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BASIC Exam Study Guide > Pediatric skin diseases management > Flashcards

Pediatric skin diseases management Flashcards

Plans for evaluation and basic management

1
Q

Name the diagnosis.

  • What is notable about its distribution?
  • Where does it typically occur?
  • What can occur when it involves the digit?
  • How can you treat it?
  • What is the disease course?
A

Lichen striatus

  • Typically asymptomatic 2-4 mm pink or hypopigmented scaly papules in linear/Blachkoid distribution
  • Extremities >> face, trunk, buttocks
  • Nail dystrophy can occur
  • Treat with TCS and TCI
  • Resolves spontaneously within 3-24 months
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2
Q

Name the diagnosis.

  • What is notable about its distribution?
  • Where does it typically occur?
  • What can occur when it involves the digit?
  • How can you treat it?
  • What is the disease course?
A

Lichen striatus

  • Typically asymptomatic 2-4 mm pink or hypopigmented scaly papules in linear/Blachkoid distribution
  • Extremities >> face, trunk, buttocks
  • Nail dystrophy can occur
  • Treat with TCS and TCI
  • Resolves spontaneously within 3-24 months
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3
Q

Name the diagnosis.

  • What is notable about its distribution?
  • Where does it typically occur?
  • What can occur when it involves the digit?
  • How can you treat it?
  • What is the disease course?
A

Lichen striatus

  • Typically asymptomatic 2-4 mm pink or hypopigmented scaly papules in linear/Blachkoid distribution
  • Extremities >> face, trunk, buttocks
  • Nail dystrophy can occur
  • Treat with TCS and TCI
  • Resolves spontaneously within 3-24 months
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4
Q

How would you treat localized (1), widespread (5) and complicated impetigo (1) ?

A
  1. Localized: topical Mupirocin (or retapamulin)
  2. Widespread: oral beta-lactamase resistant PCN (e.g., dicloxacillin, oxacillin, Augmentin) or first generation CSN (e.g., cephalexin or cefazolin) or clindamycin
  3. Complicated: IV ceftriaxone
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5
Q

Name the diagnosis.

A

Impetigo

  • Look for honey-colored crusting
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6
Q

Name the diagnosis.

A

Impetigo

  • Look for honey-colored crusting
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7
Q

Name the diagnosis.

  • What usually occurs first?
  • Where does the rash begin? (2)
  • What happens within 48 hours?
  • What happens for the next 1 week?
A

Staphylococcal scalded skin syndrome

  • Febrile prodrome followed by widespread skin tenderness
  • Eruption begins on face (periorifical radial fissuring) and intertriginous zones
  • Generalizes within 48 hours as wrinkled-appearing skin with flaccid bullae and (+) Nikolsky sign
  • Desquamation for 1 week; heals without scarring.
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8
Q

Name the diagnosis.

  • What usually occurs first?
  • Where does the rash begin? (2)
  • What happens within 48 hours?
  • What happens for the next 1 week?
A

Staphylococcal scalded skin syndrome

  • Febrile prodrome followed by widespread skin tenderness
  • Eruption begins on face (periorifical radial fissuring) and intertriginous zones
  • Generalizes within 48 hours as wrinkled-appearing skin with flaccid bullae and (+) Nikolsky sign
  • Desquamation for 1 week; heals without scarring.
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9
Q

What is the classic clinical course of Staphylococcus scalded skin syndrome (SSSS)?

  • What usually occurs first?
  • Where does the rash begin? (2)
  • What happens within 48 hours?
  • What happens for the next 1 week?
A
  • Presents with febrile prodrome and widespread skin tenderness
  • Skin eruption begins on face with periorificial radial fissuring and intertriginous areas
  • Within 48 hours, the rash generalizes and there is wrinkled-appearing skin with flaccid bullae and (+) Nikolsky sign
  • Desquamation for up to 1 week, then heals without scarring
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10
Q

Which desmoglein is targeted in Staphylococcus scalded skin syndrome?

  • What toxins are involved?
  • What is the histology similar to?
  • The same toxins are involved in what other condition?
A
  • Exfoliatoxins A and B (ETA and ETB) lead to widespread cleavage of Dsg1
  • Leads to subcorneal/intragranular acantholysis
  • Histology therefore resembles pemphigus foliaceus
  • ETA and ETB are the same exfoliatoxins as bullous impetigo, but are disseminated in the bloodstream in SSSS
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11
Q

How do you treat staph scalded skin syndrome?

