalp involvement is treated with gentle shampooing with antiseborrheic shampoo containing ___, ____, or _____ for mild presentation. 1% hydrocortisone or 2% ketoconazole is more helpful in severe cases.
• If is extremely severe and nonresponsive to tx, consider the possibility of ______ (formerly known as histiocytosis X), esp if atrophy, ulceration, or petechiae are present. Skin biopsy is diagnostic

Answer 37

Seborrheic Dermatitis

• Path:
  
  • In infants is due to lingering effects of exogenous maternal hormones on sebaceous glands, and it improves as these hormones dissipate
  • Although the cause is not fully understood, it may be the result of an inflammatory response to the yeasts of the genus Malassezia furfur
• Pt:
  
  • Scaling of the scalp (ie dandruff) or scaling and erythema of the eyebrows, eyelids, glabella, alar or retroauricular creases, beard or sideburn areas, or ear canals
• Tx:
  
  • Scalp involvement is treated with gentle shampooing with antiseborrheic shampoo containing pyrithione zinc, selenium sulfide, or ketoconazole for mild presentation. 1% hydrocortisone or 2% ketoconazole is more helpful in severe cases.
• If is extremely severe and nonresponsive to tx, consider the possibility of Langerhans cell histiocytosis (formerly known as histiocytosis X), esp if atrophy, ulceration, or petechiae are present. Skin biopsy is diagnostic

Question 38

Intertrigo
- Irritant dermatitis of the skin folds caused by moisture, maceration, and friction. Can be secondarily infected by Candida albicans.

Answer 38

Intertrigo
- Irritant dermatitis of the skin folds caused by moisture, maceration, and friction. Can be secondarily infected by Candida albicans.

Question 39

Rosacea

• Pt:
  
  • In children, a rosacea variant called perioral dermatitis is common.
  • Traditional rosacea presents with acne-like inflammatory papules (without comedones), erythema, and telangiectases on the central face.
• Tx: Topical sulfacetamide preparations, metronidazole, and oral tetracyclines (>8yo)

Answer 39

Rosacea

• Pt:
  
  • In children, a rosacea variant called perioral dermatitis is common.
  • Traditional rosacea presents with acne-like inflammatory papules (without comedones), erythema, and telangiectases on the central face.
• Tx: Topical sulfacetamide preparations, metronidazole, and oral tetracyclines (>8yo)

Question 40

Allergic Contact dermatitis (ACD)
- Path: Type ___ hypersensitivity reaction

• Rheus dermatitis/Poison ivy dermatitis
  
  • Delayed type 4 hypersensitivity reaction
  • Caused by poison ivy, plants of genus Toxicodendron, poison sumac, or poison oak
  • Allergenic compound in these plants is _____, a mixture comprising over half of the oleoresin on the plant, which is a light, colorless oil present in fruit/leaves/stem/root of plant.
    
    • Intact plants do not cause dermatitis; touching damaged or broken plant parts results in spread of urushiol. Oleoresin is capable of seeping through clothing, rubber, latex; cannot penetrate heavy duty vinyl gloves.
    • Note: poison ivy is not spread by fluid contained in the vesicular or bullous lesions.
      
      • Fluid from ruptured vesicles is not antigenic and does not cause a spread of the dermatitis; therefore rash does not need to be covered up.
  • Pt: Erythematous, pruritic, papulovesicular rash (papules, plaques, vesicles, bullae) that is often linear
    
    • _____ distribution is key to recognizing allergic contact dermatitis caused by poison ivy, oak, or sumac.
  • Prevention of poison ivy:
    
    • _______
    • Avoidance of the inciting agent
• Nickel often found in jewelry
  
  • Jewelry composed of stainless steel or 22K gold is generally safe.
• Dx:
  
  • While a clinical hx can diagnose ACD at times, the most definitive approach is to use patch testing.
• Tx:
  
  • Cool compresses (Burow compress [aluminum acetate] is helpful for weepy lesion)
  • Medium-high topical ________
  • Oral antihistamine to decrease pruritus
  • Emollients (a lotion such as calamine or a product containing pramoxine)
  • Eliminate future exposure. Use soap and water to remove remaining antigen on skin and under nails. Wash clothes

Answer 40

Allergic Contact dermatitis (ACD)
- Path: Delayed T-cell mediated type 4 hypersensitivity reaction

• Rheus dermatitis/Poison ivy dermatitis
  
  • Delayed type 4 hypersensitivity reaction
  • Caused by poison ivy, plants of genus Toxicodendron, poison sumac, or poison oak
  • Allergenic compound in these plants is urushiol, a mixture comprising over half of the oleoresin on the plant, which is a light, colorless oil present in fruit/leaves/stem/root of plant.
    
    • Intact plants do not cause dermatitis; touching damaged or broken plant parts results in spread of urushiol. Oleoresin is capable of seeping through clothing, rubber, latex; cannot penetrate heavy duty vinyl gloves.
    • Note: poison ivy is not spread by fluid contained in the vesicular or bullous lesions.
      
      • Fluid from ruptured vesicles is not antigenic and does not cause a spread of the dermatitis; therefore rash does not need to be covered up.
  • Pt: Erythematous, pruritic, papulovesicular rash (papules, plaques, vesicles, bullae) that is often linear
    
    • Linear distribution is key to recognizing allergic contact dermatitis caused by poison ivy, oak, or sumac.
  • Prevention of poison ivy:
    
    • Bentoquatam
    • Avoidance of the inciting agent
• Nickel often found in jewelry
  
  • Jewelry composed of stainless steel or 22K gold is generally safe.
• Dx:
  
  • While a clinical hx can diagnose ACD at times, the most definitive approach is to use patch testing.
• Tx:
  
  • Cool compresses (Burow compress [aluminum acetate] is helpful for weepy lesion)
  • Medium-high topical corticosteroids
  • Oral antihistamine to decrease pruritus
  • Emollients (a lotion such as calamine or a product containing pramoxine)
  • Eliminate future exposure. Use soap and water to remove remaining antigen on skin and under nails. Wash clothes

Question 41

ACNE
- Open comedonal acne - Small dome-shaped papules with an open/dilated orifice that appears blackened

• Closed comedones - tiny yellowish-white or flesh-colored papules with surrounding erythema (whiteheads)
• Mild: Comedonal noninflammatory acne (open or closed)
  
  • Topical retinoids (effective to prevent acne) are 1st line agents for most cases. Topical retinoids improve follicular obstruction.
  • Benzoyl peroxide can also be used as primary therapy. Benzoyl peroxide address the inflammatory process.
  • Combination of topical retinoids and benzoyl peroxide is a great therapeutic starting point.
    
    • However, many retinoids are deactivated by benzoyl peroxide so must be used at different times of the day.
  • Lifestyle interventions:
    
    • Use pH-neutral detergent cleansers, as opposed to more alkaline soaps, with warm water twice daily.
    • Cleansers should be applied gently without vigorous scrubbing
    • Pts should also be encouraged to use water-based rather than oil-based skin products
• Inflammatory acne (papules, pustules)
  
  • Mild: topical retinoids (to prevent acne lesions) + benzoyl peroxide (antimicrobial agent)
  • Moderate: add topical antibiotics (eg ERYTHROMYCIN, clindamycin)
• Nodulocystic acne (nodules, cysts)
  
  • Moderate: topical retinoid + benzoyl peroxide + topical antibiotics
  • Severe: Add oral antibiotics
  • Unresponsive severe: oral isotretinoin (Accutane)
    
    • ISOTRETINOIN is highly effective oral tx in resistant cases.
      
      • Female pts, regardless of sexual activity, must have 2 NEGATIVE PREGNANCY TESTS before starting therapy.

Answer 41

ACNE
- Open comedonal acne - Small dome-shaped papules with an open/dilated orifice that appears blackened

• Closed comedones - tiny yellowish-white or flesh-colored papules with surrounding erythema (whiteheads)
• Mild: Comedonal noninflammatory acne (open or closed)
  
  • Topical retinoids (effective to prevent acne) are 1st line agents for most cases. Topical retinoids improve follicular obstruction.
  • Benzoyl peroxide can also be used as primary therapy. Benzoyl peroxide address the inflammatory process.
  • Combination of topical retinoids and benzoyl peroxide is a great therapeutic starting point.
    
    • However, many retinoids are deactivated by benzoyl peroxide so must be used at different times of the day.
  • Lifestyle interventions:
    
    • Use pH-neutral detergent cleansers, as opposed to more alkaline soaps, with warm water twice daily.
    • Cleansers should be applied gently without vigorous scrubbing
    • Pts should also be encouraged to use water-based rather than oil-based skin products
• Inflammatory acne (papules, pustules)
  
  • Mild: topical retinoids (to prevent acne lesions) + benzoyl peroxide (antimicrobial agent)
  • Moderate: add topical antibiotics (eg ERYTHROMYCIN, clindamycin)
• Nodulocystic acne (nodules, cysts)
  
  • Moderate: topical retinoid + benzoyl peroxide + topical antibiotics
  • Severe: Add oral antibiotics
  • Unresponsive severe: oral isotretinoin (Accutane)
    
    • ISOTRETINOIN is highly effective oral tx in resistant cases.
      
      • Female pts, regardless of sexual activity, must have 2 NEGATIVE PREGNANCY TESTS before starting therapy.

