pemphigus foliaceus
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Introduction
Superficial form of pemphigus with bullae rarely present.
Epidemiology
- 0.5-6.6 cases/million/year
- endemic pemphigus foliaceus occurs in Brazil & Columbia
- up to 50 cases/million/year, up to 3.4% of population[3]
Pathology
- acantholysis in granular layer of epidermis
- no disruption of the epidermal basement membrane
- IgG autoantibody to desmoglein-1
* histopathology image[4]
Clinical manifestations
- lesions generally on face, scalp, upper chest & abdomen but may involve entire skin surface
- bullae rarely present
- erythematous patches (erythroderma) & erosions covered with crusts
- rarely involves mucous membranes
- Nikolsky sign is positive[3]
Laboratory
- skin biopsy for diagnosis
- direct immunofluorescence shows deposits of IgG between epidermal cells
- desmoglein-1 Ab
Differential diagnosis
- pemphigus vulgaris with suprabasal clefting
Management
- high-dose glucocorticoids
- dapsone
More general terms
More specific terms
Additional terms
- acantholysis
- desmoglein-1; cadherin family member 4; desmosomal glycoprotein 1; DG1; DGI; Pemphigus foliaceus antigen (DSG1, CDHF4)
- pemphigus vulgaris
References
- ↑ Color Atlas and Synopsis of Clinical Dermatology, Common and Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 402
- ↑ Internal Medicine News, June 2005, pg 22
- ↑ 3.0 3.1 3.2 3.3 Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018
- ↑ 4.0 4.1 4.2 Schwartz RA, Elston DM (images) Medscape: Pemphigus Foliaceus http://emedicine.medscape.com/article/1064019-overview
- ↑ 5.0 5.1 DermNet NZ. Pemphigus foliaceus (images) http://www.dermnetnz.org/immune/pemphigus-foliaceus.html