Chronic Benign Familial Pemphigus

Abstract

Ten patients with benign familial chronic pemphigus (BFCP) (Hailey-Hailey disease) were evaluated; semiologic and localization differences were described, and special attention was given to solitary or atypical forms. In all the cases, the diagnosis was confirmed by histopathology; some histopathologic differences and the results achieved by direct immunofluorescence of skin are discussed. Most of the patients responded to the treatment with corticosteroids and antibiotics.

Hailey Hailey disease-a rare case report

“…15 Systemic corticosteroids have been recommended for widespread disease. [16][17][18] Treatment may up-regulate SPCA1 expression either indirectly through an effect on cytokine production or by a direct effect on keratinocytes. 19,20 The role of antiseptics, antibiotics and/or topical antifungal agents as steroid-sparing agents is unproven, but long-term antimicrobial treatment appears to be helpful.…”

“…19,20 The role of antiseptics, antibiotics and/or topical antifungal agents as steroid-sparing agents is unproven, but long-term antimicrobial treatment appears to be helpful. 14,16,21 Topical tetracyclines, aminoglycosides, fusidic acid or imidazoles may be added to a topical corticosteroid. Topical aminoglycosides may have the added benefit of reversing the effects of pathogenic nonsense mutations by inducing read through of stop mutations.…”

Hailey-Hailey disease responding to thalidomide

“…As HHD is often recalcitrant to treatment, further options including invasive methods such as grenz ray therapy, carbon dioxide laser abrasion, erbium: YAG laser ablation, dermabrasion, electron beam therapy, botulinum toxin and full-thickness excision of affected skin with repair by split-thickness grafting have been reported as useful in treatment of HHD. [145678910]…”

“…The most efficacious treatment was the combination of betamethasone dipropionate and gentamicin. [5] HHD is linked to mutations in the Calcium-transporting ATPase type 2C member 1 gene encoding the human secretory pathway Ca 2+ /Mn 2+ ATPase. Topical aminoglycosides can induce read through of premature stop mutations in the epidermis.…”

‘Atypical’ Cases of Hailey-Hailey Disease May Represent Typical Examples of a Type 2 Segmental Manifestation

“…In 1988, Galimberti et al [9] described 10 cases of HHD. On a photograph of case 2, one forearm of this 44-year-old man shows a linear area of 'serosanguineous crusts on an infl ammatory base', running in a longitudinal direction.…”