Question 42

Telogen effluvium
- Path: Stressors cause the hair shaft to go into the telogen hair phase “hibernation” or resting phase. In several months, when the growing phase of the hair begins again, the resting hairs fall out.

• Pt:
  
  • Hair loss that occurs 6 weeks - 4 months after stress, often in the form of illness, trauma, or childbirth.
  • On exam, hair can be easily pulled from the pt’s head (unlike most other conditions involving alopecia, in which the remaining hairs are sturdy).
• Tx: Focus on the underlying condition.

Answer 42

Telogen effluvium
- Path: Stressors cause the hair shaft to go into the telogen hair phase “hibernation” or resting phase. In several months, when the growing phase of the hair begins again, the resting hairs fall out.

• Pt:
  
  • Hair loss that occurs 6 weeks - 4 months after stress, often in the form of illness, trauma, or childbirth.
  • On exam, hair can be easily pulled from the pt’s head (unlike most other conditions involving alopecia, in which the remaining hairs are sturdy).
• Tx: Focus on the underlying condition.

Question 43

Traction alopecia
- Hair loss due to prolonged or repetitive tension on hair. Common in girls who wear tight ponytails or braids. Most common cause of alopecia in African American pts

• Pt:
  
  • Hair thinning is usually seen at the scalp margin and in the temporal areas in particular
• Tx: Reduce tension on the hair.

Answer 43

Traction alopecia
- Hair loss due to prolonged or repetitive tension on hair. Common in girls who wear tight ponytails or braids. Most common cause of alopecia in African American pts

• Pt:
  
  • Hair thinning is usually seen at the scalp margin and in the temporal areas in particular
• Tx: Reduce tension on the hair.

Question 44

Alopecia areata

• Path: Autoimmune disease in which T lymphocytes target specific autoantigens expressed by hair follicles.
  
  • Associated autoimmune diseases particularly thyroiditis
• Pt:
  
  • Sudden appearance of 1 or a few round or oval well-defined patches of hair loss (focal); but the scalp is normal and black-dot hairs are not present
    
    • In contrast to tinea capitis, there is no clinically appreciable inflammation, scale, pruritus, or lymphadenopathy
  • At the periphery of patches of alopecia, one may observe short hairs that are broader distally than proximally (________ hairs).
  • Smooth areas of nonscarring hair loss
  • _____ occurs in about 20% of patients.
    
    • Pitting nails can be helpful clinical clue to the diagnosis when it is present.
• Tx:
  
  • Patch that is small and improving - no therapy is necessary
  • For significant disease: Manage by dermatologist
    
    • 1st line is potent topical _______
    • Other options: intralesional or oral corticosteroids, excimer laser, topical anthralin, or topical immunotherapy using squaric acid dibutyl ester or diphenylcyclopropenone.

Answer 44

Alopecia areata

• Path: Autoimmune disease in which T lymphocytes target specific autoantigens expressed by hair follicles.
  
  • Associated autoimmune diseases particularly thyroiditis
• Pt:
  
  • Sudden appearance of 1 or a few round or oval well-defined patches of hair loss (focal); but the scalp is normal and black-dot hairs are not present
    
    • In contrast to tinea capitis, there is no clinically appreciable inflammation, scale, pruritus, or lymphadenopathy
  • At the periphery of patches of alopecia, one may observe short hairs that are broader distally than proximally (exclamation-point hairs).
  • Smooth areas of nonscarring hair loss
  • Nail pitting occurs in about 20% of patients.
    
    • Pitting nails can be helpful clinical clue to the diagnosis when it is present.
• Tx:
  
  • Patch that is small and improving - no therapy is necessary
  • For significant disease: Manage by dermatologist
    
    • 1st line is potent topical corticosteroid
    • Other options: intralesional or oral corticosteroids, excimer laser, topical anthralin, or topical immunotherapy using squaric acid dibutyl ester or diphenylcyclopropenone.

Question 45

Trichotillomania (Hair pulling)

• Pt:
  
  • Irregular patches (often localized and geometric in appearance) with broken hair of varying lengths
• Iron deficiency anemia has also been associated with trichotillomania and trichophagia.
• Tx:
  
  • Often responds to positive reinforcement techniques / behavior modification and generally resolves within 6 months
  • Medications used in the tx of compulsion disorders, such as fluoxetine, can be helpful.

Answer 45

Trichotillomania (Hair pulling)

• Pt:
  
  • Irregular patches (often localized and geometric in appearance) with broken hair of varying lengths
• Iron deficiency anemia has also been associated with trichotillomania and trichophagia.
• Tx:
  
  • Often responds to positive reinforcement techniques / behavior modification and generally resolves within 6 months
  • Medications used in the tx of compulsion disorders, such as fluoxetine, can be helpful.

Question 46

Tinea capitis is the most common cause of alopecia in children

• Pt: Scalp inflammation, scale, and often occipital or cervical lymphadenopathy
  
  • Patches of hair loss with overlying scale and sometimes with ____ indicating broken hairs.

Answer 46

Tinea capitis is the most common cause of alopecia in children

• Pt: Scalp inflammation, scale, and often occipital or cervical lymphadenopathy
  
  • Patches of hair loss with overlying scale and sometimes with black dots indicating broken hairs.

Question 47

HIDRADENITIS SUPPURATIVE
- Inflammatory scarring process of the axillae, inguinal folds, and other intertriginous areas

• Tx:
  
  • Warm compresses and topical _____ 1% BID
  • More severe, PO _____
  • Tx modalities include topical benzoyl peroxide, diluted bleach baths, intralesional steroid injections, topical or oral tetracyclines, dapsone, and in severe cases, surgical excision of affected tissue and (more recently) anti-TNF medications.

Answer 47

HIDRADENITIS SUPPURATIVE
- Inflammatory scarring process of the axillae, inguinal folds, and other intertriginous areas

• Tx:
  
  • Warm compresses and topical clindamycin 1% BID
  • More severe, PO doxycycline
  • Tx modalities include topical benzoyl peroxide, diluted bleach baths, intralesional steroid injections, topical or oral tetracyclines, dapsone, and in severe cases, surgical excision of affected tissue and (more recently) anti-TNF medications.

Question 48

Desensitization is contraindicated for _____, such as _ syndrome, _ syndrome, and _.

• It is NEVER attempted in patients with hx of reactions involving significant skin desquamation, such as Steven-Johnson syndrome or TEN, bc even small doses of the drug may induce irreversible and potentially fatal recurrent desquamative reactions. Erythema multiforme and diffuse erythroderma with desquamation are other types of reactions for which desensitization should not be attempted. Desensitization is also not indicated for serum sickness reaction, nephritis, hepatitis, and other severe non IgE-mediated reactions.

Answer 48

Desensitization is contraindicated for severe cutaneous drug reactions, such as DRESS syndrome, Stevens-Johnson syndrome, and TEN.

• It is NEVER attempted in patients with hx of reactions involving significant skin desquamation, such as Steven-Johnson syndrome or TEN, bc even small doses of the drug may induce irreversible and potentially fatal recurrent desquamative reactions. Erythema multiforme and diffuse erythroderma with desquamation are other types of reactions for which desensitization should not be attempted. Desensitization is also not indicated for serum sickness reaction, nephritis, hepatitis, and other severe non IgE-mediated reactions.

Question 49

Vancomycin Hypersensitivity “Red man syndrome”
- Tx: Decrease the rate of the vancomycin infusion since administration of appropriate abx impacts mortality, not by decreasing the dose.

Answer 49

Vancomycin Hypersensitivity “Red man syndrome”
- Tx: Decrease the rate of the vancomycin infusion since administration of appropriate abx impacts mortality, not by decreasing the dose.

Question 50

Drug-induced exanthems (exanthematous drug reaction) - 95% of drug-induced cutaneous eruptions
- Diffuse maculopapular rash develops during the initial 5-14 days of starting a medication.

Answer 50

Drug-induced exanthems (exanthematous drug reaction) - 95% of drug-induced cutaneous eruptions
- Diffuse maculopapular rash develops during the initial 5-14 days of starting a medication.

Question 51

Serum Sickness
- Type 3 Immune complex-mediated disease process that follows the administration of certain medications.

• Path: Interaction of IgG (sometimes IgM) and a foreign antigen produces the reaction (not IgE mediated).
  
  • Serum sickness differs from IgE-mediated immediate hypersensitivity reactions in that serum sickness does NOT require prior exposure to an antigen (prior sensitization) for a reaction to occur. Thus, serum sickness can develop on the initial exposure.
• Pt: Urticarial rash, fever, polyarthralgias or polyarthritis, and malaise.
  
  • The reaction usually develops 6-12 days after exposure as opposed to immediately following exposure.

Answer 51

Serum Sickness
- Type 3 Immune complex-mediated disease process that follows the administration of certain medications.

• Path: Interaction of IgG (sometimes IgM) and a foreign antigen produces the reaction (not IgE mediated).
  
  • Serum sickness differs from IgE-mediated immediate hypersensitivity reactions in that serum sickness does NOT require prior exposure to an antigen (prior sensitization) for a reaction to occur. Thus, serum sickness can develop on the initial exposure.
• Pt: Urticarial rash, fever, polyarthralgias or polyarthritis, and malaise.
  
