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paraneoplastic pemphigus
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:
- paraneoplastic pemphigus antigen
- 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
Related
paraneoplastic pemphigus antigen
General
pemphigus
paraneoplastic dermatosis
References
- Medical Knowledge Self Assessment Program (MKSAP) 14, 15, 16,
17, 18. American College of Physicians, Philadelphia 2006, 2009,
2012, 2015, 2018.
- ARUP Consult: Paraneoplastic Pemphigus
The Physician's Guide to Laboratory Test Selection & Interpretation
https://arupconsult.com/content/paraneoplastic-pemphigus
- 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