DISCLOSURE OF RELEVANT RELATIONSHIPS WITH INDUSTRY

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1 DISCLOSURE OF RELEVANT RELATIONSHIPS WITH INDUSTRY Nicole Burkemper, MD Associate Professor of Dermatology and Pathology Saint Louis University I HAVE NO RELEVENT RELATIONSHIPS WITH ANY COMPANIES

2 The most likely diagnosis is: A. Allergic contact dermatitis B. Cellulitis C. Neuropathic diabetic ulcer D. Pyoderma gangrenosum E. Sweet syndrome

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4 The most likely diagnosis is: A. Allergic contact dermatitis B. Cellulitis C. Neuropathic diabetic ulcer D. Pyoderma gangrenosum E. Sweet syndrome

5 Cellulitis Ill-defined erythema, warmth, edema Systemic symptoms of fever, chills and malaise Leading edge not raised or sharply demarcated (distinguishes from erysipelas) Bullae and petechiae Streptococcus pyogenes and Staphylococcus aureus Often after wound; on leg, tinea pedis is most common portal of entry Predisposing factors: venous and lymphatic damage, congenital vascular malformations

6 Distractors Allergic contact dermatitis * epidermal changes Neuropathic diabetic ulcer sole of foot

7 Distractors Pyoderma gangrenosum * sharply marginated wet ulcer with purple undermined border Sweet s syndrome * pink edematous plaques on the face, upper trunk and arms

8 The most likely diagnosis is: A. Allergic contact dermatitis B. Cutaneous candidiasis C. Lichen sclerosus D. Tinea corporis E. Tinea versicolor

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10 The most likely diagnosis is: A. Allergic contact dermatitis B. Cutaneous candidiasis C. Lichen sclerosus D. Tinea corporis E. Tinea versicolor

11 Tinea versicolor Presents as hypo- or hyperpigmented coalescing scaly macules Trunk, upper arms and neck Caused by Malassezia furfur KOH short, thick hyphae and spores ( spaghetti and meatballs ) Topical or systemic antifungal treatment Recurrence rate very high ketoconazole shampoo weekly to prevent recurrence

12 Distractors Allergic contact dermatitis * pink patches or plaques, not hypo- or hyperpigmented; patterned Cutaneous candidiasis * moist patches in intertriginous areas with satellite pustules

13 Distractors Lichen sclerosus * atrophic cigarette paper plaques often in genital and perianal areas Tinea corporis * annular, sharply circumscribed, erythematous, scaly patches

14 The most likely diagnosis is: A. Contact dermatitis B. Cellulitis C. Erythema migrans D. Erythema dyschromicum perstans E. Psoriasis

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16 The most likely diagnosis is: A. Contact dermatitis B. Cellulitis C. Erythema migrans D. Erythema dyschromicum perstans E. Psoriasis

17 Contact dermatitis Well-demarcated erythematous vesicular to scaly plaque with well-defined margins corresponding to the area of contact Because irritant contact dermatitis and allergic contact dermatitis not always discernible clinically, patch testing may be required to identify an allergen and exclude allergy Most common allergens are nickel, neomycin, balsam of Peru, fragrance mix, thimerosal (often not relevant), gold, quaternium-15, formaldehyde, bacitracin and cobalt

18 Distractors Cellulitis * more welldefined, erythematous and edematous; leg most common site Erythema migrans * annular patches most commonly on legs, groin, axilla and trunk

19 Distractors Erythema dyschromicum perstans ashy-gray oval macules and patches on the face, neck, trunk and proximal extremities in patients with darker skin types Psoriasis * more well-demarcated bright red plaques with overlying silvery scale

20 This patient just returned from a beach vacation in the Caribbean and complains of this itchy rash on the buttock. The most likely diagnosis is: A. Allergic contact dermatitis B. Cutaneous candidiasis C. Cutaneous larva migrans D. Perianal strep dermatitis E. Scabies

