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1st EMIRATES PATHOLOGISTS MEET 2009-2010 SHARJAH UNIVERSITY

CASE - 1. 43 yrs old, Indian Male patient.H/o burning, irritation in Groin - 2 mths.Started with erosions and macerations.Treated with anti-fungal and antibiotics.Symptoms recurred at the same site.Punch Biopsy from Groin.Tissue bisected, both halves submitted.. Clinical pictures of a similar case.

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1st EMIRATES PATHOLOGISTS MEET 2009-2010 SHARJAH UNIVERSITY

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    1. 1st EMIRATES PATHOLOGISTS MEET 2009-2010 SHARJAH UNIVERSITY Dr Chandrakala Das, Specialist Histopathologist, Zulekha Hospital, Dubai.

    2. CASE - 1 43 yrs old, Indian Male patient. H/o burning, irritation in Groin - 2 mths. Started with erosions and macerations. Treated with anti-fungal and antibiotics. Symptoms recurred at the same site. Punch Biopsy from Groin. Tissue bisected, both halves submitted.

    3. Clinical pictures of a similar case

    4. HISTOPATHOLOGICAL FEATURES

    5. HISTOPATHOLOGICAL FEATURES

    6. MORE CLINICAL DETAILS Scrotal skin was macerated with hyperkeratotic vegetative lesions. No oral lesions. Significant family history -- mother had treatment for similar lesions.

    7. DIAGNOSIS

    8. CAUSES FOR SUPRABASILAR BLISTERS Pemphigus Vulgaris Pemphigus Vegetans Paraneoplastic Pemphigus Hailey Hailey Disease Darriers Disease Grovers Disease Acantholytic Solar Keratosis

    9. PEMPHIGUS, VARIANTS AND OTHERS Acantholysis is less extensive but complete - detached round cells. Tomb stoning with single layer of cells. Vegetative lesions, transmigration of eosinophils. No erythema multiforme like picture/ no lichenoid infiltrate. No Solar elastosis or epidermal necrosis. Features present here, not favourable.

    10. STRIKING HISTOLOGICAL FEATURES Extensive suprabasilar vesicles. Incomplete, full thickness acantholysis. Dermal papillae covered by 1-3 layers. Corps ronds and grains - in horny layer. Follicular plugging. Fungal micro-organisms superficial. Dermal infiltrate is focal, perivascular, mononuclear cells.

    11. DIFFERENTIAL DIAGNOSIS Hailey Hailey Disease (Familial benign pemphigus). Grovers Disease (Transient acantholytic dermatosis). Darriers Disease (Keratosis follicularis).

    12. DARRIERS DISEASE Autosomal dominant inheritance Follicular distribution, crusted papules. Suprabasilar small lacunae limited to lower epidermis. Acantholysis is minimal. Prominent - peculiar dyskeratosis with corps ronds and grains in granular and horny layers. Follicular plugging. Dermal villi seen. Predisposition to secondary infections.

    14. GROVERS DISEASE Discrete pruritic papules, thorax / thigh. Bullae are rare. Acantholysis is focal, requiring step sections. Mixture of histological features resembling Darriers, Hailey-Hailey, Pemphigus or spongiotic dermatitis. Eosinophils in the dermis.

    15. HAILEY-HAILEY DISEASE Lacunae Suprabasal bullae Wide spread partial acantholysis with slight separation- dilapidated brick wall Cell with normal cytoplasm and nuclei, loss of intercellular bridges Some dyskeratosis, corps ronds and grains. Elongated dermal papillae DILAPIDATED BRICK WALL

    16. DIAGNOSIS HAILEY HAILEY DISEASE

    17. HAILEY HAILEY DISEASE 1sy described Hailey brothers, 1939 Rare heriditary blistering disorder Autosomal dominant inheritance Defective ATP2 C1 gene Chromosome 3q (q21-q24) Calcium transport is defective Desmosomes are less sticky Dissociation of intra and extracellular domains of adherence junction associated proteins

    18. HAILEY-HAILEY DISEASE Pruritic vesicles on erythematous base Painful erosions involving skin folds Intertriginous areas - axilla, groin Mucosal - mouth, labia, oesophagus Malodorous. Aggravated by sweat, heat, friction Family history in 1/3 cases Recurs and heals with no scarring

    19. LONGITUDINAL RIDGES AND BANDS

    20. TREATMENT Treat the symptoms, Avoid exacerbating factors Use absorbant pads Steroids / antibiotics/ antifungals Ultraviolet light / lazer vaporization Surgical excision with skin graft Lesions come and go, but does not scar.

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