  • Mild disease? (2)
  • Severe disease?
A
  • Mild disease: Beta-lactamase resistant PCN (dicloxacillin) or 1st generation CSN (cephalexin)
  • Severe disease: Hospitalization and IV antibiotics
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12
Q

How do you treat scabies in infants, adults and the elderly?

What drug should be avoided in infants, young children and pregnant or breastfeeding women?

A
  • Permethrin cream twice 7 days apart
  • Apply permethrin to head/scalp in infants and elderly, but okay to defer this in others
  • Avoid oral ivermectin in infants, young children and pregnant or breastfeeding women
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13
Q

Name the diagnosis.

  • Treatment?
  • What condition is this associated with?
A

Pityriasis alba

  • Hypopigmented macules and patches with slight scale in patients with atopic dermatitis
  • Responds to emollient and low potency TCS
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14
Q

Name the diagnosis.

  • Treatment?
  • What condition is this associated with?
A

Pityriasis alba

  • Form of subclinical dermatitis
  • Poorly marginated, hypopigmented slightly scaly patches
  • Usually responds to emollients and mild steroids
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15
Q

What vitamin deficiency is related to acrodermatitis enteropathica?

A

Zinc

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16
Q

Name the diagnosis.

A

Trichotillomania

  • Refer to psychiatry for cognitive behavioral therapy (CBT)
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17
Q

What is the classic histopathology of acrodermatitis enteropathica?

  • What happens to keratinocytes?
A
  • Cytoplasmic pallor of keratinocytes in upper epidermis with ballooning and reticular degeneration
  • Necrosis of keratinocytes
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18
Q

Name the diagnosis.

  • What areas are involved with rash?
  • What are other symptoms in the “triad”?
A

Acrodermatitis enteropathica

  • Triad of erosive vesiculopustular eczematous lesions involving the diaper area, face (periorificial) and acral areas, along with diarrhea and alopecia
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19
Q

Name the diagnosis.

  • What areas are involved with rash?
  • What are other symptoms in the “triad”?
A

Acrodermatitis enteropathica

  • Triad of erosive vesiculopustular eczematous lesions involving the diaper area, face (periorificial) and acral areas, along with diarrhea and alopecia
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20
Q

Name the diagnosis.

  • What areas are involved with rash?
  • What are other symptoms in the “triad”?
A

Acrodermatitis enteropathica

  • Triad of erosive vesiculopustular eczematous lesions involving the diaper area, face (periorificial) and acral areas, along with diarrhea and alopecia
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21
Q

What are the laboratory abnormalities seen in acrodermatitis enteropathica? (2)

A
  • Decreased serum zinc
  • Decreased serum alkaline phosphatase
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22
Q

What is the treatment for acrodermatitis enteropathica? (1)

A
  • Life-long (in congenital cases) zinc sulfate supplementation leads to fast resolution
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23
Q

What is the gene responsible for primary acrodermatitis enteropathica and what is its function?

  • What does this encode?
A

Mutations in SLC39A4 (encodes intestinal zinc-specific transporter ZIP4)

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24
Q

What amino acid is not absorbed properly in Hartnup disease?

  • What symptoms can this lead to?
  • What CNS problems can occur?
  • What is the treatment for this? What should be avoided?
A

