DD 03-10-14 10-11am Skin Signs of Systemic Disease - Dunnick Flashcards

1
Q

Clubbing – defn.

A

Nail plate is enlarged & excessively curved (>180 degree angle btwn proximal nail fold & nail plate)

Caused by enlarge of the soft tissue of the distal digit

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2
Q

Clubbing – etiologies

A

Rarely idiopathic/primary

Pulmonary disease (idiopathic, pulmonary fibrosis, lung cancer)

Cardiac disease (cyanotic congenital heart disease)

GI disease (Crohn disease, Ulcerative colitis, Proctitis)

Malignancies (Thyroid / Thymus cancer, Hodgkin disease)

Hypoxemia (possibly related to long-term cannabis smoking)

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3
Q

Terry’s Nails

A

Liver cirrhosis in 82% (also in normal individuals)

Leukonychia affects entire nail except for 1-2 mm distal band

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4
Q

Half-n-half nails

A

Distal nail is normal, proximal nail is white

In 10% of pts w/chronic renal failure (also in normal individuals)

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5
Q

Proximal Subungual White Onychomycosis

A

Associated w/HIV disease

Usually due to Trichophyton rubrum (more specifically called tinea ungum)

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6
Q

Kaposi’s Sarcoma (KS)

A

Usually related to immunosuppression

ENDOthelial malignancy, triggered by HHV-8

Slowly progressive, not very common

Generally brownish purple/red patches

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7
Q

Classic Kaposi’s sarcoma

A

Occurs mostly in elderly men of Eastern European descent

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8
Q

Lymphadenopathic Kaposi’s sarcoma

A

Aggressive form primarily in equatorial Africa

Affects young men & is rapidly fatal

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9
Q

AIDS-Associated Kaposi’s Sarcoma

A

More frequent in homosexual pts w/AIDS

Incidence declining w/better anti-retroviral therapy against HIV

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10
Q

Therapy for Kaposi’s Sarcoma

A

Radiation therapy
Excision
Interferon alpha
Chemotherapy

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11
Q

Thyroid disease – signs/symptoms

A
  • Exophthalmos
  • Pretibial myxedema
  • May cause alopecia areata (if see alopecia, test TSH)
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12
Q

Alopecia areata – association

A

T cells fighting off hair cells, so…

Associated w/other autoimmune diseases:

  • thyroid
  • vitiligo
  • IBD
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13
Q

Alopecia areata – Clinical Non-Scarring Alopecia

A

Round/oval patches of hair loss
Short “exclamation point” hairs, broader at distal end
Hairs often re-row w/ depigmentation

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14
Q

Alopecia areata – Clinical subtypes

A
Patch focal
Ophiasis pattern
Diffuse variant
Alopecial totalis (all scalp hair)
Alopecia unversalis (all body hair)
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15
Q

Vitiligo - characteristics

A

Development of total white macules / patches

Histology show complete absence of melanocytes

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16
Q

Vitiligo – Associations

A

Most commonly thyroid disease (Hashimoto thyroiditis, Graves’ disease) – screen TSH levels

Other endocrine disorders (Diabetes Mellitis, Pernicious Anemia, Addison’s disease)

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17
Q

Vitiligo – Treatment

A

Topical steroids

Topical calcineuron inhibitor (protopic ointment)

Narrow-band UVB (311nm) or Excrimer Laser (308 nm) –> repigmentation

Psoralens plus UVA (PUVA)

Minigrafting

Depigmentation (monobenzylether of hydroquinone cream)

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18
Q

Excimer laser

A

UVB ray source (308nm)

Xenon-Chloride lamp emitting non-coherent, monochromatic 308nm light

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19
Q

Erythema Nodosum – clinical manifestations

A

Painful, erythematous subQ nodules (subQ inflammation)

Usually symmetically over pretibial lower extremities

Develop bruiselike appearance in later stages

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20
Q

Erythema nodosum – M vs. F, Associated symptoms, Histopathology

A

More common in women

May also have fever, arthralgias, malaise

Histopathology shows septal panniculitis wit neutrophils (inflame. in subQ fat)

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21
Q

Erythemia nodosum - etiology

A

Delayed hypersensitivity to various antigen stimuli (usually infections)