  • The reaction usually develops 6-12 days after exposure as opposed to immediately following exposure.

Question 52

Stevens-Johnson Syndrome

• Severe drug reaction that appears up to 3 weeks after starting a medication and is characterized by mucosal lesions at 2 or more sites and widespread targetoid or macular skin lesions preceded by a prodrome of fever, sore throat, and cough.
• <10% BSA (skin sloughing and mucous membrane involvement)
• +Nikolsky sign- sloughing of the epidermis with slight tangential pressure.
• Tx: Essential that potentially causative medications be discontinued immediately.

Answer 52

Stevens-Johnson Syndrome

• Severe drug reaction that appears up to 3 weeks after starting a medication and is characterized by mucosal lesions at 2 or more sites and widespread targetoid or macular skin lesions preceded by a prodrome of fever, sore throat, and cough.
• <10% BSA (skin sloughing and mucous membrane involvement)
• +Nikolsky sign- sloughing of the epidermis with slight tangential pressure.
• Tx: Essential that potentially causative medications be discontinued immediately.

Question 53

SJS/TEN overlap

- 10-30% of body surface area affected

Answer 53

SJS/TEN overlap

- 10-30% of body surface area affected

Question 54

Toxic Epidermal Necrolysis (TEN)

• > ____% BSA (skin sloughing and mucous membrane involvement).
• Pt: Full thickness epidermal necrosis (in contrast to SSSS where the split is more superficial (subcorneal).
• • Nikolsky sign
• Tx: Discontinue drug. The role of glucocorticoids, cyclosporine and IVIG is controversial. Glucocorticosteroids are contraindicated.

Answer 54

Toxic Epidermal Necrolysis (TEN)

• > 30% BSA (skin sloughing and mucous membrane involvement).
• Pt: Full thickness epidermal necrosis (in contrast to SSSS where the split is more superficial (subcorneal).
• • Nikolsky sign
• Tx: Discontinue drug. The role of glucocorticoids, cyclosporine and IVIG is controversial. Glucocorticosteroids are contraindicated.

Question 55

DRESS/DIH (Drug rash with eosinophilia and systemic symptoms/Drug induced hypersensitivity)

• NON-IgE mediated
• Life-threatening condition characterized by rash, hematologic abnormalities (eosinophilia), lymphadenopathy, and internal organ involvement that develops typically 2-6 weeks after starting the causative medication
• Pt:
  
  • Usually occurs 2-8 weeks after exposure to the offending drug
  • Polymorphous drug rash and systemic symptoms, including fever, lymphadenopathy, and multiorgan involvement (eg hepatitis, carditis, nephritis).

Answer 55

DRESS/DIH (Drug rash with eosinophilia and systemic symptoms/Drug induced hypersensitivity)

• NON-IgE mediated
• Life-threatening condition characterized by rash, hematologic abnormalities (eosinophilia), lymphadenopathy, and internal organ involvement that develops typically 2-6 weeks after starting the causative medication
• Pt:
  
  • Usually occurs 2-8 weeks after exposure to the offending drug
  • Polymorphous drug rash and systemic symptoms, including fever, lymphadenopathy, and multiorgan involvement (eg hepatitis, carditis, nephritis).

Question 56

Erythema Multiforme

• Typically reactive process to a viral illness, classically _______
  
  • Associated with HSV type 1 and 2, EBV, mycoplasma pneumoniae, certain medications such as NSAIDs and antibiotics
  • ____ is most common cause
• Subdivision
  
  • EM minor
  • EM major - has mucosal involvement and pts can be systemically ill
• Pt:
  
  • Compared to SJS/TEN, EM does not have systemic symptoms of fever and malaise.
  • Skin lesions in EM are targetoid without epidermal detachment, and pts have either little or no mucosal involvement.
  • Classic rash: Target or iris-like papule with erythematous border surrounding pale ring with dusky/purple center.
• Important to differentiate EM from urticaria. In contrast to EM, urticaria is migratory (is present in one place for <24h), more edematous, and pink in color without a dusky appearance.
• Self-limited rash that will spontaneously resolve after 7 days

Answer 56

Erythema Multiforme

• Typically reactive process to a viral illness, classically HSV
  
  • Associated with HSV type 1 and 2, EBV, mycoplasma pneumoniae, certain medications such as NSAIDs and antibiotics
  • NSAID is most common cause
• Subdivision
  
  • EM minor
  • EM major - has mucosal involvement and pts can be systemically ill
• Pt:
  
  • Compared to SJS/TEN, EM does not have systemic symptoms of fever and malaise.
  • Skin lesions in EM are targetoid without epidermal detachment, and pts have either little or no mucosal involvement.
  • Classic rash: Target or iris-like papule with erythematous border surrounding pale ring with dusky/purple center.
• Important to differentiate EM from urticaria. In contrast to EM, urticaria is migratory (is present in one place for <24h), more edematous, and pink in color without a dusky appearance.
• Self-limited rash that will spontaneously resolve after 7 days

Question 57

Psoriasis

• Types
  
  • Chronic plaque psoriasis - most common (75%)
    
    • Has distinctive, well-demarcated, pink plaques with micaceous (silvery) scale. Distribution is symmetrical and typically involves the extensor surfaces of the knees and elbows, the lower back, and the scalp.
    • Plaques can appear in response to trauma (Koebner phenomenon), and these have a geometric or linear appearance.
    • Auspitz sign is a feature of psoriasis and is defined by pinpoint bleeding with removal of scale.
  • Guttate psoriasis - Occurs commonly in children
  • Erythrodermic psoriasis (more severe)
  • Pustular psoriasis (more severe)
• Nail psoriasis may be an isolated finding or may precede, coincide with, or follow the onset of disease elsewhere on the body.
  
  • The most common manifestation is pitting, but individuals may develop thickening, yellowing (“oil drop” sign), roughness of the nails, nail fragility, and even separation of the nail plate from the nail bed (onycholysis caused by psoriasis of the nail bed).
  • However, these changes are not pathognomonic of psoriasis.
  • Of note, there is an association bw nail changes in psoriasis and psoriatic arthritis
• Tx to reduce inflammation and normalize epidermal proliferation
  
  • 1st line therapy consists of ____ and _____
  • Phototherapy or systemic agents (methotrexate, cyclosporin A, acitretin, or biologics) are reserved for pts with severe disease that does not adequately respond to topical tx.
  • The newest agents for moderate-severe psoriasis include the____ (eg etanercept, adalimumab, infliximab), as well as the newer IL-12/23, IL-23, and IL-17 inhibitors
    
    • FDA mandates a black box warning for increased risk of ____

Answer 57

Psoriasis

• Types
  
  • Chronic plaque psoriasis - most common (75%)
    
    • Has distinctive, well-demarcated, pink plaques with micaceous (silvery) scale. Distribution is symmetrical and typically involves the extensor surfaces of the knees and elbows, the lower back, and the scalp.
    • Plaques can appear in response to trauma (Koebner phenomenon), and these have a geometric or linear appearance.
    • Auspitz sign is a feature of psoriasis and is defined by pinpoint bleeding with removal of scale.
  • Guttate psoriasis - Occurs commonly in children
  • Erythrodermic psoriasis (more severe)
  • Pustular psoriasis (more severe)
• Nail psoriasis may be an isolated finding or may precede, coincide with, or follow the onset of disease elsewhere on the body.
  
  • The most common manifestation is pitting, but individuals may develop thickening, yellowing (“oil drop” sign), roughness of the nails, nail fragility, and even separation of the nail plate from the nail bed (onycholysis caused by psoriasis of the nail bed).
  • However, these changes are not pathognomonic of psoriasis.
  • Of note, there is an association bw nail changes in psoriasis and psoriatic arthritis
• Tx to reduce inflammation and normalize epidermal proliferation
  
  • 1st line therapy consists of topical steroids and topical vitamin D analogues (eg calcipotriene).
  • Phototherapy or systemic agents (methotrexate, cyclosporin A, acitretin, or biologics) are reserved for pts with severe disease that does not adequately respond to topical tx.
  • The newest agents for moderate-severe psoriasis include the TNF-alpha inhibitors (eg etanercept, adalimumab, infliximab), as well as the newer IL-12/23, IL-23, and IL-17 inhibitors
    
    • FDA mandates a black box warning for increased risk of hematology malignancies.

Question 58

Erythema Nodosum

• Path: Inflammation of the subcutaneous fat (panniculitis)
• Pt: Red, tender nodules on the shins.
• Tx:
  
  • Removal or tx of the inciting trigger often results in resolution of the EN.
  • Rest, elevation, NSAIDs

Answer 58

Erythema Nodosum

• Path: Inflammation of the subcutaneous fat (panniculitis)
• Pt: Red, tender nodules on the shins.
• Tx:
  
  • Removal or tx of the inciting trigger often results in resolution of the EN.
  • Rest, elevation, NSAIDs

Question 59

Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)
- Rare cutaneous inflammatory disorder of unknown cause that is hypothesized to represent a hypersensitivity reaction to a currently unidentified infectious agent.