Tryptophan

  • Leads to pellagra-like symptoms (e.g., photosensitivity)
  • Acute photodermatitis with erythema, blistering, scaling, crusting, scarring of sun-exposed areas
  • May develop cerebellar ataxia, seizures, intellectual disability, psychosis
  • Treatment: avoid sunlight and take oral nicotinamide
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25
Name the diagnosis. * What is its expected clinical course?
**Juvenile xanthogranuloma** * **Pink to yellow papule** more common on the **head/neck** of a child * Spontaneous resolution in 3-6 years
26
Name the diagnosis. * What is its expected clinical course?
**Juvenile xanthogranuloma** * **Pink to yellow papule** more common on the **head/neck** of a child * Spontaneous resolution in 3-6 years
27
Name the diagnosis. * What is its expected clinical course?
**Juvenile xanthogranuloma** * **Pink to yellow papule** more common on the **head/neck** of a child * Spontaneous resolution in 3-6 years
28
Name the diagnosis. * What are features of a poor prognosis? (2) * What might this be mistaken for in infants?
**Langerhans cell histiocytosis (LCH)** * Prognosis determined by systemic involvement * Features predictive of poor prognosis: **BRAF V600E mutation** and failure to respond to treatment within 6 weeks * In infants, it is classically seen in the diaper area and can be mistaken for a diaper dermatitis
29
Name the diagnosis. * What are features of a poor prognosis? (2) * What might this be mistaken for in infants?
**Langerhans cell histiocytosis** * Prognosis determined by systemic involvement * Features predictive of poor prognosis: **BRAF V600E** mutation and **failure to respond to treatment within 6 weeks** * In infants, it is classically seen in the diaper area and can be mistaken for a **diaper dermatitis**
30
Name the diagnosis. * What are features of a poor prognosis? (2) * What might this be mistaken for in infants?
**Langerhans cell histiocytosis** * Prognosis determined by systemic involvement * Features predictive of poor prognosis: **BRAF V600E** mutation and **failure to respond to treatment within 6 weeks** * In infants, it is classically seen in the diaper area and can be mistaken for a **diaper dermatitis**
31
Name the diagnosis. * What is the expected clinical course of this? * What "sign" is positive? * Is dermatographism positive or negative? * How do you treat this? (2)
**Solitary mastocytoma** * Usually occurs as a single tan/yellow-tan plaque or nodule * Most commonly seen on distal extremities * Generally self-resolves within 1-3 years * **Positive Darier's sign**: rub skin of lesion for 10 seconds and becomes red, swollen and/or blisters within 5 minutes * Usually dermatographism negative * Treat with **H1 antagonists (diphenhydramine or cetirizine)** and **H2 blockers (ranitidine or cimetidine) for GI symptoms**
32
Name the diagnosis. * What is the expected clinical course of this? * What "sign" is positive? * Is dermatographism positive or negative? * How do you treat this? (2)
**Solitary mastocytoma** * Usually occurs as a single tan/yellow-tan plaque or nodule * Most commonly seen on distal extremities * Generally self-resolves within 1-3 years * **Positive Darier's sign**: rub skin of lesion for 10 seconds and becomes red, swollen and/or blisters within 5 minutes * Usually dermatographism negative * Treat with **H1 antagonists (diphenhydramine or cetirizine)** and **H2 blockers (ranitidine or cimetidine) for GI symptoms**
33
Name the diagnosis. * What is the expected clinical course of this? * What "sign" is positive? * Is dermatographism positive or negative? * How do you treat this? (2)
**Solitary mastocytoma** * Usually occurs as a single tan/yellow-tan plaque or nodule * Most commonly seen on distal extremities * Generally self-resolves within 1-3 years * **Positive Darier's sign**: rub skin of lesion for 10 seconds and becomes red, swollen and/or blisters within 5 minutes * Usually dermatographism negative * Treat with **H1 antagonists (diphenhydramine or cetirizine)** and **H2 blockers (ranitidine or cimetidine) for GI symptoms**
34
Name the diagnosis. * This is the most common presentation of what disorder in children? * What are the associated symptoms?
**Urticaria pigmentosa** * **Most common presentation of cutaneous mastocytosis in children** * Multiple light brown to red-brown macules and papules which can occur anywhere * Starts on trunk, spare palms/soles/face * Can have pruritus, flushing, blistering
35
Name the diagnosis. * This is the most common presentation of what disorder in children? * What are the associated symptoms?
