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Dermatology · Vesiculobullous disorders: paraneoplastic pemphigus antigens
Sample itemA 62-year-old with non-Hodgkin lymphoma has intractable painful stomatitis and polymorphic skin lesions with blisters and target-like lesions. Indirect immunofluorescence on rat bladder epithelium is positive. Which antigens are characteristically targeted?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Envoplakin and periplakin
Paraneoplastic pemphigus is associated with lymphoproliferative disease, thymoma and Castleman disease, and the antibodies target plakins such as envoplakin, periplakin and desmoplakin along with desmogleins. Positive staining of rat bladder epithelium is a clue. Severe mucositis and bronchiolitis obliterans are typical.
Option by option
A. Type VII collagen
Type VII collagen is the target in epidermolysis bullosa acquisita.
B. Tissue transglutaminase
Tissue transglutaminase relates to coeliac disease and dermatitis herpetiformis via epidermal transglutaminase.
C. BP180 only
BP180 is the target in bullous pemphigoid.
D. Envoplakin and periplakin (correct)
Correct. Envoplakin and periplakin are characteristic targets.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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