Self assesment Case 21

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1 17-18 MAY 2018 London Dermatopathology Symposium 2018 Self assesment Case 21 MARC HASPESLAGH

2 CASE year old lady with eczematous lesions at ear helix and red patch on nose bridge since 3 years Discoid Lupus? Sarcoidosis? TBC?

3

4 CD123 L K

5

6 Diagnosis Lupus Discoid lupus Hypertrophic discoid lupus Discoid lupus with amyloidosis Hyperthrophic discoid lupus with secondary amyloidosis

7 Localised Amyloid in Skin Primary as in lichen or macular amyloidosis and secondary as clinically unapparent phenomenon Secundary associated with tumors of epithelial origin or inflammatory diseases often linked to chronic UV exposition Amyloid K from epidermal origin

8 Amyloid in skin Described rarely in lupus, mainly hypertrophic lupus, Probably chronic UV exposure pathogenic role Keratin origin with CK+ Papillary dermis along basement membrane

9 34be12 AE1AE3 CK5

10 Case 21 : meaning of perivascular presence and keratin negativity? Secondary systemic in chronic inflammatory disease?

11 Self assesment Case 22

12 CASE year old lady with six flat papules on vulva since many months Viral? Condylomas? Bowenoid papulosis? Lichen?

13

14 Diagnosis Acanthoma Condyloma Acantholytic acanthoma Multiple epidermolytic acanthoma Acantholytic dyskeratoma

15 Epidermolytic hyperkeratosis Histologic phenomenon : hyperkeratosis, hypergranulisis, perinuclear vacuolisation, reticular degeneration in granular and spinous layer Formation of amorphous, eosinophilic trichohyaline granules From generalised genetic disease in bullous ichtyosiform erythrodermia to acquired and localised aberration Keratin 1 and 10 mutations

16 Epidermolytic Acanthoma Epidermolytic acanthoma : benign tumor usually presenting as solitary, asymptomatic papule Multiple Epidermolytic Acantomas : very rare, genitocrural areas Mimicker of condylomas : older population, labia majora (scrotum in males) No HPV association

17 Lesson learned The same morfological pattern can occur in totally different diseases To classify correctly these patterns clinicopathological correlation is indespensable

18 Self assesment Case 23

19 CASE year old girl with hyperkeratotic naevoid lesion in sacral region since birth

20

21 f 50y onderbeen re M 87 onderb dsapk F 60 y arm voor re male 70y dsap + lich ker F 53 arm naevus en pk

22

23

24 Diagnosis Neurodermatitis Psoriasis Verrucous hyperplasia Bowen s disease Verrucous plaque type porokeratosis

25 Lessons learned Blind sectioning and cutting can fool the pathologist EVD can save time, money and mental and emotional energy from the pathologist and technicians

26 Self assesment Case 24

27 CASE Male of 78 years old with purpuric lesion on actinic damaged scalp. Takes anti-thrombotic medication

28

29 Keratin S100 CD34 CD31

30 Histology Anaplastic sarcomatous proliferation Fibrotic spindled, cellular cuboidal and acantholytic split like or sinusoidal areas CK- not pseudosarcomatous squamous, S100- not melanoma, angio sarcoma? : CD34 also CD31- CD10 + Similar to previous biopsy and immuno findings : again compatible with fragment from AFX?

31 24/9/2017 Telefone call from referring dermatologist : recidivating and progressive circular purpuric lesions around scars of previous lesions Clinical story is not compatible with AFX!!!!! Revision and additional stains

32

33 D2-40

34 ERG C-MYC

35

36 Diagnosis AFX Sarcoma NOS Angiosarcoma Malignant Fibrous Histiocytoma Pseudosarcomatous Squamous Cell Carcinoma

37 Poorly differentiated angiosarcoma of the face and scalp in elderly patients DD with Melanoma and AFX can be difficult in solid areas! Vacuoles are clue Misleading immunos : CD34 - CD31-20 % of cases CD10 + Differential : D2-40 +, ERG +, C-myc + 40% of cases

38 lessons Clinical story! Erythematous maculae resembling hematoma in face or scalp elderly : Pannel of antibodies When a lesion recurs be cautious with previous interpretations

39 Self assesment Case 25

40 CASE Lady of 25 years old with erythematous infiltrated macula on central lumbar region since one month. Pseudolymphoma? Dermatofibroma? Malignant?

41

42

43

44

45 SMA D2-40 CD 31 CD 34

46 Diagnosis Angiomatosis Tufted angioma Hemangioma Microvenular angioma angiosarcoma

47 Microvenular Angioma Slow growing solitary asymptomatic plaque-like lesion Upper extremities, forearm, face, neck, trunk, lower extremities D2-40 neg CD31 +,CD34+ Pseudoinfiltrative growth DD Kaposi

48 Lesson learned Rare tumors do occur Not all what infiltrates is malignant!

49 THANK YOU

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