**Urticaria pigmentosa** * **Most common presentation of cutaneous mastocytosis in children** * Multiple light brown to red-brown macules and papules which can occur anywhere * Starts on trunk, spare palms/soles/face * Can have pruritus, flushing, blistering
36
Name the diagnosis. * This is the most common presentation of what disorder in children? * What are the associated symptoms?
**Urticaria pigmentosa** * **Most common presentation of cutaneous mastocytosis in children** * Multiple light brown to red-brown macules and papules which can occur anywhere * Starts on trunk, spare palms/soles/face * Can have pruritus, flushing, blistering
37
What mutation is found in **cutaneous mastocytosis**? * What does this encode that is essential for what cell type?
**c-KIT** * Encodes **KIT** on mast cells which is essential for mast cell survival * D816V activating mutation found in 42% of patients
38
What are the most common systemic symptoms seen in **urticaria pigmentosa**? (4) * How do systemic symptoms relate to how many lesions are present?
* Diarrhea * Abdominal pain * Wheezing * Dyspnea * **Patients with more lesions are more likely to have systemic symptoms.**
39
Name the diagnosis. * How would you describe these lesions? * What systemic effects can occur and why?
**Diffuse cutaneous mastocytosis** * Infiltrated, red-brown, leathery plaques with peau d'orange appearance that can involve large areas of the body * Skin lesions can blister, leading to erosions * Can also have systemic effects (e.g., GI upset, failure to thrive) because of mast cell release
40
Describe **Darier's sign**. * What disorders is this present in?
* Local erythema or urticarial wheal after friction or rubbing * Present in all forms of mastocytosis
41
Describe the histology of **mastocytosis** and **what stains would be positive?** * What occurs in the dermis? * What may occur in the basal layer?
* Mast cell infiltrates in the dermis * Eosinophils and hyperpigmentation of the basal layer may be present * Stains: **toluidine blue, Giemsa, Leder, tryptase and CD117 (kit) antibodies**
42
What lab value should you check when evaluating for **mastocytosis?** * What urine study may also be obtained?
**Tryptase level** * **Serum tryptase may be elevated** but is often normal * Urinary histamine and metabolites (1,4 methylimidazole acetic acid and N-methylimidazoleacetic acid) may be detectable
43
What are the general treatments for **cutaneous mastocytoses**? * What should be avoided? * What medications can be given? * What medication could be given in those who have a specific, notable gene rearrangement?
* **Avoid mast cell degranulators** (e.g., alcohol, anticholinergics, NSAIDs, aspirin, narcotics) * **H1 and H2 blockers** (e.g., diphenhydramine/cetirizine and cimetidine/ranitidine, respectively) * **Imatinib** can be used in those with **systemic mastocytosis and the FIP1L1-PDGFRA gene rearrangement**
44
Name the diagnosis. * What is this derived from? * What may be deposited? What special "sign" may be present?
**Pilomatricoma** * Hair follicle tumor derived from hair matrix cells * Calcification within lesion makes it hard, bony and often angulated in shape (called the "tent" sign) * Blue, red or flesh colored * Most are less than 1 cm * Commonly found on the face, scalp or upper extremities
45
Name the diagnosis. * What is this derived from? * What may be deposited? What special "sign" may be present?
**Pilomatricoma** * Hair follicle tumor derived from hair matrix cells * Calcification within lesion makes it hard, bony and often angulated in shape (called the "tent" sign) * Blue, red or flesh colored * Most are less than 1 cm * Commonly found on the face, scalp or upper extremities
46
Name the diagnosis. * What is this derived from? * What may be deposited? What special "sign" may be present?
**Pilomatricoma** * Hair follicle tumor derived from hair matrix cells * Calcification within lesion makes it hard, bony and often angulated in shape (called the "tent" sign) * Blue, red or flesh colored * Most are less than 1 cm * Commonly found on the face, scalp or upper extremities
47
Name the diagnosis. * What is this derived from? * What may be deposited? What special "sign" may be present?
**Pilomatricoma** * Hair follicle tumor derived from hair matrix cells * Calcification within lesion makes it hard, bony and often angulated in shape (called the "tent" sign) * Blue, red or flesh colored * Most are less than 1 cm * Commonly found on the face, scalp or upper extremities
48
