Path Quiz Case- 41
Dr Sampurna Roy
MD
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Surgical-Pathology.com
Environmental Pathology Online
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Bullous pemphigoid first described in 1953, is a chronic subepidermal blistering disease. Age: Most commonly occurs in elderly persons ( in the seventh or eighth decade of life) but occasionally is seen in young adults and even in children. Site: Lower part of abdomen, inner part of thighs, flexor surface of of the arm and leg , axillae, groin, and palmar and plantar surfaces; oral lesions have been reported ; Other mucosal surfaces are usually not involved. Clinical presentation: Multiple tense bulla on normal or erythematous skin of variable size . May present as erythematous macules, verrucous and vegetating lesions, urticarial plaques & crusted erosions, or annular lesions. Cause of the disease: Caused by autoantibody-mediated disruption of adhesion between basal keratocytes and the basement membrane, i.e., antibodies there bind the antigens of bullous pemphigoid. Antibodies against two hemidesmosomal components, BP antigen 2 (180 KDa) and BP antigen 1 (230 KDa), have been detected in sera of the patients. Circulating antibodies against the BP180 component are sufficient to produce a subepidermal blister. Triggering factors: Trauma ; burns ; ultraviolet irradiation, Shortly after vaccination; Drugs: penicillin derivatives, antibiotics , ibuprofen , other non-steroidal anti-inflammatory drugs, antipsychotic drugs. May occur in association with other diseases: Eg. Rheumatoid arthritis, lichen planus, psoriasis, systemic lupus erythematosus, diabetes mellitus, primary biliary cirrhosis , ulcerative colitis. etc.
Histolopathological
features:
Image1 Diagnostic clue: A subepidermal blister containing numerous eosinophils ; Scattering of eosinophils, mostly as solitary units in the epidermis and across a broad front of it is a clue to the urticarial stage of bullous pemphigoid/herpes gestationis ; "Flame figures" (also present in response to the assault of an arthropod. Early (urticarial)- Perivascular and interstitial infiltrate of lymphocytes and eosinophils ; Eosinophils scattered throughout the upper part of the reticular dermis, in the edematous papillary dermis, and along the dermoepidermal junction; Eosinophils may be present in tiny collections together with spongiosis Fully developed (vesicular)- Perivascular and interstitial infiltrate of lymphocytes and many eosinophils ; Numerous eosinophils throughout the edematous upper dermis; Subepidermal blister within which numerous eosinophils usually are present ; dermal papillae often are preserved ; spongiosis is often present at the sides of the blister. Late (bullous) - All the changes seen in developed fully lesions ; together with sometimes of spongiotic vesicles and edema of the papillary dermis, so prominent at times that it forms subepidermal vesiculation ; Re-epithelialization from infundibular and eccrine ductal epithelium is noted in some lesions Rarely other histological changes may be present: Cell-poor type ; an intraepidermal blister occurs in conjunction with a subepidermal blister; Presence of an infiltrate of lymphocytes and plasma cells ; Presence of predominantly neutrophils.
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Clinical Images:(DermAtlas)
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Skin Tumours
Skin Adnexal (Appendage) Tumours Syringocystadenoma Papilliferum Classification of Malignant Sweat Gland Tumours Digital papillary adenocarcinoma Folliculosebaceous Cystic Hamartoma |
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Cutaneous lesion in
graft- versus host disease |