DISCLOSURE OF RELEVANT RELATIONSHIPS WITH INDUSTRY

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1 DISCLOSURE OF RELEVANT RELATIONSHIPS WITH INDUSTRY Ian A. Maher, MD, FAAD, FACMS Assistant Professor of Dermatology Saint Louis University I HAVE NO RELEVANT CONFLICTS OF INTEREST

2 The most likely diagnosis is: A. Wart B. Malignant melanoma C. Basal cell carcinoma D. Verrucous carcinoma E. Corn

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4 The most likely diagnosis is: A. Wart B. Malignant melanoma C. Basal cell carcinoma D. Verrucous carcinoma E. Corn

5 Verrucous carcinoma Low grade SCC Associated with high risk HPV subtypes (6b, 11, 16 and 18) Burrowed appearance Soles most common location Histology Minimal atypia Pushing border

6 Distractors *Wart Lesion very large, ulcerated and burrowed. Makes this diagnosis less likely *Melanoma Normally pigmented Can ulcerate *Basal cell carcinoma Abnormal on soles Normally pink, pearly Corn Hyperkeratotic papule at pressure point Accentuated skin lines

7 The most likely diagnosis is: A.Contact dermatitis B.Dermatomyositis C.Glomus tumor D.Hutchinson s sign E.Maffucci's syndrome Immersion Dermoscopy

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9 The most likely diagnosis is: A.Contact dermatitis B.Dermatomyositis C.Glomus tumor D.Hutchinson s sign E.Maffucci's syndrome Immersion Dermoscopy

10 Associated with connective tissue diseases Dermatomyositis Systemic lupus erythematosus Scleroderma Dermatomyositis Cutaneous signs Heliotrope rash Shawl sign Gottron sign Proximal muscle weakness Dermatomyositis

11 Shawl like rash on chest and Gottron papules

12 Proximal Nailfold (Periungual) Telangiectasia Dermatomyositis Systemic lupus erythematosus Scleroderma Sausage-shaped loops of dilated capillaries and vascular dropout accentuated with oil immersion dermoscopy Immersion Dermoscopy

13 Distractors *Contact dermatitis erythema, hyperkeratosis, scaling and fissuring

14 Distractors Glomus tumor Painful distal or nailbed benign proliferation of glomus cells

15 Distractors Hutchinson s sign spread of pigmentation onto nailfold from subungual *melanoma

16 The patient presents for evaluation of these asymptomatic papules. He has a personal history of 3 episodes of pneumothorax. His family history is significant for renal carcinoma. What is the most likely diagnosis? a) Birt-Hogg-Dube b) Tuberous sclerosis c) Brooke-Spiegler d) Neurofibromatosis e) Cowden syndrome

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18 The patient presents for evaluation of these asymptomatic papules. He has a personal history of 3 episodes of pneumothorax. His family history is significant for renal carcinoma. What is the most likely diagnosis? a) Birt-Hogg-Dube b) Tuberous sclerosis c) Brooke-Spiegler d) Neurofibromatosis e) Cowden syndrome

19 Birt-Hogg-Dube Mutation in folliculin, AD inheritance Skin manifestations Fibrofolliculomas Trichodiscomas Like many appendageal tumors these are non-descript flesh colored papules. Often around the nose Tend to be flat-topped Spontaneous pneumothorax Renal Carcinoma

20 *Tuberous sclerosis Distractors Skin findings Angiofibromas Renal findings angioleiomyomas Lung finding lymphangiomyomas CNS finding cortical tubers, seizures Brooke-Spiegler *Trichoepitheliomas Cylindromas

21 Distractors (cont.) *Type 1 Neurofibromatosis Neurofibromas *Cowden syndrome Skin findings tricholemmomas, mucosal papules Associated cancers breast, endometrium, thyroid

22 The most likely diagnosis is: A. Acne keloidalis nuchae B. Discoid lupus erythematosus C. Folliculitis decalvans D. Lichen planopilaris E. Trichotillomania Photo Courtesy Dr. M. Ioffreda

23 Photo Courtesy Dr. M. Ioffreda

24 The most likely diagnosis is: A. Acne keloidalis nuchae B. Discoid lupus erythematosus C. Folliculitis decalvans D. Lichen planopilaris E. Trichotillomania Photo Courtesy Dr. M. Ioffreda

25 Initially starts with chronic folliculitis and perifolliculitis of the posterior scalp and neck

26 Gradually develops into large plaques with alopecia and tufted hairs (like doll s hairs) at periphery

27 Acne keloidalis nuchae African Americans >>> Hispanics > Asians > Caucasians Men (20):Women (1), almost always after puberty Cause uncertain; possibly reaction to curling hairs penetrating skin Subcutaneous abscesses and draining sinuses may develop Treatment options Prevention: avoid close shave and irritating collars Topical retinoids or antibiotics Excision (may need serial excision)

