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Paraneoplastic Pemphigus
Aka: Paraneoplastic Pemphigus, Neoplasia-Associated Pemphigus
- See Also
- Bullous Disease
- Pemphigus
- Epidemiology
- Incidence: Very rare
- Gender predominence: Female to male ratio of 2:1
- Age of onset: 60 years
- Pathophysiology
- Autoantibodies to Desmoplakin 1 and 3
- Lymphoid malignancy associated Pemphigus
- Lymphoma
- Thymoma
- Chronic Lymphocytic Leukemia
- Waldenstrom's Macroglobulinemia
- Sarcoma
- Castleman's Disease
- Signs
- Diffuse, painful bullous lesions
- Severe mucosal erosions in oropharynx and Conjunctiva
- Polymorphic target-like skin lesions
- Differential Diagnosis
- Erythema Multiforme
- Lab
- Histology
- Suprabasilar Acantholysis
- Dyskeratotic cells
- Basal cell vacuolization
- Immunofluorescence
- IgG and C3 on Keratinocyte cell surface
- Prognosis
- Poor prognosis often with rapidly fatal course
- References
- Parker in Goldman (2000) Cecil Medicine, p. 2285
- Bickle (2002) Am Fam Physician 65(9):1861-70 [PubMed]
- Cotell (2000) Am J Emerg Med 18(3): 288-99 [PubMed]