A Study on the Role of Bacteria in the pathogenesis of Familial Benign chronic pemphigus

Chung, Tae Ahn; Lim, Myeong Kyoo; Kwon, Kyung Sool; Jang, Ho Sun

Abstract

BACKGROUND: Familial benign chronic pemphigus is a rare autosomal dominant disorder characterized by persistently recurrent bullous and vesicular dermatitis of the sides of the neck, axillae, and apposing surfaces. Hailey and Hailey, Montes, and other some investigators reported the presence of bacteria in the lesions of familial benign chronic pemphigus, and suggested that bacteria was associated in the its pathogenesis.
OBJECTIVE
The purpose of this study was to elucidate the association of bacteria in thepathogenesis of familial benign chronic pemphigus.
METHODS
We performed Brown & Brenn Gram stain in 14 biopsy tissues, bacterial culture and sensitivity test in 10 cases, and decided the effective treatment methods in each case.
RESULTS
1. Man to woman ratio was 3.67: 1 and about 60% of patients were in their third and forth decades. The family history was found in 6 out of 14 cases(42.9%). 2. The lesions were predomiriantly distributed on the groins(85.7%), axillae(71.4%), neck(64.3 %), trunk(50.5%), antecubital fossa(214%). 3. In Brown & Brenn gram stain, the bacterial colonies in the stratum corneum were found in 12(85.7% ) out of 14 biopsy tissues. The cells and its keratohyaline granule in granular layer had blue or nealy black color in 13(92.9%) out of 14 cases. 4. In bacterial culture and sensitivity test, Staphylococcus aureus was identified in 7 cases(70.0 %), Staphylococcus hemolyticus, 2 cases(20.0%) and Streptococcus species, 1 case(10.0%). 5. The number of cases which showed effective response to antibiotics therapy was 8 (57.1%) out of 14 cases.
CONCLUSION
These results suggest that bacteria or its toxin play a part of role in the production of lesions of familial benign chronic pemphigus and recurrent bacterial infection may cause recurring of this disease in the genetically predisposed persons.