II. Epidemiology
- Incidence: Very rare
- Gender predominence: Female to male ratio of 2:1
- Age of onset: 60 years
III. Pathophysiology
- Autoantibodies to Desmoplakin 1 and 3
-
Lymphoid malignancy associated Pemphigus
- Lymphoma
- Thymoma
- Chronic Lymphocytic Leukemia
- Waldenstrom's Macroglobulinemia
- Sarcoma
- Castleman's Disease
IV. Signs
- Diffuse, painful bullous lesions
- Severe mucosal erosions in oropharynx and Conjunctiva
- Polymorphic target-like skin lesions
V. Differential Diagnosis
VI. Lab
- Histology
- Suprabasilar Acantholysis
- Dyskeratotic cells
- Basal cell vacuolization
- Immunofluorescence
- IgG and C3 on Keratinocyte cell surface
VII. Prognosis
- Poor prognosis often with rapidly fatal course
VIII. 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]
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Related Studies
Concepts | Neoplastic Process (T191) |
ICD10 | L10.81 |
LNC | LP94506-0 |
English | Paraneoplastic pemphigus, paraneoplastic pemphigus, paraneoplastic pemphigus (diagnosis), pemphigus paraneoplastic |
Dutch | paraneoplastische pemphigus |
French | Pemphigus paranéoplasique |
German | paraneoplastischer Pemphigus |
Italian | Pemfigo paraneoplastico |
Portuguese | Pênfigo paraneoplásico |
Spanish | Pénfigo paraneoplásico |
Japanese | 腫瘍随伴性天疱瘡, シュヨウズイハンセイテンポウソウ |
Czech | Paraneoplastický pemfigus |
Hungarian | Paraneoplasiás pemphigus |