A 52-year-old man presents with severe painful stomatitis, conjunctival injection, and polymorphic lichenoid papules and blisters on the trunk and palms. He has known chronic lymphocytic leukemia. Biopsy shows suprabasal acantholysis with necrotic keratinocytes. The autoantibodies in this condition are directed primarily against which proteins?
- A Plakin family proteins such as envoplakin and periplakin ✓
- B Desmogleins 1 and 3 only
- C Type IV collagen of the basal lamina
- D Transglutaminase 3 in the papillary dermis
Correct answer: A. Plakin family proteins such as envoplakin and periplakin
Explanation
Paraneoplastic pemphigus, classically associated with non-Hodgkin lymphoma and CLL, features intractable mucosal ulceration with a polymorphic cutaneous eruption and antibodies against plakin family proteins including envoplakin, periplakin, and desmoplakin, often with additional antidesmoglein antibodies. Desmoglein-only reactivity defines pemphigus vulgaris and foliaceus, and transglutaminase antibodies belong to dermatitis herpetiformis.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.