Most common cause: Strep infections, esp. URI

Other infections (1/3 of the cases) – viral URIs, Mycoplasma, TB

Coccidioidomycosis

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22
Q

Erythema nodosum – other more common associations

A

Idiopathic (35-55%)
Drugs – estrogens, oral contraceptives, sulfonamides, penicillin, bromides, iodides
Sarcoidosis (11-22%)
IBD (esp. Crohn’s)

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23
Q

Treatment of Erythema nodosom

A

Bed rest
Treat underlying condition
NSAIDs – Naproxen, Indomethacin

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24
Q

Pyroderma Gangrenosum - clinical manifestations of lesions

A

Initial lesion often pustule on erythematous base or erythematous nodule

Characteristic lesion is an ulcer w/necrotic, undermined (rolled) border

Painful

Usually on lower extremities

Often begins in sites of minor trauma (pathergy)

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25
Pyoderma Gangrenosum – associations
- 50-70% have underying associated condition - IBD (20-30%) - Arthritis (seronegative arthritis, RA – 20%) - Monoclonal gammopathy (often IgA, up to 15%) - Other hematologic disorders (Myelogenous leukemia, hair cell leukemia, myelofibrosis – 10%)
26
Treatment of Pyoderma Gangrenosum
- Conservative wound care - AVOID SURGERY/DEBRIDEMENT (causes more injury, more inflammation, never heals) - Oral / topical anti-inflammatory agents like steroids
27
Lichen Planus – clinical manifestations
- Purple polygonal pruritic papules - Wickham’s striae = overlying lace-like pattern of white lines on the surface - Mucosal lesions w/out skin findings in 15-25% of pts - Nail involvement in 10% (may be only involvement)
28
Lichen Planus – Clinical Variants
``` Annular or Linear (Koebner) Hypertrophic or Atrophic Erosive/ulcerative Actinic Bullous ```
29
Lichen Planus – Associations
*Hepatits C – screen for* Contact allergy to dental metals (amalgams [mercury], copper, gold)
30
Lichen Planus – Treatment
Nothing works very well :( - Corticosteroids (Topical, Intralesional, Oral - Phototherapy - Systemic retinoids (isotretinoin, acetretin) - Griseofulvin - Dapsone - Plaquenil - Cyclosporine *treatment of underlying HepC won’t necessarily make lichen planus go away
31
Acanthosis Nigricans – clinical manifestations
Velvety hyperpigmentation of intertriginous surfaces & extensor surfaces (less commonly) Most often neck, axillae, dorsal hands
32
Acanthosis Nigricans – due to…
Due to factors stimulating epidermal keratinocytes & dermal fibroblast proliferations
33
Acanthosis Nigricans – Associations
- Rarely Familial : AD, onset in childhood - *Obesity - *Diabetes mellitus & Insulin resistance - Endocrinopathies (hyperandrogenemia, Cushing’s, polycystic ovary, total lipodystrophy) - Drugs (rare) – nicotinic acids, systemic steroids - Malignancy
34
Acanthosis Nigricans –Malignancy
- May precede or accompany or follow onset of internal cancer - Malignancies include gastric adenocarcinoma (also, lung & breast cancer, others) - Rapid onset w/ weight loss (to distinguish from other reasons for acanthosis nigricans)
35
Acanthosis Nigricans – Treatment
Treat underlying disorder Keratolytics like ammonium lactate or urea cream (exfoliate)
36
Dermatomyositis – clinical manifestations
- Photodistributed violaceous poikiloderma - favors scalp, periocular & extensor skin sites - Heliotrope = eruption on upper eyelids +/- periorbital edema - Samitz sign - Gottron’s papules - Gottron’s sign - Shawl sign
37
Dermatomyositis – Samitz sign
ragged cuticles in Dermatomyositis
38
Dermatomyositis – Gottron’s papules
Lichenoid papules overlying knuckles, elbows, knees in Dermatomyositis
39
Dermatomyositis – Gottron’s sign
Poikiloderma over knuckles, elbows, knees in Dermatomyositis
40
Dermatomyositis – shawl sign