28 Distractors *Discoid Lupus Erythematosus

29 Distractors *Folliculitis Decalvans

30 Distractors *Lichen Planopilaris

31 The most likely diagnosis is: A. Acquired digital fibrokeratoma B. Pyogenic granuloma C. Squamous cell carcinoma D. Supernumerary digit E. Verruca vulgaris

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33 The most likely diagnosis is: A. Acquired digital fibrokeratoma B. Pyogenic granuloma C. Squamous cell carcinoma D. Supernumerary digit E. Verruca vulgaris

34 Acquired Digital Fibrokeratoma Usually a solitary, skin-colored to pink, slightly keratotic exophytic papulonodule with collarette of skin Location: fingers most common, but may be anywhere on acral skin

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36 When periungual or subungual may be associated with tuberous sclerosus Koenen s Tumor

37 Distractors *Pyogenic Granuloma wet, red friable nodule

38 Distractors *Squamous cell carcinoma rare on the plantar foot or to not have any crusting or ulceration (would usually be verrucous carcinoma or Marjolin s ulcer)

39 Distractors *Verruca Vulgaris (Wart) no thrombosed capillaries

40 The most likely diagnosis is: A. Berloque dermatitis B. Dermatomyositis C. Erythromelanosis follicularis faciei D. Poikiloderma of Civatte E. Subacute cutaneous lupus

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42 The most likely diagnosis is: A. Berloque dermatitis B. Dermatomyositis C. Erythromelanosis follicularis faciei D. Poikiloderma of Civatte E. Subacute cutaneous lupus

43 Poikiloderma of Civatte Reticulated hyperpigmented patches associated with telangiectases and mild atrophy with sparing of perifollicular skin Most common on the lateral aspects of neck of fair-skinned people with chronic sun exposure Look for other signs of photoaging Histology: telangiectasias, dermal atrophy, irregular pigmentation of basal layer

44 Distractors Berloque Dermatitis

45 Distractors *Dermatomyositis

46 Distractors *Subacute Cutaneous Lupus

47 A. Actinic keratosis B. Atopic dermatitis C. Cutaneous T-cell lymphoma D. Psoriasis E. Tinea manuum The most likely diagnosis is:

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49 A. Actinic keratosis B. Atopic dermatitis C. Cutaneous T-cell lymphoma D. Psoriasis E. Tinea manuum The most likely diagnosis is:

50 Look for similar lesions on sun-exposed areas Advanced actinic keratoses

51 Rough, sandpaper texture, sometimes better felt than seen

52 Many clinical variants with colors ranging from flesh-colored to pink to brown

53 Prominent horn may develop; AKs may progress to SCCs (biopsy may be necessary)

54 Destructive treatment options: cryotherapy, curettage

55 Topical therapy: 5-fluorouracil, imiquimod

56 Distractors: *Atopic Dermatitis

57 Distractors: *Cutaneous T-cell Lymphoma

58 Distractors: *Psoriasis

59 Distractors: *Tinea Manuum

60 This developmentally normal patient presents to you for cosmetic removal of these lesions. What is the most likely diagnosis?: A. Hidrocystoma B. Sebaceous carcinomas C. Syringomas D. Sebaceous hyperplasia E. Tricholemmomas

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62 This developmentally normal patient presents to you for cosmetic removal of these lesions. What is the most likely diagnosis? A. Hidrocystoma B. Xanthelasma C. Syringomas D. Sebaceous hyperplasia E. Tricholemmomas

63 Syringomas Benign eccrine neoplasm May be a/c Down s syndrome Localized periorbital m/c Eruptive Familial

64 Distractors Sebaceous hyperplasia Colonoscopy Muir Torre Syndrome Sebaceous neoplasms, KAs GI, GU malignancies

65 Incorrect Choice- Xanthelasma Generally larger and planar Check lipid panel Associated with type II and IV familial hyperlipidemia

66 Incorrect Choice - *Tricholemmoma Fleshy papules favoring perinasal areas Associated with Cowden s syndrome (PTEN mutation) risk breast, thyroid, uterine, GI, GU cancer Check mammogram, colonoscopy

67 Distractor - Hidrocystoma Cystic papule Normally solitary Most common location is head and neck

68 The most likely diagnosis is: A. Angiosarcoma B. Masson lesion (intravascular endothelial hyperplasia) C. Minocycline hyperpigmentation D. Pigmented basal cell cancer E. Pyogenic granuloma

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70 The most likely diagnosis is: A. Angiosarcoma B. Masson lesion (intravascular endothelial hyperplasia) C. Minocycline hyperpigmentation D. Pigmented basal cell cancer E. Pyogenic granuloma