What is the classic histopathology of **pilomatricoma**? * What is the derm path *buzz word*? * What special cells are present? * What type of inflammation may occur? * What may be deposited?
* Complex cystic proliferation with internal **"*rolls and scrolls*"** appearance * **Matrical (basaloid) cells** with abrupt transition to **anucleate "shadow/ghost cells" (eosinophilic)** * Keratin production leads to **granulomatous inflammation and calcification**
49
What are the mutations associated with **tuberous sclerosis**? (2) * What is the inheritance pattern?
**Mutations in hamartin (TSC1)** or **tuberin (TSC2**) * **Autosomal dominant** inheritance * Tuberin and hamartin form a complex that inhibits signal transduction of downstream effectors of **mTOR** * Leads to abnormal regulation of cellular differentiation, proliferation, and migration
50
List the five cutaneous findings of **tuberous sclerosis**. * What is the first cutaneous finding?
1. **Adenoma sebaceum** (facial angiofibromas) 2. **Hypopigmented "ash-leaf" macules** (first cutaneous finding; basically inverse cafe au lait macules) 3. **Shagreen patch** (connective tissue nevus) 4. **Periungual fibromas** ("Koenen tumors") 5. **Cafe-au-lait macules** (CALM)
51
What is the treatment for **facial angiofibromas (adenoma sebaceum)** in **tuberous sclerosis**? (4) * Think about (1) topical medication, as well as (3) different procedures.
* Topical rapamycin (mTOR inhibitor) * PDL * Ablative laser * Excision
52
What are the **neurologic** findings in **tuberous sclerosis**? (5) * What is the #1 cause of mortality?
1. **Cortical tubers (hamartomas)** 2. **Infantile spasms/seizures** (#1 cause of mortality) 3. **Paraventricular calcification** 4. Subependymal nodules (may lead to hydrocephalus) 5. Subependymal giant cell astrocytomas
53
Name the diagnosis. * What syndrome does this suggest?
**Tuberous sclerosis** (Periungual fibromas, a.k.a. **"Koenen tumors"**)
54
Name the diagnosis. * What syndrome does this suggest?
**Tuberous sclerosis** | (Hypopigmented "ash-leaf" macules)
55
Name the diagnosis. * What syndrome does this suggest?
**Tuberous sclerosis** | (Facial angiofibromas)
56
What are other findings of **tuberous sclerosis** of the eyes, heart and kidney? (3)
* Kidney: angiomyolipomas * Eye: retinal phakomas (hamartomas) * Heart: cardiac rhabdomyomas
57
What is the order by which the cutaneous findings of **tuberous sclerosis** occurs? * What is the first finding that occurs as early as infancy? * What before puberty? * What happens in adolescence?
* Infancy to early childhood = "ash-leaf" macules * Prepubertal = angiofibromas, Shagreen patch * Adolescence = ungual fibromas
58
What is the **inheritance pattern** and **genetic mutation** of **incontinentia pigmenti**? * In whom is this a lethal mutation? * What leads to the Blaschkoid pattern on the skin?
* **X-linked dominant** * **Mutation in *NEMO*** prevents activation of NF-κB, which regulates cell proliferation/apoptosis * **Mutation is lethal in males** * **Functional mosaicism** leads to Blaschkoid pattern on skin
59
Name the diagnosis.
**Incontinentia pigmenti**
60
Name the diagnosis.
**Incontinentia pigmenti**
61
Name the diagnosis.
**Incontinentia pigmenti**
62
What are the four distinct morphologic stages of **incontinentia pigmenti**?
63
Name the diagnosis. * When is its onset? * What different morphologies may be present? * What is typically spared? * What is the expected clinical course?
**Erythema toxicum neonatorum** * **Usually presents at 24-48 hours** * Erythematous macules, papules, pustules and wheals * May occur anywhere except palms and soles * Self-limited and resolves over several weeks
64
Name the diagnosis. * When is its onset? * What different morphologies may be present? * What is typically spared? * What is the expected clinical course?
**Erythema toxicum neonatorum** * **Usually presents at 24-48 hours** * Erythematous macules, papules, pustules, and wheals * May occur anywhere except palms and soles * Self-limited and resolves over several weeks
65
Name the diagnosis. * When is its onset? * What different morphologies may be present? * What is typically spared? * What is the expected clinical course?
**Erythema toxicum neonatorum** * **Usually presents at 24-48 hours** * Erythematous macules, papules, pustules, and wheals * May occur anywhere except palms and soles * Self-limited and resolves over several weeks
66
What is included in the mnemonic for **HAAPPIE for eosinophilic spongiosis**?
67