Poikiloderma across back & shoulders in Dermatomyositis
41
Dermatomyositis – Malignancy prevalence
- Internal malignancy in adults from 10-50% - More often in females - May be higher in pts w/dermatomyositis sine myositis - With new onset dermatomyositis in older adult (usually not kids), think about malignancy
42
Dermatomyositis – Malignancy types
Most common: GU malignancies, esp. ovarian Others: breast, lung, gastric carcinoms Nasopharyngeal carcinoma in Asian men
43
Dermatomyositis – Malignancy work-up
Complete Hx & PE Age appropriate malignancy screening (mammo, CXR, colonoscopy, Pap, PSA, CBC) Repeat malignancy screen ever 6-12 mo for 1st 2 yrs or more Risk of malignancy declines after 1st 2 years & reaches baseline after 5yrs
44
Dermatomyositis – Treatment of Skin disease
``` Sunscreen Topical steroids Hydroxychloroquine Quinacrine Methotrexate Retinoids ```
45
Dermatomyositis – Treatment of Systemic disease
- Oral prednisone (tapered over 2-3 years) - Methotrexate - Azathiprine - High dose IVIG
46
Acute urticaria – rxn type
Immediate type I hypersensitivity rxn by IgE antibodies
47
Urticaria – Acute vs. Chronic
Acute: 6 week duration, women - most often in women 20-40 yo - may be related to circulating autoAbs against Ig-epsilon-R1 or IgE
48
Causes of Urticaria
Usually idiopathic/autoimmune Infection, Drugs, Foods, Vasculitis, Contactants, Inhalants, Pregnancy, Meds
49
Drugs & Urticaria
Cause <10% of all urticaria Commmon drugs : Penicillins, Cephalosporins, NSAIDs, mAbs, Contrast media, Latex
50
Eczematous eruptions
Most common type of drug rxn in skin Usually cell-mediated type IV hypersensitivity Begins 7-14 days after new med (sooner if receiving “old” med)
51
Exanthematous eruptions - causes
Occur all over the body – systemic drug rxn or infection, usually More commonly drug-induced in adults Aminopenicillins, sulfonamides, cephalosporins, anticonvulsants, allopurinol
52
Exanthematous drug eruption - treatment
- Discontinue offending med & check of results in three weeks (educated guesswork) - Supportive topical steroids & anti-histamines - Generally resolves spontaneously after 1-2 weeks (but can take up to 3 mo to resolve completely)
53
Stevens-Johnson Syndrome vs. Toxic Epidermal Necrolysis
30% = Toxic epidermal necrolysis In between = overlap syndrome
54
Stevens-Johnson Syndrome – clinical manifestations
- Eruption on face/upper trunk --> may become confluent - Evolves to skin necrosis & flaccid bullae - Involves mucous membrane
55
Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis –Cause
Due to abnormal drug metabolism & immune-complex mediated hypdersensitivity
56
Stevens-Johnson Syndrome – characterized by
Epidermal detachment
57
Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis – Long term sequelae
Ocular problems including conjunctival synechiae & blindness
58
Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis – Med causes
- Antibiotics (sulfur, ampicillin) - Seizure meds - NSAIDS - Allopurinol - Antiretroviral (Nevirapine, Abacavir)
59
Stevens-Johnson Syndrome – Treatment
- Early withdrawal of all possible offending drugs - Symptomatic (dressing, IVF, nutrition) - Steroids / High dose IVIG = controversial
60
Toxic Epidermal Necrolysis – SCORTEN
Score based on Prognostic factors to determine Mortality: - Age >40yrs - HR >120 bpm - Malignancy - Body Surface Area (BSA) >10% at day 1 - high BUN & Glucose, low Bicarb
61
Skin signs of Thyroid disease
Vitiligo Alopecia areata Exophthalmos Pretibial myxedema
62
Signs of immunosuppression
Kaposi’s sarcoma | Proximal subungual onychomycosis
63
Skin & GI disorders
Hep C virus = Lichen planus IBD = Erythema nodosum, Pyoderma grangrenosum
64
Skin signs of internal malignancy
Acanthosis Nigricans Dermatomyositis Clubbing
65
Drug eruptions
Urticaria Morbilliform eruptions Steven Johnson syndrome Toxic Epidermal Necrolysis