71 Angiosarcoma Uncommon malignant neoplasm of endothelium (<1% of all sarcomas) Unlike most sarcomas, has a predilection for skin and superficial soft tissue Most commonly affects the scalp and face of elderly and areas of chronic lymphedema or radiodermatitis; rare in children Prognosis is extremely poor, with fewer than 15% surviving 5 years Treatment: surgical excision with wide margins (recurrence and metastases common) Chemotherapy and radical radiotherapy may be palliative, but do not improve survival

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73 Angiosarcoma: 50% occur on head and neck; facial swelling and edema may be present

74 Advanced angiosarcoma may develop nodules and ulcerate

75 Distractors: Minocycline Hyperpigmentation

76 Distractors: Pigmented *Basal Cell Carcinoma

77 The most likely diagnosis is: A. Amelanotic melanoma B. Basal cell cancer C. Chondrodermatitis nodularis helicis D. Gouty tophus E. Squamous cell cancer

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79 The most likely diagnosis is: A. Amelanotic melanoma B. Basal cell cancer C. Chondrodermatitis nodularis helicis D. Gouty tophus E. Squamous cell cancer

80 Chondrodermatitis nodularis helicis Exquisitely tender nodules on the helix and antihelix of ear Risk factors: cold exposure, significant sun damage, and trauma Treatment Excision of inflamed cartilage most effective Other options Steroids (topical and intralesional) Offloading of pressure Cryosurgery CO 2 laser ablation

81 Distractors: Amelanotic *Melanoma

82 Distractors: *Basal cell cancer

83 Distractors: Gouty tophus

84 The most likely diagnosis is: A. Amelanotic melanoma B. Basal cell cancer C. Dermal nevus D. Dermatofibroma E. Squamous cell cancer

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86 The most likely diagnosis is: A. Amelanotic melanoma B. Basal cell cancer C. Dermal nevus D. Dermatofibroma E. Squamous cell cancer

87 Dermatofibroma More common on lower extremities, but may arise anywhere

88 Commonly hyperpigmented, but may appear pink in light-skinned individuals

89 Firm to palpation; pinching the lesion may result in dimple sign

90 Dermatofibromas Many histologic variants exist e.g., atrophic, palisading, clear cell, xanthomatous, with granular cells Treatment Observation is appropriate Excision if removal desired

91 This patient has other family members with similar lesions. A. Verruca plana B. Angiofibromas C. Molluscum contagiosum D. Trichoepitheliomas E. Fibrofolliculoma Larger image on next slide Vincent A. et al. JAAD 2003;49(4):

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93 The most likely diagnosis is: A. Verruca plana B. Angiofibromas C. Molluscum contagiosum D. Trichoepitheliomas E. Fibrofolliculoma

94 Trichoepithelioma Benign adnexal neoplasm Associated with Familial trichoepithelioma Brooke-Spiegler syndrome mutation in cylindroma oncogene: cylindroma, trichoepithelioma, and spiradenoma Rombo syndrome: vermiculate atrophoderma, milia, trichoepithelioma, basal cell carcinoma Lesions are skin colored firm papules Located on the nasolabial folds, nose, and upper lip

95 *Angiofibroma Associated with *tuberous sclerosis Pinkish/telangiectatic papules More centrally located on the nose rather than nasolabial folds Fibrofolliculoma Small white dome-shaped papules Associated with *Birt-Hogg-Dube AD Recurrent spontaneous pneumothorax Increase risk of malignant renal tumor

96 Trichoepithelioma Brooke-Spiegler Skin colored, firm, on NLF *Angiofibroma *Tuberous Sclerosis Pink, telangiectatic papules Vincent A. et al. Birt-Hogg-Dubé syndrome: A review of the literature and the differential diagnosis of firm facial papules. JAAD 2003;49(4): Fibrofolliculoma *Birt-Hogg-Dube White smooth papules

97 Verruca Plana (*wart) less symmetrically distributed discrete slightly raised flap top papules with fine verrucous surface *Molluscum contagiosum less symmetrically distributed Discrete papules with central umbilication

98 The most likely diagnosis is: A. Acral melanoma B. Dysplastic nevus C. Pigmented actinic keratosis D. Pigmented purpuric dermatosis E. Talon noir Location: heel of foot

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100 The most likely diagnosis is: A. Acral melanoma B. Dysplastic nevus C. Pigmented actinic keratosis D. Pigmented purpuric dermatosis E. Talon noir Location: heel of foot

101 Talon noir Also known as black heel Post-traumatic hemorrhage in the stratum corneum seen most often in athletes Thought to occur from shearing forces that rupture papillary dermal blood vessels with leakage into epidermis Paring of stratum corneum reveals re-brown specks of dried blood, distinguishing it from a melanocytic lesion

102 Distractors: Acral *melanoma

103 Distractors: Pigmented Purpuric Dermatosis

104 The patient shown has a history of seizures. What is the most likely diagnosis of the cutaneous lesions? a) Trichoepitheliomas b) Neurofibromas c) Trichodiscomas d) Angiofibromas e) Fibrofolliculomas

