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February 1997

Is Cyproheptadine Effective in the Treatment of Subjects With Epidermolysis Bullosa Simplex-Dowling-Meara?

Author Affiliations

Seattle, Wash

Division of Dermatology CH-25 Children's Hospital and Medical Center PO Box 5371 Seattle, WA 98105

Arch Dermatol. 1997;133(2):251-252. doi:10.1001/archderm.1997.03890380125030

Epidermolysis bullosa simplex—Dowling-Meara (EBS-DM) is a severe form of EBS, with oral and nail involvement as well as severe skin blistering. Reports1,2 of treatment with 5-hydroxytryptamine—2 antagonists reducing the severity of blisters in subjects with EBS-DM prompted us to study cyproheptadine hydrochloride therapy in 13 individuals with a diagnosis of EBS-DM confirmed by electron microscopic examination.

Participants served as their own controls. There was a 2-week observational period, an initial 6-week period during which cyproheptadine was taken daily, a 4-week period with no treatment, and a second 6-week regimen of daily administration of cyproheptadine. Standard dosages of cyproheptadine were used.3 The study was designed in this manner in an attempt to compensate for temporal variation in blister formation as well as variations in season, weather, and activity levels of the participants. Subjects were asked to record the number of blisters lanced each day rather than the