• Pt:
  
  • Lesions begin as reddish-brown macules surrounded by an erythematous halo that rapidly progress into papules and papulovesicles.
  • As older lesions crust over and heal, new lesions often appear in successive crops.
  • The chronicity of the rash serves as an important diagnostic finding in that new lesions may continue to appear over a period of 4-6 weeks or longer.
• Tx:
  
  • _______ 30-50 mg/kg/day for 6-8 weeks followed by a slow taper over several months usually results in resolution of symptoms and prevents recurrence

Answer 59

Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)
- Rare cutaneous inflammatory disorder of unknown cause that is hypothesized to represent a hypersensitivity reaction to a currently unidentified infectious agent.

• Pt:
  
  • Lesions begin as reddish-brown macules surrounded by an erythematous halo that rapidly progress into papules and papulovesicles.
  • As older lesions crust over and heal, new lesions often appear in successive crops.
  • The chronicity of the rash serves as an important diagnostic finding in that new lesions may continue to appear over a period of 4-6 weeks or longer.
• Tx:
  
  • Erythromycin 30-50 mg/kg/day for 6-8 weeks followed by a slow taper over several months usually results in resolution of symptoms and prevents recurrence

Question 60

Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)

• Pt:
  
  • Usually preceded by upper respiratory symptoms.
  • Painful nodules, papules, and/or plaques asymmetrically distributed on the face, neck, and limbs.
• Leukocytosis and renal, hepatic, and pancreatic dysfunction often accompany the disorder.
• Biopsy of the lesions reveals dense neutrophilic perivascular infiltration of the dermis.
• Bc >50% of cases have underlying disorders, thoroughly evaluate all children with Sweet syndrome for immunodeficiency, collagen vascular disease, inflammatory bowel disease, or malignancy.
• Tx: Systemic ______ until the disease is controlled (typically 1-2 weeks), then taper over 4-6 weeks.

Answer 60

Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)

• Pt:
  
  • Usually preceded by upper respiratory symptoms.
  • Painful nodules, papules, and/or plaques asymmetrically distributed on the face, neck, and limbs.
• Leukocytosis and renal, hepatic, and pancreatic dysfunction often accompany the disorder.
• Biopsy of the lesions reveals dense neutrophilic perivascular infiltration of the dermis.
• Bc >50% of cases have underlying disorders, thoroughly evaluate all children with Sweet syndrome for immunodeficiency, collagen vascular disease, inflammatory bowel disease, or malignancy.
• Tx: Systemic corticosteroids until the disease is controlled (typically 1-2 weeks), then taper over 4-6 weeks.

Question 61

Impetigo

• Infection confined to the epidermis (most cases are due to ______)
• Pt: Pink patches or superficial erosions with honey-colored crust.

Answer 61

Impetigo

• Infection confined to the epidermis (most cases are due to S aureus)
• Pt: Pink patches or superficial erosions with honey-colored crust.

Question 62

Erysipelas

• Superficial infection
• Path: Beta-hemolytic streptococci (Group A strep / Strep pyogenes), Staph aureus
• Pt: Bright pink-red, well-demarcated plaques. In contrast to impetigo, the skin is tender.

Answer 62

Erysipelas

• Superficial infection
• Path: Beta-hemolytic streptococci (Group A strep / Strep pyogenes), Staph aureus
• Pt: Bright pink-red, well-demarcated plaques. In contrast to impetigo, the skin is tender.

Question 63

Cellulitis
- Path: Beta-hemolytic streptococci (Group A strep / Strep pyogenes), Staph aureus

• Pt
  
  • Skin erythema, warmth, and edema.
  • Infected area is also tender but not as well demarcated from the surrounding healthy skin when compared to erysipelas.
• Tx:
  
  • For hemodynamically stable children with cellulitis and risk factors for MRSA, initial antibiotic choice should include coverage for MRSA and B-hemolytic Streptococcus. _____ is the preferred 1st line agent.
    
    • In uncomplicated cellulitis, a 5-day course is as effective as a 10-day course
  • In communities where the prevalence of clindamycin-resistance MRSA is >15%, an alternative antibiotic strategy such as combination therapy (eg amoxicillin or cephalexin + TMP/SMX or doxycycline), vancomycin, or linezolid must be considered.

Answer 63

Cellulitis
- Path: Beta-hemolytic streptococci (Group A strep / Strep pyogenes), Staph aureus

• Pt
  
  • Skin erythema, warmth, and edema.
  • Infected area is also tender but not as well demarcated from the surrounding healthy skin when compared to erysipelas.
• Tx:
  
  • For hemodynamically stable children with cellulitis and risk factors for MRSA, initial antibiotic choice should include coverage for MRSA and B-hemolytic Streptococcus. Clindamycin is the preferred 1st line agent.
    
    • In uncomplicated cellulitis, a 5-day course is as effective as a 10-day course
  • In communities where the prevalence of clindamycin-resistance MRSA is >15%, an alternative antibiotic strategy such as combination therapy (eg amoxicillin or cephalexin + TMP/SMX or doxycycline), vancomycin, or linezolid must be considered.

Question 64

Necrotizing fasciitis (streptococcal gangrene)

• Deep cellulitis involving the subcutaneous fat and fascia.
• Path: Often polymicrobial, can be caused by strep and staph and anaerobic bacteria
  
  • Varicella skin lesions can serve as a portal of entry for group A streptococcal infection / strep pyogenes
• Pt: Poorly demarcated erythema and swelling, and involved areas are exquisitely tender (pts experience pain out of proportion to exam)
  
  • Crepitus is a common finding and can be a sign of anaerobic infection.
  • After 4-6 days, frank gangrene can occur
• Tx:
  
  • Any delay in tx of ANF significantly increases the risk of a poor outcome
  • Emergent surgical exploration and debridement in the OR
  • IV penicillin and clindamycin
    
    • Broad-spectrum IV antibiotics 3rd gen cephalosporin, clindamycin, ampicillin.

Answer 64

Necrotizing fasciitis (streptococcal gangrene)

• Deep cellulitis involving the subcutaneous fat and fascia.
• Path: Often polymicrobial, can be caused by strep and staph and anaerobic bacteria
  
  • Varicella skin lesions can serve as a portal of entry for group A streptococcal infection / strep pyogenes
• Pt: Poorly demarcated erythema and swelling, and involved areas are exquisitely tender (pts experience pain out of proportion to exam)
  
  • Crepitus is a common finding and can be a sign of anaerobic infection.
  • After 4-6 days, frank gangrene can occur
• Tx:
  
  • Any delay in tx of ANF significantly increases the risk of a poor outcome
  • Emergent surgical exploration and debridement in the OR
  • IV penicillin and clindamycin
    
    • Broad-spectrum IV antibiotics 3rd gen cephalosporin, clindamycin, ampicillin.

Question 65

Perianal streptococcal infection / Perianal bacterial dermatitis (formerly termed perianal streptococcal dermatitis or perianal cellulitis) “Strep butt”

• Superficial cellulitis caused by Strep pyogenes or Staph aureus
• Pt:
  
  • 80% present with pruritus
  • 50% with perineal pain/burning (often leading to constipation due to pain with defecation)
  • 33% with blood-streaked stools
• Tx:
  
  • 10 day course of oral ________.
  • Bc may be caused by Strep pyogenes or Staph aureus, empiric tx with PO cephalexin or another antistaphylococcal abx based on local sensitivity patterns.

Answer 65

Perianal streptococcal infection / Perianal bacterial dermatitis (formerly termed perianal streptococcal dermatitis or perianal cellulitis) “Strep butt”

• Superficial cellulitis caused by Strep pyogenes or Staph aureus
• Pt:
  
  • 80% present with pruritus
  • 50% with perineal pain/burning (often leading to constipation due to pain with defecation)
  • 33% with blood-streaked stools
• Tx:
  
  • 10 day course of oral penicillin.
  • Bc may be caused by Strep pyogenes or Staph aureus, empiric tx with PO cephalexin or another antistaphylococcal abx based on local sensitivity patterns.

Question 66

Staphylococcal scalded skin syndrome (SSSS) / Pemphigus neonatorum / Ritter’s disease

• Path: Develops after localized staph infection, caused by exfoliative toxin produced by Staph aureus - epidermolytic exotoxins A and B that are proteases that cleave _______
• Pt: Diffuse cutaneous bullous disease. Diffuse blanching erythematous skin, often starting in the perioral region. Sheets of epidermis peel away. Flaccid blisters appear 1-2 days later
  
  • Affected infants present with fever and are irritable with diffuse, blanching erythema, often starting in the perioral region. Flaccid blisters appear 1-2 days later, esp in areas of mechanical stress
• Tx: IV antistaphylococcal antibiotics such as ____, ____, or ____
• Prognosis: Excellent, with complete resolution usually within 10 days for infants. Lesions heal without scarring.

Answer 66

Staphylococcal scalded skin syndrome (SSSS) / Pemphigus neonatorum / Ritter’s disease

• Path: Develops after localized staph infection, caused by exfoliative toxin produced by Staph aureus - epidermolytic exotoxins A and B that are proteases that cleave desmoglein-1
• Pt: Diffuse cutaneous bullous disease. Diffuse blanching erythematous skin, often starting in the perioral region. Sheets of epidermis peel away. Flaccid blisters appear 1-2 days later
  
  • Affected infants present with fever and are irritable with diffuse, blanching erythema, often starting in the perioral region. Flaccid blisters appear 1-2 days later, esp in areas of mechanical stress
• Tx: IV antistaphylococcal antibiotics such as oxacillin, nafcillin, or vancomycin
• Prognosis: Excellent, with complete resolution usually within 10 days for infants. Lesions heal without scarring.