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106 The patient shown has a history of seizures. What is the most likely diagnosis of the cutaneous lesions? a) Trichoepitheliomas b) Neurofibromas c) Trichodiscomas d) Angiofibromas e) Fibrofolliculomas

107 Angiofibromas Benign and common Commonly located on central face Path shows dermal fibrosis and dilated vessels Associated with tuberous sclerosis History of seizures provided suggests *TS

108 Distractors *Neurofibromas More fleshy Umbilicate with pressure Associated with NF *Trichoepitheliomas Also flesh colored papules frequently around nose Tend to be a little larger Associated several syndromes Trichodiscomas and fibrofolliculomas Flat topped flesh colored papules around nose Associated with *Birt-Hogg-Dube

109 The most likely diagnosis is: A. Dermatofibroma B. Dermatofibrosarcoma protuberans C. Epidermoid cyst D. Keloid E. Melanoma

110 The most likely diagnosis is

111 A. Dermatofibroma B. Dermatofibrosarcoma protuberans C. Epidermoid cyst D. Keloid E. Melanoma The most likely diagnosis is:

112 DFSP Soft tissue sarcoma, young to middle-aged adults Plaque/multinodular and locally aggressive Fibrosarcomatous transformation - bad Spindled cells in storiform pattern CD34(+), factor XIIIa(-) (differentiates from DF) Pigmented variant = Bednar tumor

113 The most likely diagnosis is

114 The most likely diagnosis is: A. Dermatofibroma B. Dermatofibrosarcoma protuberans C. Epidermoid cyst D. Keloid E. Melanoma

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116 The most likely diagnosis is: A. Dermatofibroma B. Dermatofibrosarcoma protuberans C. Epidermoid cyst D. Keloid E. Melanoma

117 Keloid vs. Hypertrophic Scar Extend beyond the edge of the original site

118 Keloid vs. Hypertrophic Scar May regress centrally but maintain an active border

119 Keloid vs. *Hypertrophic Scar Do not extend beyond the surgical site or have central regression

120 The most likely diagnosis is: A. Keratoacanthoma B. Metastatic carcinoma C. Prurigo nodule D. Sporotrichosis E. Verrucous carcinoma Courtesy Dr. C. Travelute

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122 The most likely diagnosis is: A. Keratoacanthoma B. Metastatic carcinoma C. Prurigo nodule D. Sporotrichosis E. Verrucous carcinoma Courtesy Dr. C. Travelute

123 SCC variant Keratoacanthoma Rapidly growing, spontaneous regression Solitary dome-shaped nodule with central crater or keratin plug Face, neck, upper extremity

124 Keratoacanthoma

125 Keratoacanthoma

126 Keratoacanthoma Ferguson-Smith syndrome Multiple familial KAs in early adulthood AD Grzybowski type Generalized eruptive KAs

127 Keratoacanthoma Can be part of Muir-Torre syndrome Sebaceous neoplasms, KAs, and GI cancers

128 Distractors: Metastatic Carcinoma

129 Distractors: *Prurigo Nodularis

130 Distractors: *Sporotrichosis

131 The patient presents for evaluation of the lesion shown below. Review of systems reveals a history of dysphagia as well as digital pain on cold exposure. Physical exam is significant for telangiectasias. What is the most likely diagnosis of the lesion shown? A) Calcinosis Cutis B) Pilomatrixoma C) Keratoacanthoma D) Pilar cyst E) Osteoma cutis Journal of the American Academy of Dermatology , 1-12DOI: ( /j.jaad )

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133 The patient presents for evaluation of the lesion shown below. Review of systems reveals a history of dysphagia as well as digital pain on cold exposure. Physical exam is significant for telangiectasias. What is the most likely diagnosis of the lesion shown? A) Calcinosis Cutis B) Pilomatrixoma C) Keratoacanthoma D) Pilar cyst E) Osteoma cutis Journal of the American Academy of Dermatology , 1-12DOI: ( /j.jaad )

134 Calcinosis Cutis Pathogenesis deposition of insoluble calcium salts in skin Associated with variety of conditions Occurs in setting of several connective tissue diseases CREST syndrome (described in question stem) Systemic sclerosis Dermatomyositis Cutaneous calcium deposits appear, hard, white nodular. Frequently ulcerate Often present on fingers and elbows in CTD

135 Distractors Pilomatrixoma Firm cystic papule in hair bearing area + rocker bottom sign *Keratoacanthoma Rapidly growing keratotic nodule Favor head, neck, and extremities

136 Distractors Pilar cyst Soft cystic mass on scalp Osteoma cutis Most commonly presents with multiple small flesh colored papules on cheeks Can be milia-like