What do you see on histology and Wright's stain for **erythema toxicum neonatorum**? * What occurs in the epidermis and follicles? * What cell type occurs in Wright's stain of pustule fluid?
* H&E: subcorneal and intrafollicular eosinophilic pustules * Wright's stain of pustule fluid: **eosinophils**
68
What is the classic histopathology and Wright's stain of pustule fluid of **transient neonatal pustular melanosis**? * What occurs in the epidermis? * What cell type occurs in Wright's stain of pustule fluid?
* Subcorneal pustules with neutrophils, fibrin and _rarely_ eosinophils * Wright's stain: neutrophils
69
Name the diagnosis. * In whom is this most common? * What are the three stages of this? * When is its onset? * What is the expected clinical course?
**Transient neonatal pustular melanosis** * Term infants, more common in **blacks** * Three stages: * **1. Pustules without underlying erythema** * **2.** **_Collarettes of scale_** * **3. Hyperpigmented macules** * **​Present at birth or shortly after** * Self-limited and resolves over weeks
70
Name the diagnosis. * In whom is this most common? * What are the three stages of this? * When is its onset? * What is the expected clinical course?
**Transient neonatal pustular melanosis** * Term infants, more common in **blacks** * Three stages: * **1. Pustules without underlying erythema** * **2.** **_Collarettes of scale_** * **3. Hyperpigmented macules** * **​Present at birth or shortly after** * Self-limited, resolves over weeks
71
Name the diagnosis. * In whom is this most common? * What are the three stages of this? * When is its onset? * What is the expected clinical course?
**Transient neonatal pustular melanosis** * Term infants, more common in **blacks** * Three stages: * **1. Pustules without underlying erythema** * **2. _Collarettes of scale_** * **3. Hyperpigmented macules** * **​**Present at birth or shortly after * Self-limited, resolves over weeks
72
What is the onset of **neonatal acne (benign cephalic pustulosis)** compared with **infantile acne**?
* **Neonatal acne: 2 weeks old to 3 months old** * **Infantile acne: 3 months to 2 years**
73
Describe **neonatal cephalic pustulosis.** * What is it caused by? * When is its onset? * What is its expected clinical course? * How do you treat it? * What hormone is it thought to be associated with? What glands are stimulated by it?
* Type of **neonatal acne** caused by **Malassezia** * Appears within first few weeks of life and resolves by 3 months * Treat with **ketoconazole cream** * Can also be related to sebaceous gland stimulation by maternal androgens or transient androgen production
74
Name the diagnosis. * What areas are most commonly involved?
**Nevus simplex ("salmon patch")** * Benign transient vascular ectasia of capillary bed * **Ill-defined pink** to light-red blanchable macules on glabella, eyelids and occiput most commonly * Most **resolve over few months to years** * If persist, can laser if desired
75
Name the diagnosis. * What areas are most commonly involved?
**Nevus simplex ("salmon patch")** * Benign transient vascular ectasia of capillary bed * **Ill-defined pink** to light-red blanchable macules on glabella, eyelids and occiput most commonly * Most **resolve over few months to years** * If persist, can laser if desired
76
Name the diagnosis. * Is it GLUT-1 negative or positive? * What syndromes is it associated with? * When is imaging needed?
**Port-wine stain** * **Present at birth** and **does not rapidly enlarge** * **GLUT-1 negative** * Syndromic associations: Maffuci, Klippel-Trenaunay, Sturge-Weber, Blue Rubber Bleb * If on the lower back and crossing the midline, then obtain US/MRI to evaluate for tethered cord
77
Name the diagnosis. * Is it GLUT-1 negative or positive? * What syndromes is it associated with? * When is imaging needed?
**Port-wine stain** * **Present at birth** and **does not rapidly enlarge** * **GLUT-1 negative** * Syndromic associations: Maffuci, Klippel-Trenaunay, Sturge-Weber, Blue Rubber Bleb * If on the lower back and crossing the midline, then obtain US/MRI to evaluate for tethered cord
78
Name the diagnosis. * Is it GLUT-1 negative or positive? * What syndromes is it associated with? * When is imaging needed?
**Port-wine stain** * **Present at birth** and **does not rapidly enlarge** * **GLUT-1 negative** * Syndromic associations: Maffuci, Klippel-Trenaunay, Sturge-Weber, Blue Rubber Bleb * If on the lower back and crossing the midline, then obtain US/MRI to evaluate for tethered cord
79
Name the diagnosis. * What is the classic histology of this? How are melanocytes and melanosomes affected?