Question 67

Toxic shock syndrome (TSS)

• Path: _____ produces bacterial exotoxin / toxic shock syndrome toxin-1 (TSST-1) is responsible for over 90% of menstrual cases and at least half of nonmenstrual causes. Other toxins, including staphylococcal enterotoxins (A-E, H) as well as streptococcal pyogenic exotoxins A and B, can cause TSS
  
  • Half of cases are not associated with menstruation
• Dx: Clinical criteria
  
  • Fever > 38.9 (102)
  • Rash (typically diffuse erythroderma)
    
    • With subsequent Desquamation (commonly palms/soles 1-2 weeks after onset of symptoms)
  • Hypotension (SBP <5% for age for children <16yo, <90mmhg for >16yo)
  • Multisystem involvement (in >3 organ symptoms)
    
    • GI - vomiting or diarrhea at onset of illness
    • MSK - severe myalgias at onset of illness or CPK >2x upper limit of normal
    • Mucocutaneous - vaginal, oropharyngeal, and/or conjunctival hyperemia
    • Renal - BUN or Cr >2x upper limit of normal, or urine with >5 WBC/hpf without UTI
    • Hepatic - total bilirubin or AST/ALT >2x upper limit of normal
    • Hematology - platelet count <100x10^3/uL (100x10^9/L)
    • CNS - AMS without focal neurologic signs when afebrile and normotensive
  • Negative results on the following tests or serology, if obtained
    
    • Blood, throat, or CSF cultures (blood culture may be + for S aureus)
    • Rocky Mountain spotted fever, leptospirosis, or measles
• Dx: Clinical criteria. While 80-90% of wound and mucosal cultures are positive for S aureus in TSS patients, <5% of blood cultures are positive.
• Tx:
  
  • Fluid management, _____ (or other appropriate antistaphylococcal antibiotic) plus _____ (decreases toxin production)

Answer 67

Toxic shock syndrome (TSS)

• Path: Staph aureus produces bacterial exotoxin / toxic shock syndrome toxin-1 (TSST-1) is responsible for over 90% of menstrual cases and at least half of nonmenstrual causes. Other toxins, including staphylococcal enterotoxins (A-E, H) as well as streptococcal pyogenic exotoxins A and B, can cause TSS
  
  • Half of cases are not associated with menstruation
• Dx: Clinical criteria
  
  • Fever > 38.9 (102)
  • Rash (typically diffuse erythroderma)
    
    • With subsequent Desquamation (commonly palms/soles 1-2 weeks after onset of symptoms)
  • Hypotension (SBP <5% for age for children <16yo, <90mmhg for >16yo)
  • Multisystem involvement (in >3 organ symptoms)
    
    • GI - vomiting or diarrhea at onset of illness
    • MSK - severe myalgias at onset of illness or CPK >2x upper limit of normal
    • Mucocutaneous - vaginal, oropharyngeal, and/or conjunctival hyperemia
    • Renal - BUN or Cr >2x upper limit of normal, or urine with >5 WBC/hpf without UTI
    • Hepatic - total bilirubin or AST/ALT >2x upper limit of normal
    • Hematology - platelet count <100x10^3/uL (100x10^9/L)
    • CNS - AMS without focal neurologic signs when afebrile and normotensive
  • Negative results on the following tests or serology, if obtained
    
    • Blood, throat, or CSF cultures (blood culture may be + for S aureus)
    • Rocky Mountain spotted fever, leptospirosis, or measles
• Dx: Clinical criteria. While 80-90% of wound and mucosal cultures are positive for S aureus in TSS patients, <5% of blood cultures are positive.
• Tx:
  
  • Fluid management, vancomycin (or other appropriate antistaphylococcal antibiotic) plus clindamycin (decreases toxin production)

Question 68

Pseudomonas

• Pt:
  
  • “Hot tube folliculitis” - edematous follicular papules, usually on the trunk following use of a nonchlorinated hot tub. Eruption typically resolves without tx in 1 week.
  • Pseudomonal septicemia - small necrotic macules and papules.
    
    • Neutropenic pts who are very ill can present with such findings, sometimes termed ecthyma gangrenosum (contrast to ecthyma caused by group A strep)

Answer 68

Pseudomonas

• Pt:
  
  • “Hot tube folliculitis” - edematous follicular papules, usually on the trunk following use of a nonchlorinated hot tub. Eruption typically resolves without tx in 1 week.
  • Pseudomonal septicemia - small necrotic macules and papules.
    
    • Neutropenic pts who are very ill can present with such findings, sometimes termed ecthyma gangrenosum (contrast to ecthyma caused by group A strep)

Question 69

Erythrasma

• Path: ______ minutissimum
• Pt: Well-defined brown-to-pink slightly scaly patches of the axillae, groin, and toe webs.
• Dx: Fluoresces bright ____ with a Wood’s lamp
• Tx: PO or typical _____ +/- an -azole antifungal cream.

Answer 69

Erythrasma

• Path: Corynebacterium minutissimum
• Pt: Well-defined brown-to-pink slightly scaly patches of the axillae, groin, and toe webs.
• Dx: Fluoresces bright red with a Wood’s lamp
• Tx: PO or typical erythromycin +/- an -azole antifungal cream.

Question 70

Warts (Verrucae)

• Tx for verruca vulgaris/plana/plantaris
  
  • Most (>65%) resolve spontaneously within 2 years. Therefore, small single lesions often do not require tx.
  • Options: Destructive modalities (liquid nitrogen), topical irritants (eg salicylic acid, retinoids), intralesional therapies (eg Candida [skin test antigen], bleomycin), and sometimes laser therapies.
  • Topical salicylic acid is preferred tx.

Answer 70

Warts (Verrucae)

• Tx for verruca vulgaris/plana/plantaris
  
  • Most (>65%) resolve spontaneously within 2 years. Therefore, small single lesions often do not require tx.
  • Options: Destructive modalities (liquid nitrogen), topical irritants (eg salicylic acid, retinoids), intralesional therapies (eg Candida [skin test antigen], bleomycin), and sometimes laser therapies.
  • Topical SALICYLIC ACID is preferred tx.

Question 71

Molluscum Contagiosum

• Path: Infection by poxvirus
• Pt: Smooth, umbilicated, yellowish papules that vary in size <1mm to several millimeters.
• Management: The majority of cases will resolve on their own in 2-3 years (6-18mo) without treatment.

Answer 71

Molluscum Contagiosum

• Path: Infection by poxvirus
• Pt: Smooth, umbilicated, yellowish papules that vary in size <1mm to several millimeters.
• Management: The majority of cases will resolve on their own in 2-3 years (6-18mo) without treatment.

Question 72

Papular Acrodermatitis (Gianotti-Crosti Syndrome)

• Exact etiology is unknown; possibly viral or bacterial infection
  
  • Hepatitis was the most common triggering viral illness prior to routine immunization for hepatitis A and B
  • EBV is the most common
• Pt: Multiple, slightly edematous pink-tan 1-10mm papules in a symmetric distribution on the face, buttocks, and extensor surfaces of the knees and elbows.
• Perform hepatitis serologies and liver function studies in pts with lymphadenopathy and/or hepatosplenomegaly.
• Tx: Tx toward the triggering illness, if indicated.
  
  • Rash is tx with topical corticosteroid or antihistamines if symptomatic.

Answer 72

Papular Acrodermatitis (Gianotti-Crosti Syndrome)

• Exact etiology is unknown; possibly viral or bacterial infection
  
  • Hepatitis was the most common triggering viral illness prior to routine immunization for hepatitis A and B
  • EBV is the most common
• Pt: Multiple, slightly edematous pink-tan 1-10mm papules in a symmetric distribution on the face, buttocks, and extensor surfaces of the knees and elbows.
• Perform hepatitis serologies and liver function studies in pts with lymphadenopathy and/or hepatosplenomegaly.
• Tx: Tx toward the triggering illness, if indicated.
  
  • Rash is tx with topical corticosteroid or antihistamines if symptomatic.

Question 73

Tinea corporis (ringworm) - Body

• Tx:
  
  • 1st line/localized - topical ______ BID for as long as it takes to resolve (1-3 weeks depending on resolution) plus 1 additional week afterwards.
    
    • Topical antifungals: clotrimazole, ketaoconazole, haloprogin, miconazole, tolnaftate, terbinafine, naftifine, or griseofulvin
  • 2nd line/extensive: Oral antifungals (eg terbinafine, griseofulvin, itraconazole).
  • Avoid sharing clothing/contact.

Answer 73

Tinea corporis (ringworm) - Body

• Tx:
  
  • 1st line/localized - topical antifungals (eg clotrimazole, terbinafine, miconazole) BID for as long as it takes to resolve (1-3 weeks depending on resolution) plus 1 additional week afterwards.
    
    • Topical antifungals: clotrimazole, ketaoconazole, haloprogin, miconazole, tolnaftate, terbinafine, naftifine, or griseofulvin
  • 2nd line/extensive: Oral antifungals (eg terbinafine, griseofulvin, itraconazole).
  • Avoid sharing clothing/contact.

