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Isolated Familial Benign Chronic Pemphigus

Isolated Familial Benign Chronic Pemphigus Abstract To the Editor.— Familial benign chronic pemphigus (FBCP), or HaileyHailey disease, is a rare hereditary disorder that is characterized by recurrent vesicles and bullae in the intertriginous areas, such as the axillae and groin. The eruption is rarely confined to one area of the body. Report of a Case.— A 29-year-old woman was examined in November 1975 for pruritus vulvae of seven months' duration. Numerous small, flat, white papules and thickened lichenified skin were found on the vulvar surface. A skin biopsy specimen of a papule showed suprabasilar clefts, villi, and acantholytic epidermal cells characteristic of FBCP. The results of the rest of the skin examination were noncontributory. Fungal and yeast cultures grew no organisms, and pelvic and breast examination results were normal. There was no family history of recurrent bullous disease. The FBCP has not responded to steroid creams, antimonilial creams and suppositories, antifungal creams, steroid antibiotic combination cream, References 1. Cooper DL: Familial benign chronic pemphigus of perianal skin . Arch Dermatol 103:219-220, 1971.Crossref 2. Lyles TW, Knox JM, Richardson JB: Atypical features in familial benign chronic pemphigus . Arch Dermatol 78:446-453, 1958.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Isolated Familial Benign Chronic Pemphigus

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Isolated Familial Benign Chronic Pemphigus

Abstract

Abstract To the Editor.— Familial benign chronic pemphigus (FBCP), or HaileyHailey disease, is a rare hereditary disorder that is characterized by recurrent vesicles and bullae in the intertriginous areas, such as the axillae and groin. The eruption is rarely confined to one area of the body. Report of a Case.— A 29-year-old woman was examined in November 1975 for pruritus vulvae of seven months' duration. Numerous small, flat, white papules and thickened lichenified skin...
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References (3)

Publisher
American Medical Association
Copyright
Copyright © 1977 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1977.01640090150051
Publisher site
See Article on Publisher Site

Abstract

Abstract To the Editor.— Familial benign chronic pemphigus (FBCP), or HaileyHailey disease, is a rare hereditary disorder that is characterized by recurrent vesicles and bullae in the intertriginous areas, such as the axillae and groin. The eruption is rarely confined to one area of the body. Report of a Case.— A 29-year-old woman was examined in November 1975 for pruritus vulvae of seven months' duration. Numerous small, flat, white papules and thickened lichenified skin were found on the vulvar surface. A skin biopsy specimen of a papule showed suprabasilar clefts, villi, and acantholytic epidermal cells characteristic of FBCP. The results of the rest of the skin examination were noncontributory. Fungal and yeast cultures grew no organisms, and pelvic and breast examination results were normal. There was no family history of recurrent bullous disease. The FBCP has not responded to steroid creams, antimonilial creams and suppositories, antifungal creams, steroid antibiotic combination cream, References 1. Cooper DL: Familial benign chronic pemphigus of perianal skin . Arch Dermatol 103:219-220, 1971.Crossref 2. Lyles TW, Knox JM, Richardson JB: Atypical features in familial benign chronic pemphigus . Arch Dermatol 78:446-453, 1958.Crossref

Journal

Archives of Dermatology – American Medical Association

Published: Sep 1, 1977

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