BSD SELF-ASSESSMENT CASES 21-24

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1 BSD SELF-ASSESSMENT CASES EDINBURGH, 2 JULY 2018 LASZLO IGALI

2 CASE 21 CLINICAL HISTORY Female, 43 years, nodule on left side of face/jaw angle area. Slow growth over years. MACRO (NOT GIVEN) Skin coloured nodule, up to 8 mm, contained within a punch biopsy. Single slice of the punch taken.

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11 S100 EMA

12 CASE 21 DIFFERENTIALS Schwannoma - unocmmon in dermis, complete perineual capsule, can be GFAP positive, could have Antoni A and B areas neurofibroma - no capsule, can be GFAP positive, frequent mast cells, neural fibroblasts and fibrillary collagen (CD34 and polarised light), can be associated with NF leiomyioma - S100 vs SMA/desmin positivity palisaded encapsulated neuroma - solitary, S100 pos schwann cells, weak peripheral EMA pos capsule, no Antoni A/B areas

13 CASE 21 HISTOLOGY FEATURES OF THIS CASE usually solitary, dermal or subcutaneous/submucosal, could be multifocal or plexiform (hyalinised vessels) poor/partial encapsulation retraction artefact predominantly spindled schwann cells - micro-bundles only vague palisading 90% - face, lips, oral cavity

14 CASE 21 PREFERRED DIAGNOSIS palisaded encapsulated neuroma

15 CASE 22 CLINICAL HISTORY Male, 53, nodule on scalp MACRO (NOT GIVEN) Skin ellipse - 2.4x1/8 cm, with a central depression up to 0.5 cm diameter with slightly raised edge and?keratotic debris in the centre.

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24 CASE 22 DIFFERENTIALS Hailey-Hailey disease - follicular syskeratosis, AD, 2-3rd decade, intertriginous sites, hspca1(golgi ATPase - Ca signalling); dilapidated brick wall Darier s disease - follicular dyskeratosis, AD, 2nd decde, seborrhoeic and acral distribution, SERCA2 (endoplasmic reticulum ATPase - Ca signalling), corps ronds, grains Grover s disease - can be similar to either (and other acanthilytic diseases), 6th decade or later, male predominance, transient Warty dyskeratoma - isolated follicular keratosis, head-neck, related to actinic keratosis or is it follicular neoplasia, Hellwig - isolated Darier s disease, no isolated reproducible molecular defect

25 CASE 22 DermatologyEdited by Jean L.BologniaJulie V.SchafferLorenzoCerroniFourth editionchina: Elsevier, 2018,

26 CASE 22 DermatologyEdited by Jean L.BologniaJulie V.SchafferLorenzoCerroniFourth editionchina: Elsevier, 2018,

27 CASE 22 PREFERRED DIAGNOSIS warty dykeratoma

28 CASE 23 CLINICAL HISTORY Male 33, blistering and crusting on back of the hands. Worse at summer. MACRO (NOT GIVEN) 6 mm punch biopsy with unremarkable epidermis, bisected.

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37 CASE 23 DIFFERENTIALS hidroa vacciniforme - photo-aggravated /photodermatosis - childhood -first 2 decades, epidermal necrosis,?? EBV PCT - subepidermal blister, festooning, thinkened vessel walls (igg/m deposition in walls, woods light, urine red->brown if exposed to light, porphyrin screen ) EBA - mechanobullous disease, similar histology, blood tests, IMF Bullous lupus - can have identical picture, Ig deposition,

38 CASE 23 DermatologyEdited by Jean L.BologniaJulie V.SchafferLorenzoCerroniFourth editionchina: Elsevier, 2018,

39 CASE 23 DIAGNOSIS porphyria cutanea tarda

40 CASE 24 CLINICAL HISTORY Female, 70, extensive erythema on chest. MACRO (NOT GIVEN) 4 mm punch biopsy with unremarkable epidermis, bisected.

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50 CASE 24 DIFFERENTIALS Scleredema - may be associated with diabtetes (late onset type1, male), thickened collagen +/- interstitial mucin Lupus erythematosus (?tumid) - could be plaque/pacth like, DIF positive, serology positive, tumid LE us photo-inducible, response to antimalarials REM - 3-4th decade of life, plaques/patches -in midline of chest/back, serology negative, could represenet ff of LE? Polymorphous light eruption - photodermatosis, mainly papules, rarely plaques, oedema, spongiosis, perivascular lymphoid infiltrate

51 CASE 24 PREFERRED DIAGNOSIS Reticulate erythematous mucinosis (REM)

52 THANKS FOR YOUR ATTENTION

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