Question 74

Tinea cruris - Groin
- Tx: ______ antifungals (OTC terbinafine or prescription strength -azole antifungals). Length of therapy is 1-3 weeks, depending on resolution.

Answer 74

Tinea cruris - Groin
- Tx: Topical antifungals (OTC terbinafine or prescription strength -azole antifungals). Length of therapy is 1-3 weeks, depending on resolution.

Question 75

Tinea unguium (Onychomycosis) - Nail
- Pt: White or yellow, brittle, and/or thickened nails

• Tx: Treatment is difficult and requires 6-12 weeks of _____. Confirm with KOH prep
  
  • Monitoring for toxicity with ____ and ____ tests is needed bc of the prolonged course of therapy.

Answer 75

Tinea unguium (Onychomycosis) - Nail
- Pt: White or yellow, brittle, and/or thickened nails

• Tx: Treatment is difficult and requires 6-12 weeks of Oral antifungals (terbinafine or itraconazole). Confirm with KOH prep
  
  • Monitoring for toxicity with CBC and liver function tests is needed bc of the prolonged course of therapy.

Question 76

Tinea capitis - Head - oral antifungal (griseofulvin). Confirm with KOH prep.

• Pt: 3 patterns of infection
  
  • Alopecia: One or more well-defined patches of hair loss with associated scale.
    
    • Infection with T tonsurans causes hairs to break at the scalp, resulting in black-dot hairs and complete alopecia.
  • Seborrheic
  • Inflammatory: When there is an immune response to the fungus, may develop pustules and crusting or a tender, boggy mass, usually devoid of hair and covered with exudate (_____).
• Tx: Oral therapy
  
  • 1st line is Oral antifungal ______ for 6-8 weeks
    
    • It does not require ___ monitoring (terbinafine and itraconazole require liver monitoring every 4-6 weeks).
  • Terbinafine tablets (10-20 kg, 62.5mg/d; 21-40kg, 125mg/day, >40kg, 250mg/day) have become 1st line therapy for some clinicians bc of the shorter tx course (4-6 weeks) and favorable cost profile

Answer 76

Tinea capitis - Head - oral antifungal (griseofulvin). Confirm with KOH prep.

• Pt: 3 patterns of infection
  
  • Alopecia: One or more well-defined patches of hair loss with associated scale.
    
    • Infection with T tonsurans causes hairs to break at the scalp, resulting in black-dot hairs and complete alopecia.
  • Seborrheic
  • Inflammatory: When there is an immune response to the fungus, may develop pustules and crusting or a tender, boggy mass, usually devoid of hair and covered with exudate (KERION).
• Tx: Oral therapy
  
  • 1st line is Oral antifungal griseofulvin for 6-8 weeks
    
    • It does not require LFT monitoring (terbinafine and itraconazole require liver monitoring every 4-6 weeks).
  • Terbinafine tablets (10-20 kg, 62.5mg/d; 21-40kg, 125mg/day, >40kg, 250mg/day) have become 1st line therapy for some clinicians bc of the shorter tx course (4-6 weeks) and favorable cost profile

Question 77

Tinea pedis - Foot

• Tx:
  
  • Topical antifungals like ____ or miconazole nitrates or econazole is 1st line, applied twice daily until eruption resolves (usually 2-4 weeks).
  • If tx if ineffective, a fungicidal topical agent like terbinafine or naftifine should be used.
  • For widespread or resistant infections, an oral antifungal is required, like griseofulvin, terbinafine, or fluconazole

Answer 77

Tinea pedis - Foot

• Tx:
  
  • Topical antifungals like clotrimazole or miconazole nitrates or econazole is 1st line, applied twice daily until eruption resolves (usually 2-4 weeks).
  • If tx if ineffective, a fungicidal topical agent like terbinafine or naftifine should be used.
  • For widespread or resistant infections, an oral antifungal is required, like griseofulvin, terbinafine, or fluconazole

Question 78

Lice
- Dx: Finding live lice on the scalp or eggs attached to hairs that likely are viable (darker in color and located <1cm from the scalp)

• Tx for head lice:
  
  • OTC ______% cream (eg Nix cream rinse, Elimite) (vs permethrin 5% is for scabies). If live lice are still seen 7-10 days after application, a repeat application is recommended.
  • ___ and ___ are the most reliably ovicidal agents and, as a result, a single application often is sufficient.
    
    • For resistant head lice or failed permethrin, one-time topical ivermectin
    • Malathion 0.5% lotion (Ovide) is highly effective (with some egg-killing ability) and is approved for children >6yo.
  • Any person that shared a bed with patient should be treated even if no live lice are found.
  • A child should not be restricted from school or allowed to miss school time because of lice

Answer 78

Lice
- Dx: Finding live lice on the scalp or eggs attached to hairs that likely are viable (darker in color and located <1cm from the scalp)

• Tx for head lice:
  
  • OTC Permethrin 1% cream (eg Nix cream rinse, Elimite) (vs permethrin 5% is for scabies). If live lice are still seen 7-10 days after application, a repeat application is recommended.
  • Malathion and ivermectin are the most reliably ovicidal agents and, as a result, a single application often is sufficient.
    
    • For resistant head lice or failed permethrin, one-time topical ivermectin
    • Malathion 0.5% lotion (Ovide) is highly effective (with some egg-killing ability) and is approved for children >6yo.
  • Any person that shared a bed with patient should be treated even if no live lice are found.
  • A child should not be restricted from school or allowed to miss school time because of lice

Question 79

Pediculosis Pubis (Pthirus pubis / Pubic louse, crab louse, “crabs”)
- Almost always transmitted by sexual contact (although may occur through unwashed towels, linens, or clothing). In young children, it may be a sign of \_\_\_\_.

• Tx:
  
  • ______% or 5% cream rinse applied to affected areas and washed off after 10 mins, OR pyrethrins + piperonyl butoxide applied to affected areas and washed off after 10 mins
  • 2nd line is Oral ____.
    
    • Cases resistant to all forms of topical therapy are tx with ivermectin 200-400 ug/kg PO in 1 dose
  • Repeat application of either agent is recommended in 7-10 days after treatment
  • Patients should be screened for other sexually transmitted diseases
  • All bedding, towels, and clothing should be washed and dried using heat, or dry cleaned, or contained in air-tight bags for 2 weeks.
  • Treatment of all sexual partners within the last month is indicated, but treating other household members is NOT indicated unless they are experiencing symptoms

Answer 79

Pediculosis Pubis (Pthirus pubis / Pubic louse, crab louse, “crabs”)
- Almost always transmitted by sexual contact (although may occur through unwashed towels, linens, or clothing). In young children, it may be a sign of sexual abuse.

• Tx:
  
  • Permethrin 1% or 5% cream rinse applied to affected areas and washed off after 10 mins, OR pyrethrins + piperonyl butoxide applied to affected areas and washed off after 10 mins
  • 2nd line is Oral ivermectin.
    
    • Cases resistant to all forms of topical therapy are tx with ivermectin 200-400 ug/kg PO in 1 dose
  • Repeat application of either agent is recommended in 7-10 days after treatment
  • Patients should be screened for other sexually transmitted diseases
  • All bedding, towels, and clothing should be washed and dried using heat, or dry cleaned, or contained in air-tight bags for 2 weeks.
  • Treatment of all sexual partners within the last month is indicated, but treating other household members is NOT indicated unless they are experiencing symptoms

Question 80

SCABIES

• Pt:
  
  • Intensely pruritic, especially at night
  • In adolescents, the sites of predilection include interdigital web spaces, flexor aspects of wrists, extensor surfaces of elbows, waistline, upper medial thighs, abdomen, and buttocks.
  • Infants and younger children often have a more diffuse eruption with papules, vesicles, or pustules that involve the face, palms, and soles.
• Tx:
  
  • _______% cream applied to all areas of the body from neck down (include scalp for infants), washed off after 8-14 hours on skin, repeated in 1 week (total of 2x treatments)
    
    • OR _____ 200 ug/kg PO in 1 dose, repeated in 1-2 weeks
      
      • It is approved in adults with uncomplicated scabies; is not approved in children and is not recommended for use in those <5yo or weight <15kg.
    • In unresponsive cases, consider ______% lotion, but it must be used cautiously (if at all) due to potential _____.
      
      • Apply 1oz of lotion or 30g of cream in a thin layer to all areas of body from neck down and thoroughly wash off after 8 hours. Do not apply after bathing.
      • Do not give lindane to individuals with extensive atopic dermatitis.
  • Rash and itching of scabies can persist for several weeks after treatment. Persistent itching in the absence of new lesions is not an indication for retreatment.
  • Consider retreatment if new lesions appear or if post-tx itching persists >2-4 weeks.
  • Encourage all household members to be treated.
  • Examine and treat sexual partners and close contacts within the last month.

Answer 80

SCABIES

• Pt:
  
  • Intensely pruritic, especially at night
  • In adolescents, the sites of predilection include interdigital web spaces, flexor aspects of wrists, extensor surfaces of elbows, waistline, upper medial thighs, abdomen, and buttocks.
  • Infants and younger children often have a more diffuse eruption with papules, vesicles, or pustules that involve the face, palms, and soles.
• Tx:
  
  • Permethrin 5% cream applied to all areas of the body from neck down (include scalp for infants), washed off after 8-14 hours on skin, repeated in 1 week (total of 2x treatments)
    
    • OR Ivermectin 200 ug/kg PO in 1 dose, repeated in 1-2 weeks
      
      • It is approved in adults with uncomplicated scabies; is not approved in children and is not recommended for use in those <5yo or weight <15kg.
    • In unresponsive cases, consider lindane 1% lotion, but it must be used cautiously (if at all) due to potential neurotoxicity/seizures.
      