**Nevus depigmentosus** * Hypomelanotic patches that are well demarcated with irregular borders that classically **respects the midline and may be segmental** * Histopathology: normal number of melanocytes with **fewer melanosomes** in melanocytes and keratinocytes * **Segmental pigmentation disorder** is a variant with a checkerboard pattern of hypopigmentation/hyperpigmentation (see photo)
80
Name the diagnosis. * What is the classic histology of this? How are melanocytes and melanosomes affected?
**Nevus depigmentosus** * Hypomelanotic patches that are well demarcated with irregular borders that classically **respects the midline and may be segmental** * Histopathology: normal number of melanocytes with **fewer melanosomes** in melanocytes and keratinocytes * **Segmental pigmentation disorder** is a variant with a checkerboard pattern of hypopigmentation/hyperpigmentation
81
Name the diagnosis.
**Dermal melanocytosis** (formerly known as a Mongolian spot) * **Slate blue, grey,** or black patches * Most common over **buttocks and sacrum** * Treatment usually not recommended, but if decided can use **Q-switched laser** * **Sacral lesions tend to fade/disappear on their own**
82
Name the diagnosis.
**Dermal melanocytosis** (formerly known as Mongolian spot) * **Slate blue, grey,** or black patches * Most common over **buttocks and sacrum** * Treatment usually not recommended, but if desided can use **Q-switched lasers** * **Sacral lesions tend to fade/disappear on their own**
83
Name the diagnosis. * Where is this usually found?
**Nevus of Ito** * Unilateral dermal melanocytosis that presents as hyperpigmented/blue confluent and/or mottled **patch on side of neck and shoulder**
84
Name the diagnosis. * Where is this usually found?
**Nevus of Ito** * Unilateral dermal melanocytosis that presents as hyperpigmented/blue confluent and/or mottled **patch on side of neck and shoulder**
85
Name the diagnosis. * Where is this usually found?
**Nevus of Ito** * Unilateral dermal melanocytosis that presents as hyperpigmented/blue confluent and/or mottled **patch on side of neck and shoulder**
86
Name the diagnosis. * What branchial arch was malformed?
**Accessory tragus** * Exophytic papule with or without cartilage * Can occur anywhere from **preauricular region to angle of mouth** * Faulty formation of **first branchial arch** * Treatment: surgical excision
87
Name the diagnosis. * What branchial arch was malformed?
**Accessory tragus** * Exophytic papule with/without cartilage * Can occur anywhere from **preauricular region to angle of mouth** * Faulty formation of **first branchial arch** * Treatment: surgical excision
88
Name the diagnosis. * What nerve dermatome(s) is usually involved?
**Nevus of Ota** * Blue or brown confluent or mottled pigmentation of the upper face and periorbital areas (CN V1/ophthalmic and V2/maxillary nerve distribution) * Can involve the ocular surface and be bilateral
89
Name the diagnosis. * What nerve dermatome(s) is usually involved?
**Nevus of Ota** * Blue or brown confluent or mottled pigmentation of the upper face and periorbital areas (CN V1/ophthalmic and V2/maxillary nerve distribution) * Can involve the ocular surface and be bilateral
90
Name the diagnosis. * What nerve dermatome(s) is usually involved?
**Nevus of Ota** * Blue or brown confluent or mottled pigmentation of the upper face and periorbital areas (CN V1/ophthalmic and V2/maxillary nerve distribution) * Can involve the ocular surface and be bilateral
91
Describe the histological findings of an **epidermal nevus**. (2) * What happens to the epidermis? Corneum?
* Epidermal papillomatosis * Orthohyperkeratosis
92
Name the diagnosis. * What kind of proliferation or malformation is this? * When is its typical onset?
**Epidermal nevus** * ***Hamartoma* of epidermis and papillary dermis with onset in first year of life** * Papillomatous, pigmented, linear plaques along Blaschko's lines * May be part of an epidermal nevus syndrome that includes other CNS, skeletal and ocular abnormalities * CO2 laser has been used but can lead to scarring
93
Name the diagnosis. * What kind of proliferation or malformation is this? * When is its typical onset?
**Epidermal nevus** * **Hamartoma of epidermis and papillary dermis with onset in first year of life** * Papillomatous, pigmented, linear plaques along Blaschko's lines * May be part of an epidermal nevus syndrome that includes other CNS, skeletal and ocular abnormalities * CO2 laser has been used but can lead to scarring
94
Name the diagnosis. * What kind of proliferation or malformation is this? * When is its typical onset?