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22 This patient just returned from a beach vacation in the Caribbean and complains of this itchy rash on the buttock. The most likely diagnosis is: A. Allergic contact dermatitis B. Cutaneous candidiasis C. Cutaneous larva migrans D. Perianal strep dermatitis E. Scabies

23 Cutaneous larva migrans Most commonly caused by larvae of cat and dog hookworm, Ancylostoma braziliense; SE United States, Caribbean From environment contaminated with animal feces, beach Human is dead-end host Sxs: 1-6 days from exposure; creep several cm per day; die in 2 to 8 weeks without treatment Tx: single-dose ivermectin or 3 days albendazole

24 Distractors Allergic contact dermatitis * can be linear, not serpiginous Cutaneous candidiasis * Often in the folds; beefy red, moist patches with satellite pustules

25 Distractors Perianal strep dermatitis Perianal well-demarcated rim of erythema Scabies * Short burrows that prefer the finger webs, wrists, axillae, areolae, umbilicus and genitals

26 This patient presents with fatigue and anemia. The most likely diagnosis is: A. Basal cell nevus syndrome B. Blue rubber bleb nevus syndrome C. Gaucher disease D. Pachyonychia congenita E. Peutz Jeghers

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28 This patient presents with fatigue and anemia. The most likely diagnosis is: A. Basal cell nevus syndrome B. Blue rubber bleb nevus syndrome C. Gaucher disease D. Pachyonychia congenita E. Peutz Jeghers

29 Blue rubber bleb nevus syndrome Autosomal dominant Dark blue papules and nodules and skincolored compressible protuberances ( rubber blebs ) and larger venous malformations affecting skin and mucosa GI lesions bleed and lead to iron deficiency anemia

30 Distractors Basal cell nevus syndrome * palmar pits Gaucher disease Bronze coloration of skin; some have congenital ichthyosis

31 Distractors Pachyonychia congenita doorstop nails ; steatocystomas when mutations in keratin 17 Peutz-Jeghers * hyperpigmented macules on lips and oral mucosa; polyposis of SI with GI bleeding

32 The most likely diagnosis is: A. Cellulitis B. Ichthyosis vulgaris C. Mycosis fungoides D. Necrolytic migratory erythema E. Psoriasis

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34 The most likely diagnosis is: A. Cellulitis B. Ichthyosis vulgaris C. Mycosis fungoides D. Necrolytic migratory erythema E. Psoriasis

35 Ichthyosis Vulgaris Ichthyosis is derived from the Greek word ichthys, meaning fish Autosomal dominant; prevalence as high as 1 in 250 Onset in early childhood Extensor LE primarily affected with scales; hyperlinear palms Loss-of-function mutations in the filaggrin gene (FLG)

36 Distractors Cellulitis * well-defined erythema, warmth, edema; no scale Mycosis fungoides * fairly welldemarcated patches/plaques typically in sun-protected areas

37 Distractors Necrolytic migratory erythema * pink and eroded plaques classically in intertriginous areas Psoriasis * well-demarcated red plaques with silver scale

38 This patient also has a fever. The most likely diagnosis is: A. Acne keloidalis B. Arthropod bites C. Nummular dermatitis D. Prurigo nodularis E. Sweet syndrome

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40 This patient also has a fever. The most likely diagnosis is: A. Acne keloidalis B. Arthropod bites C. Nummular dermatitis D. Prurigo nodularis E. Sweet syndrome

41 Sweet syndrome Acute febrile neutrophilic dermatosis Adults; F3:M1 Sharply demarcated, tender, pink-violaceous edematous plaque involving face, neck, upper trunk and extremities Fever, arthralgias, myalgias Elevated ESR and neutrophilia Associated with URI (most common), hematologic malignancy or solid tumors, inflammatory diseases and pregnancy Standard treatment is systemic corticosteroids

42 Distractors Acne keloidalis * no fever; papules, pustules and keloidal plaques at back of neck Arthropod bite * no fever; edematous papule, plaque or bulla

43 Distractors Nummular eczema * no fever; discrete, coin-shaped vesicular or crusted pruritic plaques Prurigo nodularis * no fever; pruritic dome-shaped papules

