Paraneoplastic pemphigus
Overview
- Association: Obligately occurs in the context of neoplasms, mostly B- and T-cell lymphomas or Castleman's tumor.
- Antigens: IgG/IgA against plakins (envoplakin, periplakin) and Desmocollin 2/3.
- Clinical features: Severe involvement of all mucous membranes (incl. conjunctivae, esophagus) as well as palms and soles; the feared complication is bronchiolitis obliterans — it largely determines the high lethality.
- Diagnostics: Besides ELISA/immunoblot for envoplakin/periplakin, indirect immunofluorescence on rat bladder epithelium (transitional epithelium) is the classic screening test — it contains plakins but no desmogleins and is therefore negative in pemphigus vulgaris.
- Classification: PNP is one of the obligate paraneoplasias — alongside erythema gyratum repens, acquired hypertrichosis lanuginosa, necrolytic migratory erythema (glucagonoma) and malignant acanthosis nigricans. Consequence: mandatory tumour search (imaging, lymph node ultrasound, haematological work-up).
Differential diagnoses
- Pemphigus vulgaris
- Pemphigus foliaceus (drug-induced, antihypertensive medication)
- Stevens-Johnson syndrome
- Mucous Membrane Pemphigoid
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