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Path Quiz Case- 29
Dr Sampurna Roy
MD
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Cutaneous Deposits
Cutaneous Ossification (Osteoma Cutis) Role of immunohistochemistry in Dermatopathology Interstitial Granulomatous Dermatitis |
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Skin Tumours
Skin Adnexal (Appendage) Tumours Syringocystadenoma Papilliferum Classification of Malignant Sweat Gland Tumours Digital papillary adenocarcinoma |
Hailey-Hailey disease (familial benign chronic pemphigus)) is an autosomal dominant disorder with recurrent eruption of vesicles and bullae. It is caused by mutations in the ATP2C1 gene encoding a novel Ca(2+) pump. The blistering dermatitis is characterized by acantholytic cells present in foci throughout at least half of the thickness of the epidermis. Site: Predominantly involve the neck, groin , inframammary, perianal and axillary regions. Rarely oral, ocular, esophageal and vaginal cases have been reported Age: Usually occurs in the second or third decade of life. Clinical presentation: Usually presents as well demarcated plaques. Rare variants: Papular, annular, verrucous, and vesiculopustular variants. Course: This chronic disease is characterized by spontaneous remission with subsequent exacerbation. Histopathological
features:
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Link1 Histologically Hailey-Hailey Disease has a characteristic 'dilapidated brick wall appearance'. Early: Superficial perivascular infiltrate of mainly lymphocytes ; Changes are located in focal areas in the lower half of the epidermis ; Vacuoles are present between basal cells and spinous cells and between spinous cells . Fully developed: Moderately dense perivascular and interstitial infiltrate of lymphocytes ; Edema of the papillary dermis ; Acanthosis ; Suprabasal blister ; Acantholytic dyskeratotic cells, some with polygonal outlines, above the basal layer either singly or in clumps lining the clefts ; Present throughout at least the lower half of the epidermis ; Infundibular epidermis and epithelial structures of adnexa spared by the acantholytic process ; Scale-crusts ; Neutrophils are sometimes numerous within the vesicle or in the surface crust. Late: All the features of fully developed lesions with the addition of a mixed-cell infiltrate, acantholytic cells increasingly dyskeratotic, and more prominent scale-crusts. Differential diagnosis: Hailey-Hailey variant of Grover's disease: Narrow vesicle involving not more than a few rete ridges. Hailey-Hailey disease is more florid e.g., more confluence, more markedly thickened epidermis, more acantholytic cells, more dyskeratotic cells with polygonal outlines, more vesiculation, and more scale-crusts. Pemphigus vulgaris: Less acantholysis & acantholytic cells are confined to the suprabasal region; some cells show more prominent dyskeratosis ; epidermis of relatively normal thickness ; infundibular epidermis and epithelial structures of adnexa are involved by the process ; there are no scale-crusts. |
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Histopathology-India.net diagnostichistopathology. blogspot.com Environmental Pathology Online
E-book - History of Medicine with special reference to India. |
Inflammatory
diseases of the Eyelid
Tumour and tumour-like lesions of the Eyelid
Epidermal
tumours of the Eyelid
Classification
of Soft Tissue Tumour
Gross examination of soft tissue specimen
A practical approach to
histopathological reporting of soft tissue tumours
Grading of soft tissue tumours
Fibroblastic/Myofibroblastic tumours
Annular
Elastolytic
Giant Cell Granuloma
Pathology of the Eyelid
Myxoid
Tumours of Soft Tissue
Granulomatous Reaction Pattern of the Skin