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Paraneoplastic Pemphigus (pp.205-220) $100.00
Authors:  (Angeline Yong, Hong Liang Tey, National Skin Centre, Singapore)
Abstract:
Paraneoplastic pemphigus (PNP) is a chronic blistering mucocutaneous disease which is almost always associated with an underlying neoplasm, both malignant and benign. The most commonly associated tumors are lymphoproliferative neoplasms, including Castlemanís disease, which is the most commonly associated tumor in younger people. The clinical presentation of PNP comprises typically of painful, severe oral erosions which may be accompanied by a generalized cutaneous eruption and systemic involvement.
The eruption can be of different morphology, consisting of pemphigus-like, pemphigoid-like, erythema multiforme-like, graft versus host disease-like, as well as lichen planus-like lesions. The histological findings similarly show considerable variability, comprising of pemphigus vulgaris-like, lichen planus-like, or erythema multiforme-like features. The most characteristic histological feature, nevertheless, is the presence of vesicular lesions. Other suggestive features include suprabasal acantholysis with scattered necrotic keratinocytes and vacuolar interface changes.
PNP is characterized by the presence of auto-antibodies against a unique complex of keratinocyte cell surface protein of the plakin family, consisting of desmoplakin I (250 kd), bullous pemphigoid antigen I (230 kd), desmoplakin II (210 kd), envoplakin (210 kd), periplakin (190 kd), plectin (500 kd), and an unidentified 170-kd protein. PNP has a poor prognosis and is often fatal.
The prognosis seems to be better, however, when the disease is associated with benign tumors and these tumors should be surgically excised. The treatment for patients with malignant neoplasms is difficult and the aim is largely supportive. Immunosuppressive agents are required to decrease blistering but often yield poor results. Treating the underlying malignancy with chemotherapy may control autoantibody production, but whilst cutaneous lesions may respond more rapidly, stomatitis is usually more refractory to most treatment. 


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Paraneoplastic Pemphigus (pp.205-220)