44 This condition becomes more prominent with cold exposure. The most likely diagnosis is: A. Erythema ab igne B. Livedo reticularis C. Lymphangioma D. Necrolytic acral erythema E. Small vessel vasculitis

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46 This condition becomes more prominent with cold exposure. The most likely diagnosis is: A. Erythema ab igne B. Livedo reticularis C. Lymphangioma D. Necrolytic acral erythema E. Small vessel vasculitis

47 Livedo reticularis Netlike, mottled or reticulated, pink or reddish-blue discoloration of the skin, mostly on the legs Livedo racemosa (fixed LR) broken circular segments; almost always associated with significant systemic disease such as hypercoagulable states (including myelodysplasias, cancer, and antiphospholipid and Sneddon syndromes), vasculitis (especially medium- and large-vessel), emboli, medications, and neurologic disorders

48 Livedo racemosa

49 Distractors Erythema ab igne no variability with temperature; reticulated erythema or pigmentation in area of of persistent heat exposure Lymphangioma *--no worse with change in temperature; white vesicles that can become hemorrhagic (frog spawn)

50 Distractors Necrolytic acral erythema * no change with temperature; dusky plaques on feet +/- hands Small vessel vasculitis * no change with temperature; palpable purpura

51 The most likely diagnosis is: A. Darier disease B. Lichen planus C. Onychomycosis D. Psoriasis E. Yellow nail syndrome

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53 The most likely diagnosis is: A. Darier disease B. Lichen planus C. Onychomycosis D. Psoriasis E. Yellow nail syndrome

54 Onychomycosis Yellowing, hypertrophy, and dystrophy of the distal nail plate with subungual hyperkeratosis (distal subungual onychomycosis) Trichophyton rubrum most common cause Usually multiple nails Toenail infection is much more common than fingernail infection Nail dystrophy is secondary to onychomycosis in more than 50% of cases (90% dermatophytes)

55 Distractors Darier disease * red and white longitudinal streaks; V-shaped nicking of distal margin Lichen planus * dorsal pterigium; trachyonychia (twenty nail dystrophy)

56 Distractors Psoriasis * salmon patches, oil drop sign, pitting, onycholysis; can be clinically indistinguishable from onychomycosis Yellow nail syndrome * marked thickening and yellow discoloration

57 The most likely diagnosis is: A. Cowden syndrome B. Epidermodysplasia verruciformis C. Heck disease D. Traumatic fibromas E. Tuberous sclerosis

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59 The most likely diagnosis is: A. Cowden syndrome B. Epidermodysplasia verruciformis C. Heck disease D. Traumatic fibromas E. Tuberous sclerosis

60 Cowden syndrome Autosomal dominant; PTEN tumor suppressor gene Mucocutaneous findings: Facial papules: flesh-colored, flat-topped, or elongated verrucoid papules ranging from 1- to 5-mm Most are trichilemmomas and have a keratin-plugged center Oral lesions: 1- to 3-mm smooth whitish oral papules on gingiva, lips, palate or tongue in 80% May coalesce giving cobblestone appearance Acral keratoses: flesh-colored smooth or verrucoid papules on dorsal hands and feet in 60% Neoplasms of the breast and thyroid occur in up to two thirds of patients, can be malignant

61 Distractors Heck disease Small white to pink papules diffusely in the mouth, not on face; HPV- 13 Epidermodysplasia verruciformis flat, wart-like lesions on dorsal hands, extremities, face and neck; tinea versicolor type patches on trunk; HPV-5 and -8

62 Distractors Traumatic fibromas solitary, smooth, pink nodule at bite line of buccal or labial mucosa or tongue; no skin findings Tuberous sclerosis * oral findings are gingival fibromas and dental pits; would also have facial angiofibromas

63 The most likely diagnosis is: A. Acne vulgaris B. Eosinophilic folliculitis C. Pityriasis lichenoides D. Prurigo nodularis E. Steatocystoma multiplex