      • Apply 1oz of lotion or 30g of cream in a thin layer to all areas of body from neck down and thoroughly wash off after 8 hours. Do not apply after bathing.
      • Do not give lindane to individuals with extensive atopic dermatitis.
  • Rash and itching of scabies can persist for several weeks after treatment. Persistent itching in the absence of new lesions is not an indication for retreatment.
  • Consider retreatment if new lesions appear or if post-tx itching persists >2-4 weeks.
  • Encourage all household members to be treated.
  • Examine and treat sexual partners and close contacts within the last month.

Question 81

Pemphigus vulgaris
- Path: Mediated by autoantibodies to desmogleins, which are epidermal proteins. Autoimmune disease caused by antibodies against both _____ and _____.

• Pt:
  
  • Flaccid bullae that erode, leavine denuded skin.
  • ____ involvement is common and often is the 1st manifestation of this serious disorder.
  • ____ sign is lateral displacement of the epidermis when tangential pressure is applied due to lack of cohesion bw cells.
• Tx: High-dose _____

Answer 81

Pemphigus vulgaris
- Path: Mediated by autoantibodies to desmogleins, which are epidermal proteins. Autoimmune disease caused by antibodies against both desmoglein 1 and desmoglein 3.

• Pt:
  
  • Flaccid bullae that erode, leavine denuded skin.
  • Oral mucosal involvement is common and often is the 1st manifestation of this serious disorder.
  • Nikolsky sign is lateral displacement of the epidermis when tangential pressure is applied due to lack of cohesion bw cells.
• Tx: High-dose glucocorticoids

Question 82

Bullous pemphigoid

• Classically seen in older adults
• Autoimmune: IgG against _______, separation of epidermis from basement membrane
• Lesions initially appear similar to hives, and then tense bullae erupt. It is key to note that these bullae are tense and do not rupture as easily as bullae in epidermolysis bullosa.
• Nikosky negative
• Rarely affect oral mucosa, which differentiates from pemphigus vulgarisms

Answer 82

Bullous pemphigoid

• Classically seen in older adults
• Autoimmune: IgG against hemidesmosomes’ dystonin, separation of epidermis from basement membrane
• Lesions initially appear similar to hives, and then tense bullae erupt. It is key to note that these bullae are tense and do not rupture as easily as bullae in epidermolysis bullosa.
• Nikosky negative
• Rarely affect oral mucosa, which differentiates from pemphigus vulgarisms

Question 83

Papular Urticaria

• Path: Hypersensitivity reaction to bites from insects, esp fleas, mosquitoes, mites, and bedbugs that leads to recurrent and sometimes chronic itchy papules on exposed areas of skin (eg arms, LEs, upper back, scalp).
• Tx:
  
  • Eventually resolves spontaneously, but typical corticosteroids and antihistamines are helpful.

Answer 83

Papular Urticaria

• Path: Hypersensitivity reaction to bites from insects, esp fleas, mosquitoes, mites, and bedbugs that leads to recurrent and sometimes chronic itchy papules on exposed areas of skin (eg arms, LEs, upper back, scalp).
• Tx:
  
  • Eventually resolves spontaneously, but typical corticosteroids and antihistamines are helpful.

Question 84

Mastocytosis - Characterized by abnormal proliferation of mast cells, thus resulting in the abnormal release of mast cell mediators. Mastocytosis affects males and females equally, occurs in all races (more commonly in whites), and typically occurs before age 2 years, although 10% of pts develop symptoms 2-15 years.
- Skin condition in which mast cells infiltrate the skin.

• Dx:
  
  • ______ level - Because is a by-product of mast cells, levels are elevated (>20 ng/mL) in all types of mastocytosis.
• Tx:
  
  • ______ most asymptomatic, lesions resolve spontaneously
  • Avoid triggers. Stay away from cold, heat, friction, alcohol, medications that trigger mast cell degranulation (aspirin, NSAIDs, opiates, vancomycin, succinylcholine, certain anesthetic agents, iodinated radiocontrast media).
  • In patients with cutaneous mastocytosis, _______ has been shown to improve symptoms, as have intralesional injections of steroids.

Answer 84

Mastocytosis - Characterized by abnormal proliferation of mast cells, thus resulting in the abnormal release of mast cell mediators. Mastocytosis affects males and females equally, occurs in all races (more commonly in whites), and typically occurs before age 2 years, although 10% of pts develop symptoms 2-15 years.
- Skin condition in which mast cells infiltrate the skin.

• Dx:
  
  • Tryptase level - Because tryptase is a by-product of mast cells, tryptase levels are elevated (>20 ng/mL) in all types of mastocytosis.
• Tx:
  
  • No treatment, most asymptomatic, lesions resolve spontaneously
  • Avoid triggers. Stay away from cold, heat, friction, alcohol, medications that trigger mast cell degranulation (aspirin, NSAIDs, opiates, vancomycin, succinylcholine, certain anesthetic agents, iodinated radiocontrast media).
  • In patients with cutaneous mastocytosis, psoralen plus ultraviolet A light therapy (PUVA) has been shown to improve symptoms, as have intralesional injections of steroids.

Question 85

• Systemic mastocytosis
  
  • There is a systemic form of mastocytosis that involves infiltration of the bone marrow, liver, spleen, and GI tract. These patients do very poorly.
  • Path: Increased mast cells in extracutaneous sites - usually the bone marrow
  • Pt: Systemic manifestations, including abdominal symptoms, flushing, and fatigue, with or without signs of cutaneous mastocytosis.
  • 1) Indolent systemic mastocytosis (ISM)
  • 2) Systemic mastocytosis with an associated hematologic neoplasm (SM-AHN)
  • 3) Aggressive systemic mastocytosis (ASM)
  • 4) Mast cell leukemia (MCL)

Answer 85

• Systemic mastocytosis
  
  • There is a systemic form of mastocytosis that involves infiltration of the bone marrow, liver, spleen, and GI tract. These patients do very poorly.
  • Path: Increased mast cells in extracutaneous sites - usually the bone marrow
  • Pt: Systemic manifestations, including abdominal symptoms, flushing, and fatigue, with or without signs of cutaneous mastocytosis.
  • 1) Indolent systemic mastocytosis (ISM)
  • 2) Systemic mastocytosis with an associated hematologic neoplasm (SM-AHN)
  • 3) Aggressive systemic mastocytosis (ASM)
  • 4) Mast cell leukemia (MCL)

Question 86

• Cutaneous mastocytosis
  
  • Path: Dermal cellular infiltrate characterized by accumulation of mast cells
    
    • 1) Solitary Mastocytomas (A): Solitary or few in number. Yellow to orange-brown papules or plaques.
    • 2) Urticaria pigmentosa (B) / Maculopapular cutaneous mastocytosis (MPCM): Most common form of cutaneous mastocytosis. Multiple tan-brown macules or thin plaques
    • 3) Diffuse cutaneous mastocytosis (DCM)
  • Pt: Orange-brown macules or papules (orange-peel/ peau d’orange) that, upon stroking, develop erythema and swelling
    
    • ______ sign: Localized urticaria, erythema, bullae after rubbing/scratching/stroking the skin or skin lesions that are the result of mast cell degranulation
  • Dx: Appearance and presence of Darier sign.

Answer 86

• Cutaneous mastocytosis
  
  • Path: Dermal cellular infiltrate characterized by accumulation of mast cells
    
    • 1) Solitary Mastocytomas (A): Solitary or few in number. Yellow to orange-brown papules or plaques.
    • 2) Urticaria pigmentosa (B) / Maculopapular cutaneous mastocytosis (MPCM): Most common form of cutaneous mastocytosis. Multiple tan-brown macules or thin plaques
    • 3) Diffuse cutaneous mastocytosis (DCM)
  • Pt: Orange-brown macules or papules (orange-peel/ peau d’orange) that, upon stroking, develop erythema and swelling
    
    • Darier sign: Localized urticaria, erythema, bullae after rubbing/scratching/stroking the skin or skin lesions that are the result of mast cell degranulation
  • Dx: Appearance and presence of Darier sign.

Question 87

Epidermolysis bullosa for neonate presenting with blistering lesions or skin erosions without other etiology

• _____ inheritance.
• Classification:
  
  • 1) EB simplex (EBS): Separation at intraepidermal level. Healing occurs without scarring
    
    • AD.
    • Fragility within the basal keratinocytes (deepest layer of the epidermis).
  • 2) Junctional EB (JEB): Separation at the intra-lamina lucida of dermal-epidermal junction; leads to atrophic scarring
    
    • AR.
    • Defects in basement membrane proteins
    • Death by sepsis is common at <6 months of age.
  • 3) Dystrophic EB (DEB): Separation at the sub-basal lamina level
    
    • AR or AD.
    • Pt: The more severe recessive type of DEB (RDEB) results in generalized blistering with extensive scarring and risk of aggressive squamous cell carcinoma
• Pt: Neonate presenting with blistering lesions or skin erosions without other etiology
  
  • Skin fragility and blister formation after minor skin trauma
  • Malnutrition is a common complication
• Dx: ______ most likely to confirm
• Tx:
  
  • _______ Topical antiseptics/antibiotic (with oral antibiotics for full-blown infections), pain medications (eg NSAIDs, opioids), and nutritional support (for severe forms).
  • Avoid using tape or other adhesives on the skin of pts with EB as this induces blistering.

