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pemphigus foliaceus
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:
1) acantholysis in granular layer of epidermis
- high granular or subcorneal clefting [3]
2) no disruption of the epidermal basement membrane
3) IgG autoantibody to desmoglein-1
* histopathology image [4]
Clinical manifestations:
1) lesions generally on face, scalp, upper chest & abdomen but may involve entire skin surface
2) bullae rarely present
3) erythematous patches (erythroderma) & erosions covered with crusts
4) rarely involves mucous membranes
5) Nikolsky sign is positive [3]
* images [4,5]
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:
1) high-dose glucocorticoids
2) dapsone
Interactions
disease interactions
Related
acantholysis
desmoglein-1; cadherin family member 4; desmosomal glycoprotein 1; DG1; DGI; Pemphigus foliaceus antigen (DSG1, CDHF4)
pemphigus vulgaris
Specific
pemphigus erythematosus (Senear-Usher syndrome)
General
pemphigus
Properties
ANTIGEN: desmoglein-1
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
- Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18.
American College of Physicians, Philadelphia 2009, 2012, 2015, 2018
- Schwartz RA, Elston DM (images)
Medscape: Pemphigus Foliaceus
http://emedicine.medscape.com/article/1064019-overview
- DermNet NZ. Pemphigus foliaceus (images)
http://www.dermnetnz.org/immune/pemphigus-foliaceus.html