**Epidermal nevus** * **Hamartoma of epidermis and papillary dermis with onset in first year of life** * Papillomatous, pigmented, linear plaques along Blaschko's lines * May be part of an epidermal nevus syndrome that includes other CNS, skeletal and ocular abnormalities * CO2 laser has been used but can lead to scarring
95
Name the diagnosis. * What kind of proliferation or malformation is this? * When is its typical onset?
**Epidermal nevus** * **Hamartoma of epidermis and papillary dermis with onset in first year of life** * Papillomatous, pigmented, linear plaques along Blaschko's lines * May be part of an epidermal nevus syndrome that includes other CNS, skeletal and ocular abnormalities * CO2 laser has been used but can lead to scarring
96
Name the diagnosis and clinical sign. * What special "sign" is present? * What does this condition suggest?
**Aplasia cutis congenita** * **Hair collar sign**: congenital ring of dense, dark hair around area of aplasia cutis * Suggests **cranial dysraphism**
97
Name the diagnosis. * What is absent? * How can this initially present? * When this resolves, what can be left behind? * What is the most common location?
**Aplasia cutis congenita** * Solitary (rarely multiple) round area of **localized absense of epidermis, dermis, and sometimes subcutis and calvarium** * Presents as ulcer, erosion or glistening membrane at birth * Resolves leaving **alopecic scar** * 90% on scalp but can be elsewhere
98
Name the diagnosis. * What is absent? * How can this initially present? * When this resolves, what can be left behind? * What is the most common location?
**Aplasia cutis congenita** * Solitary (rarely multiple) round area of **localized absense of epidermis, dermis, and sometimes subcutis and calvarium** * Presents as ulcer, erosion or glistening membrane at birth * Resolves leaving **alopecic scar** * 90% on scalp but can be elsewhere
99
Name the diagnosis. * What is absent? * How can this initially present? * When this resolves, what can be left behind? * What is the most common location?
**Aplasia cutis congenita** * Solitary (rarely multiple) round area of **localized absense of epidermis, dermis, and sometimes subcutis and calvarium** * Presents as ulcer, erosion or glistening membrane at birth * Resolves leaving **alopecic scar** * 90% on scalp but can be elsewhere
100
What are classic associations with **aplasia cutis congenita/hair collar sign**? (3)
* **Teratogens (methimazole)** * **Adams-Oliver syndrome** (aplasia cutis with cranial defect + congenital heart defect + CMTC + limb abnormalities) * **Bart syndrome** (aplasia cutis + DDEB)
101
Name the diagnosis. * Where can this occur? * What does histopathology show? What happens to the epidermis? Corneum? Dermis?
**Supernumerary nipple** * Small soft pink or brown papule with or without an areola and may be concave or umbilicated * Can occur on the **inner proximal extremities, axillae, trunk and groin** * Histopathology: **papillomatous epidermal hyperplasia, hyperkeratosis, smooth muscle bundles and mammary glands and ducts** in dermis and subcutis
102
Name the diagnosis. * Where can this occur? * What does histopathology show? What happens to the epidermis? Corneum? Dermis?
**Supernumerary nipple** * Small soft pink or brown papule with or without an areola and may be concave or umbilicated * Can occur on the **inner proximal extremities, axillae, trunk and groin** * Histopathology: **papillomatous epidermal hyperplasia, hyperkeratosis, smooth muscle bundles and mammary glands and ducts** in dermis and subcutis
103
Name the diagnosis. * Where can this occur? * What does histopathology show? What happens to the epidermis? Corneum? Dermis?
**Supernumerary nipple** * Small soft pink or brown papule with or without an areola and may be concave or umbilicated * Can occur on the **inner proximal extremities, axillae, trunk and groin** * Histopathology: **papillomatous epidermal hyperplasia, hyperkeratosis, smooth muscle bundles and mammary glands and ducts** in dermis and subcutis
104
What is on the differential diagnosis for **diaper dermatitis**? * Name at least five things.
* **Acrodermatitis enteropathica, cystic fibrosis** (both due to zinc deficiency) * **Irritant contact dermatitis** (spares inguinal folds) * **Candidal dermatitis** (papulopustules, "satellite" lesions, +intertriginous, +scrotum, thrush) * **Langerhans cell histiocytosis** * **Psoriasis** (+intertriginous, minimal scale)

BASIC Exam Study Guide (11 decks)

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