Answer 87

Epidermolysis bullosa for neonate presenting with blistering lesions or skin erosions without other etiology

• AD inheritance.
• Classification:
  
  • 1) EB simplex (EBS): Separation at intraepidermal level. Healing occurs without scarring
    
    • AD.
    • Fragility within the basal keratinocytes (deepest layer of the epidermis).
  • 2) Junctional EB (JEB): Separation at the intra-lamina lucida of dermal-epidermal junction; leads to atrophic scarring
    
    • AR.
    • Defects in basement membrane proteins
    • Death by sepsis is common at <6 months of age.
  • 3) Dystrophic EB (DEB): Separation at the sub-basal lamina level
    
    • AR or AD.
    • Pt: The more severe recessive type of DEB (RDEB) results in generalized blistering with extensive scarring and risk of aggressive squamous cell carcinoma
• Pt: Neonate presenting with blistering lesions or skin erosions without other etiology
  
  • Skin fragility and blister formation after minor skin trauma
  • Malnutrition is a common complication
• Dx: Skin punch biopsy most likely to confirm
• Tx:
  
  • Supportive care. Topical antiseptics/antibiotic (with oral antibiotics for full-blown infections), pain medications (eg NSAIDs, opioids), and nutritional support (for severe forms).
  • Avoid using tape or other adhesives on the skin of pts with EB as this induces blistering.

Question 88

Incontinentia Pigmenti (IP)
- \_\_\_\_\_Inheritance? Disorder that is usually lethal in males; there may be maternal hx of recurrent spontaneous abortions. Most cases in \_\_\_\_\_

• Pt: Often presents as a rash on an otherwise healthy baby girl. Affects the skin, hair, teeth, nails, eyes, and CNS.
  
  • 4 progressive and sometimes overlapping stages of skin manifestations:
    
    • Stage 1) ____: Pink papulovesicles arising along the ______
      
      • Erythematous Vesicles and papules in a linear arrangement on extremities. Vesicles are present at birth or within first 6-8 weeks.
    • Stage 2) ____: Verrucous papules and plaques replace the vesicles after the first 2-6 weeks of life and persist for months. Wart-like rash.
    • Stage 3) ____: Hyperpigmented patches supplant the verrucous plaques at 3-6 months and then persist for years (longest stage)
    • Stage 4) ____: Hypopigmented atrophic streaks and alopecia replace the hyperpigmented patches
  • Delayed eruption of teeth occurs in ⅔ of pts. If affected, the teeth can be______.
  • Strabismus is a common eye association
  • Seizures
  • The alopecia seen in IP is a _____, without potential for hair regrowth
• Tx:
  
  • _______
  • Genetic consultation is essential.
  • Refer all babies with IP to ophthalmology, dental, and neurology.

Answer 88

Incontinentia Pigmenti (IP)
- XLD Disorder that is usually lethal in males; there may be maternal hx of recurrent spontaneous abortions. Most cases in girls

• Pt: Often presents as a rash on an otherwise healthy baby girl. Affects the skin, hair, teeth, nails, eyes, and CNS.
  
  • 4 progressive and sometimes overlapping stages of skin manifestations:
    
    • Stage 1) Bullous: Pink papulovesicles arising along the lines of Blaschko
      
      • Erythematous Vesicles and papules in a linear arrangement on extremities. Vesicles are present at birth or within first 6-8 weeks.
    • Stage 2) Verrucous: Verrucous papules and plaques replace the vesicles after the first 2-6 weeks of life and persist for months. Wart-like rash.
    • Stage 3) Pigmentary: Hyperpigmented patches supplant the verrucous plaques at 3-6 months and then persist for years (longest stage)
    • Stage 4) Atretic: Hypopigmented atrophic streaks and alopecia replace the hyperpigmented patches
  • Delayed eruption of teeth occurs in ⅔ of pts. If affected, the teeth can be peg- or cone-shaped or absent.
  • Strabismus is a common eye association
  • Seizures
  • The alopecia seen in IP is a scarring (cicatricial) alopecia, without potential for hair regrowth
• Tx:
  
  • No treatment.
  • Genetic consultation is essential.
  • Refer all babies with IP to ophthalmology, dental, and neurology.

Question 89

Granuloma annulare

• Relatively common benign disorder.
• Pt:
  
  • Pink annular plaques without scale. Often appears on the distal extremities
  • May be differentiated from tinea corporis by the _____, the firmness of the border, and tendency of lesions to have a violaceous color.
  • May also mimic nummular eczema. However, nummular eczema does not exhibit border elevation or central clearing. In addition, a ____ is present in nummular eczema.
• Tx: _____ Self-limited. Disappear within months-years

Answer 89

Granuloma annulare

• Relatively common benign disorder.
• Pt:
  
  • Pink annular plaques without scale. Often appears on the distal extremities
  • May be differentiated from tinea corporis by the absence of scale, the firmness of the border, and tendency of lesions to have a violaceous color.
  • May also mimic nummular eczema. However, nummular eczema does not exhibit border elevation or central clearing. In addition, a crust is present in nummular eczema.
• Tx: Asymptomatic. Self-limited. Disappear within months-years

Question 90

Pityriasis Rosea
- Path: Acute, self-limited inflammatory disorder. Most likely due to viral etiology, thought to be caused by human herpesviruses 6 and/or 7.

• Pt:
  
  • Begins with herald patch (round erythematous scaling patch with central clearing), although this is only present in about 50% of patients.
  • Within 2 weeks from the appearance of the herald patch (2-21 days), the classic secondary eruption will develop and worsen over the next 2 weeks. The classic lesions are salmon-pink papules/plaques with a delicate scale collarette on the extremities and trunk in distribution along natural skin tension lines giving a ________ appearing.About ¼ of patients have significant pruritus.
    
    • The plaques have scale that is located at the _____ edge of lesions, unlike the leading edge in tinea corporis.
• Rule out: ______ always must be considered as the papulosquamous eruption of secondary syphilis can closely resemble pityriasis. However, secondary syphilis generally presents with red-brown macules on the palms and soles.
• Tx:
  
  • _____ New lesions appear for 2-3 weeks and the eruption resolves in 1- 2 months
  • Symptomatic management with oral hydroxyzine

Answer 90

Pityriasis Rosea
- Path: Acute, self-limited inflammatory disorder. Most likely due to viral etiology, thought to be caused by human herpesviruses 6 and/or 7.

• Pt:
  
  • Begins with herald patch (round erythematous scaling patch with central clearing), although this is only present in about 50% of patients.
  • Within 2 weeks from the appearance of the herald patch (2-21 days), the classic secondary eruption will develop and worsen over the next 2 weeks. The classic lesions are salmon-pink papules/plaques with a delicate scale collarette on the extremities and trunk in distribution along natural skin tension lines giving a Christmas Tree appearing.About ¼ of patients have significant pruritus.
    
    • The plaques have scale that is located at the trailing edge of lesions, unlike the leading edge in tinea corporis.
• Rule out: Secondary syphilis always must be considered as the papulosquamous eruption of secondary syphilis can closely resemble pityriasis. However, secondary syphilis generally presents with red-brown macules on the palms and soles.
• Tx:
  
  • Self-limited. New lesions appear for 2-3 weeks and the eruption resolves in 1- 2 months
  • Symptomatic management with oral hydroxyzine

Question 91

Cold panniculitis (“Popsicle panniculitis” or Haxthausen disease)

• Rash of cold panniculitis is caused by direct contact of the skin or mucosal surfaces to cold, which leads to local trauma.
• Resolves without treatment. Reassure that it will resolve within ___ weeks although hyperpigmentation can last for several months. Cold panniculitis commonly re-occurs with repeated cold exposure, and educating parents on prevention is important.

Answer 91

Cold panniculitis (“Popsicle panniculitis” or Haxthausen disease)

• Rash of cold panniculitis is caused by direct contact of the skin or mucosal surfaces to cold, which leads to local trauma.
• Resolves without treatment. Reassure that it will resolve within 2-3 weeks although hyperpigmentation can last for several months. Cold panniculitis commonly re-occurs with repeated cold exposure, and educating parents on prevention is important.

Question 92

Adenoma Sebaceum

• Skin disorder consisting of angiofibromas that start in childhood
• Initially often confused with _____
• Pt: Classically, the lesions begin appearing between 2-5yo of life, as red papules on the face, classically in nasolabial folds, cheeks, and on the chin.
• Tx: Cosmetic only; done either by ______. Surgical removal is also possible.

Answer 92

Adenoma Sebaceum

• Skin disorder consisting of angiofibromas that start in childhood
• Initially often confused with acne
• Pt: Classically, the lesions begin appearing between 2-5yo of life, as red papules on the face, classically in nasolabial folds, cheeks, and on the chin.
• Tx: Cosmetic only; done either by argon or pulse dye laser. Surgical removal is also possible.