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Chronic benign familial pemphigus.
R L Galimberti1, A M Kowalczuk, O Bianchi
1Department of Dermatology, Italian Hospital, Buenos Aires, Argentina.
International Journal of Dermatology
|September 1, 1988
Summary
Benign familial chronic pemphigus (Hailey-Hailey disease) presents with varied symptoms and locations. Most patients with this chronic skin condition responded well to corticosteroids and antibiotics.
Area of Science:
- Dermatology
- Histopathology
- Immunofluorescence
Background:
- Benign familial chronic pemphigus (BFCP), also known as Hailey-Hailey disease, is a rare genetic blistering disorder.
- The condition is characterized by recurrent blistering, erosions, and crusting, primarily affecting intertriginous areas.
Purpose of the Study:
- To describe the semiologic and localization characteristics of benign familial chronic pemphigus.
- To highlight solitary or atypical presentations of the disease.
- To discuss histopathologic findings and direct immunofluorescence results.
Main Methods:
- Evaluation of ten patients diagnosed with benign familial chronic pemphigus.
- Histopathological confirmation of diagnosis in all cases.
- Analysis of direct immunofluorescence findings in skin biopsies.
Main Results:
- Varied semiologic and localization patterns were observed among the ten patients.
- Histopathological differences were noted and discussed.
- Direct immunofluorescence provided diagnostic insights.
- The majority of patients showed a positive response to treatment with corticosteroids and antibiotics.
Conclusions:
- Benign familial chronic pemphigus exhibits diverse clinical presentations.
- Histopathology and direct immunofluorescence are crucial for diagnosis.
- Corticosteroids and antibiotics are effective therapeutic options for most patients.