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Fitzpatrick's Dermatology in General Medicine, 7e
| Part 2. Disorders Presenting in Skin and Mucous Membranes > Section 8. Disorders of Epidermal and Dermal-Epidermal Cohesion and Vesicular and Bullous Disorders > |
Chapter 53. Paraneoplastic Pemphigus
Sections: Paraneoplastic Pemphigus: Introduction, Epidemiology, Etiology and Pathogenesis, Clinical Findings, Laboratory Tests, Diagnosis and Differential Diagnosis, Prognosis and Clinical Course, Treatment, Prevention, References.
Topics Discussed: paraneoplastic pemphigus.
Excerpt:
"Paraneoplastic pemphigus (PNP) is an autoimmune disorder that
is almost always linked to an underlying lymphoproliferative disorder.
These features define it: (1) Painful stomatitis and a polymorphous
cutaneous eruption with lesions that may be blistering, lichenoid,
or resemble erythema multiforme. (2) Histologic findings that reflect
the variability of the cutaneous lesions, showing acantholysis,
lichenoid, or interface change. (3) Direct immunofluorescence demonstrating
deposition of immunoglobulin G (IgG) and complement in the epidermal
intercellular spaces, and often granular/linear complement
deposition along the epidermal basement membrane zone. (4) Serum
autoantibodies that bind the cell surface of skin and mucosae in
a pattern typical for pemphigus, but in addition, bind to simple,
columnar, and transitional epithelia. (5) These serum autoantibodies
identify desmogleins 1 and 3, but additionally identify members
of the plakin family of epithelial proteins, such as desmoplakins,
envoplakin, and periplakin.1 The disease is associated,
in the majority of cases, with non-Hodgkin lymphoma (NHL), chronic lymphocytic
leukemia (CLL), and Castleman disease. There is no regularly effective
treatment and most patients..."
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