TY - JOUR
T1 - Paraneoplastic pemphigus. A report of two cases associated with chronic B-cell lymphocytic leukaemia
AU - Marzano, A. V.
AU - Grammatica, A.
AU - Cozzani, E.
AU - Terracina, M.
AU - Berti, E.
PY - 2001
Y1 - 2001
N2 - Paraneoplastic pemphigus (PNP) is an autoimmune blistering and erosive mucocutaneous disease associated with neoplasia. Clinical manifestations are polymorphous, and include erythema, bullae, erythema multiforme-like lesions and severe mucous membrane involvement. PNP manifesting as lichenoid dermatitis has recently been observed. We describe two Italian men with fatal PNP featuring typical PNP autoantigens associated with chronic B-cell lymphocytic leukaemia. The first patient presented with an extensive blistering eruption, several erythema multiforme-like lesions and severe mucosal involvement. The second patient presented with a lichenoid dermatitis, then developed bullae, and died with an erythrodermic and exfoliative dermatosis resembling pemphigus foliaceus. Our patients represent two Italian cases of well-documented PNP. In patient 2, the sequence of clinical presentations was unique, and strongly supports the hypothesis of epitope spreading through chronic lichenoid inflammation of the dermo-epidermal junction exposing new self antigens, leading to the humoral response characteristic of PNP.
AB - Paraneoplastic pemphigus (PNP) is an autoimmune blistering and erosive mucocutaneous disease associated with neoplasia. Clinical manifestations are polymorphous, and include erythema, bullae, erythema multiforme-like lesions and severe mucous membrane involvement. PNP manifesting as lichenoid dermatitis has recently been observed. We describe two Italian men with fatal PNP featuring typical PNP autoantigens associated with chronic B-cell lymphocytic leukaemia. The first patient presented with an extensive blistering eruption, several erythema multiforme-like lesions and severe mucosal involvement. The second patient presented with a lichenoid dermatitis, then developed bullae, and died with an erythrodermic and exfoliative dermatosis resembling pemphigus foliaceus. Our patients represent two Italian cases of well-documented PNP. In patient 2, the sequence of clinical presentations was unique, and strongly supports the hypothesis of epitope spreading through chronic lichenoid inflammation of the dermo-epidermal junction exposing new self antigens, leading to the humoral response characteristic of PNP.
KW - Chronic B-cell lymphocytic leukaemia
KW - Epitope spreading
KW - Paraneoplastic pemphigus
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U2 - 10.1046/j.1365-2133.2001.04296.x
DO - 10.1046/j.1365-2133.2001.04296.x
M3 - Article
C2 - 11453921
AN - SCOPUS:0034832661
VL - 145
SP - 127
EP - 131
JO - British Journal of Dermatology
JF - British Journal of Dermatology
SN - 0007-0963
IS - 1
ER -