paraneoplastic pemphigus
Jump to navigation
Jump to search
Etiology
- non Hodgkin's lymphoma (42%)
- chronic lymphocytic leukemia (29%)
- Castleman's disease (10%)
- Waldenstrom's macroglobulinemia
- thymoma
Pathology
- variant of pemphigus vulgaris
- associated with underlying malignancy
- mixed pattern of suprabasal acantholysis & interface dermatitis
- direct immunofluorescence
- IgG binds in intercellular pattern within the epidermis at the dermal-epidermal junction
* histopathology image[3]
Clinical manifestations
- polymorphous skin eruption may precede diagnosis of malignancy
- confluent erythema, bullae, erosions, & intractable stomatitis
- tense & flaccid bullae may be widespread
- painful oral, conjunctival, esophageal, & laryngeal erosions common
- respiratory disorder (unclear nature of respiratory disorder)
- may be fatal[1]
- manifestations of lymphoid neoplasm may be present
Laboratory
- identification of specific autoantibodies:
- laboratory investigation for lymphoid neoplasm
- see ARUP consult[2]
Complications
- respiratory disorder can be fatal
- high mortality (up to 90%)[1]
Management
- treatment of the malignancy does not always alter the course of the skin disease
More general terms
Additional terms
References
- ↑ Jump up to: 1.0 1.1 1.2 Medical Knowledge Self Assessment Program (MKSAP) 14, 15, 16, 17, 18. American College of Physicians, Philadelphia 2006, 2009, 2012, 2015, 2018.
- ↑ Jump up to: 2.0 2.1 ARUP Consult: Paraneoplastic Pemphigus The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/paraneoplastic-pemphigus
- ↑ Jump up to: 3.0 3.1 Goldberg LJ, Elston DM (histopathology image) Medscape: Paraneoplastic Pemphigus http://emedicine.medscape.com/article/1064452-overview
- ↑ DermNet NZ. Paraneoplastic pemphigus http://www.dermnetnz.org/immune/paraneoplastic-pemphigus.html