Korean J Dermatol.  1964 Jun;3(1):47-52.

A Case of Familial Benign Chronic Pemphigus (Hailey and Hailey)

• Suh CK

Abstract

A case of familial benign chronic pemphigus in 28 year old male is described which is the first case reported in Korea, and the literature is reviewed The grandmothcr had similar symptoms and the 52 years old father has suffered from the same trouble since puberty The patient's lesions were vesieular, pustular, and crusted patches with erosions which have been persistent since 12 years of age, mainly affecting lateral and posterior sides of the neck, axillary and inguinal regions, lower abdomen, and popliteal fossae symmetrically. Moderate itching was associated with the lesions, which were worse in summer and better in winter. Staphylococcus aureus and p-hemolytic streptococcus were cultured from the lesions and found to be sensitive to aureomycin, terramycin and erythromycin. Biopsy findings showed hyperkeratosis, irregular acanthosis, multilocular suprabasal clefts or bullae with extensive acantholysis. villi formation was also noted. Dyskeratotic changes were present but no mitosis was seen. Local application of 1% hydrocortisone ointment gave temporary improvement but no effect was noted with systemic prednisolone, aureomycin, sulfadrugs, and vitamin A & D, or local treatment with antibiotic ointments.

MeSH Terms

Abdomen
Acantholysis
Adolescent
Adult
Biopsy
Chlortetracycline
Erythromycin
Fathers
Humans
Hydrocortisone
Korea
Male
Middle Aged
Mitosis
Neck
Ointments
Oxytetracycline
Pemphigus, Benign Familial*
Prednisolone
Pruritus
Puberty
Staphylococcus aureus
Streptococcus
Vitamin A
Chlortetracycline
Erythromycin
Hydrocortisone
Ointments
Oxytetracycline
Prednisolone
Vitamin A