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65 The most likely diagnosis is: A. Acne vulgaris B. Eosinophilic folliculitis C. Pityriasis lichenoides D. Prurigo nodularis E. Steatocystoma multiplex

66 Acne vulgaris Comedones, papules, pustules, cysts, scarring Patients have a variety of lesions in various states of formation and resolution Follicular hyperkeratosis, sebum production, Propionibacterium acnes bacteria, inflammation Retinoids = 1 st line for all forms of acne vulgaris due to their comedolytic and anti-inflammatory effects as well as their ability to help penetration of other topicals

67 Distractors Eosinophilic folliculitis papulopustules and plaques studded with pustules and central clearing; no comedones; pruritic Pityriasis lichenoides * eroded or scaly papules that heal with hypopigmentation; no comedones

68 Distractors Prurigo nodularis * domeshaped nodules with central crust in accessible areas Steatocystoma multiplex Yellowish cysts without puncta; no pustules

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73 This patient presents with fever and rash. The most likely diagnosis is: A. Acute generalized exanthematous pustulosis B. Bacterial folliculitis C. Disseminated zoster D. Measles E. Toxic epidermal necrolysis

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76 This patient presents with fever and rash. The most likely diagnosis is: A. Acute generalized exanthematous pustulosis B. Bacterial folliculitis C. Disseminated zoster D. Measles E. Toxic epidermal necrolysis

77 Acute generalized exanthematous pustulosis (AGEP) Fever and countless nonfollicular, sterile, 1- to 2-mm pustules on background of erythema Typically 4 days of new drug β-lactam antibiotics, macrolides, Ca ++ channel blockers, antimalarials Treatment: Drug cessation, topical steroids, antipyretics Acuity and drug history help distinguish from pustular psoriasis

78 Bacterial folliculitis * follicular pustules without background erythema Distractors Disseminated zoster * vesicles (not pustules) on an erythematous base; immunocompromised

79 Distractors Measles morbilliform eruption; spreads cephalocaudad; no pustules Toxic epidermal necrolysis * painful, pink to dusky-red patches with vesicles, bullae and erosions; mucosal erosions

80 This patient also complains of loss of the eyebrows. Of the following, the most likely diagnosis is: A. Androgenetic alopecia B. Central centrifugal cicatricial alopecia C. Lichen planopilaris D. Telogen effluvium E. Trichotillomania

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82 This patient also complains of loss of the eyebrows. Of the following, the most likely diagnosis is: A. Androgenetic alopecia B. Central centrifugal cicatricial alopecia C. Lichen planopilaris D. Telogen effluvium E. Trichotillomania

83 Lichen planopilaris Keratotic plugs surrounded by violaceous rim on the scalp resulting in scarring alopecia Peri-infundibular lymphocytic inflammation with vacuolar interface dermatitis Women affected more frequently than men Frontal fibrosing alopecia is a variant of LPP that presents in older women with bandlike frontotemporal scarring alopecia with loss of eyebrows Treatment often difficult hydroxychloroquine; topical, IL and po steroids, pioglitazone (PPAR-gamma agonist) and immunosuppressive agents

84 Distractors Central centrifugal cicatricial alopecia--almost exclusively black women; scarring alopecia at the vertex or crown Androgenetic alopecia * thinning of crown with wider part anteriorly and preservation of frontal hairline

85 Telogen effluvium thinning of hair of entire scalp, not just crown Distractors Trichotillomania * patches of alopecia with hairs of varying lengths interspersed with uninvolved areas of the scalp

86 The most likely diagnosis is: A. Allergic contact dermatitis B. Amyloidosis C. Dermatomyositis D. Necrobiotic xanthogranuloma E. Xanthelasma

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88 The most likely diagnosis is: A. Allergic contact dermatitis B. Amyloidosis C. Dermatomyositis D. Necrobiotic xanthogranuloma E. Xanthelasma

89 Amyloidosis Primary systemic amyloidosis (AL amyloid) Multiorgan disease with underlying plasma cell dyscrasia Cutaneous manifestations in 25%: Purpura/ecchymoses (most common) Rubbery swelling and infiltration of tongue/oral mucosa Waxy infiltration of palms/fingertips Waxy or purpuric periorificial papules Diffuse sclerodermoid infiltration Bullae Onychodystrophy

