Av. Marzano et al., Paraneoplastic pemphigus. A report of two cases associated with chronic B-cell lymphocytic leukaemia, BR J DERM, 145(1), 2001, pp. 127-131
Paraneoplastic pemphigus (PNP) is an autoimmune blistering and erosive muco
cutaneous disease associated with neoplasia. Clinical manifestations are po
lymorphous, and include erythema, bullae, erythema multiforme-like lesions
and severe mucous membrane involvement. PNP manifesting as lichenoid dermat
itis has recently been observed. We describe two Italian men with fatal PNP
featuring typical PNP autoantigens associated with chronic B-cell lymphocy
tic leukaemia. The first patient presented with an extensive blistering eru
ption, several erythema multiforme-like lesions and severe mucosal involvem
ent. The second patient presented with a lichenoid dermatitis, then develop
ed bullae, and died with an erythrodermic and exfoliative dermatosis resemb
ling pemphigus foliaceus. Our patients represent two Italian cases of well-
documented PNP. In patient 2, the sequence of clinical presentations was un
ique, and strongly supports the hypothesis of epitope spreading through chr
onic lichenoid inflammation of the dermo-epidermal junction exposing new se
lf antigens, leading to the humoral response characteristic of PNP.