90 Cutaneous amyloidosis Macular amyloidosis: brown rippled macules, often on back Lichen amyloidosis: persistent, brown, pruritic papules/rippled plaques on the shins Nodular amyloidosis: waxy nodule(s) often at acral sites, AL amyloid but rare progression to systemic amyloidosis

91 Distractors Allergic contact dermatitis * scaly pink patches Dermatomyositis * violaceous erythema (heliotrope)

92 Distractors Necrobiotic xanthogranuloma-- indurated yellowish plaques that can ulcerate Xanthelasma--yellow papules and plaques

93 The most likely diagnosis is: A. Grave disease B. HAART-associated lipodystrophy C. Lupus panniculitis D. Rosacea E. Scleromyxedema

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95 The most likely diagnosis is: A. Grave disease B. HAART-associated lipodystrophy C. Lupus panniculitis D. Rosacea E. Scleromyxedema

96 HAART-associated lipodystrophy Lipodystrophy occurs in up to 80% of HIV-infected patients, most of whom are on anti-retroviral therapy Fat of the face, buttocks and limbs is lost with increased fat at the upper back and abdomen Related to non-nucleoside reverse transcriptase inhibitors which also lead to adipocyte apoptosis May also have hypertriglyceridemia, hypercholesterolemia and insulin resistance, especially if also on a protease inhibitor Treatment with rosiglitazone, metformin, growth hormone and fillers

97 Distractors Grave disease exophthalmos Lupus panniculitis * proximal extremities; nodules or indurated plaques atrophy

98 Rosacea * conjunctivitis, styes; centrofacial erythema, papules and micropustules Distractors Scleromyxedema--waxy firm papules that coalesce into infiltrated plaques

99 The most likely diagnosis is: A. Brown recluse spider bites B. Bed bug bites C. Erythema migrans D. Scabies E. Sporotrichosis

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101 The most likely diagnosis is: A. Brown recluse spider bites B. Bed bug bites C. Erythema migrans D. Scabies E. Sporotrichosis

102 Bites, stings, infestations Excoriated, pruritic papules, often grouped Central puncta Exaggerated responses: bullous lesions or papular urticaria more common in children Bedbug bites (Cimex lectularius) often linear

103 Distractors Erythema migrans * erythematous expanding annular plaque; usually solitary Brown recluse spider bite Painful edematous reaction progressing to bulla with surrounding erythema and ischemia

104 Distractors Scabies * burrows in finger webs, umbilicus, nipples, male genitalia Sporotrichosis * ulcerated papule at site of inoculation (hand) and nodules along path of lymphatic drainage

105 The most likely diagnosis is: A. Confluent and reticulated papillomatosis B. Hailey-Hailey disease C. Recurrent and disseminated infundibulofolliculitis D. Tinea versicolor E. Transient acantholytic dermatosis

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107 The most likely diagnosis is: A. Confluent and reticulated papillomatosis B. Hailey-Hailey disease C. Recurrent and disseminated infundibulofolliculitis D. Tinea versicolor E. Transient acantholytic dermatosis

108 Confluent and reticulated papillomatosis Onset: puberty-young adulthood, more common in black patients Multiple brown verrucous papules/patches, central chest/abdomen/upper back; confluent centrally and reticulated peripherally May mimic tinea versicolor (KOH will be negative) or acanthosis nigricans Treatment: Minocycline, azithromycin, topical and oral retinoids

109 Hailey-Hailey disease * macerated plaques in intertriginous areas Distractors Recurrent and disseminated infundibulofolliculitis-- numerous, pruritic, 1-2 mm skincolored papules on trunk, neck and upper extremities; darker pigmented patients

110 Tinea versicolor * brown, tan or hypopigmented scaly round to oval patches on trunk Distractors Transient acantholytic dermatosis * crusted pink papules on upper trunk of older adults