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Clinical features

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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Painful mouth and genital ulcers develop early and may be followed by the development of fragile skin blisters. The lesions rarely itch but are quickly denuded by friction or pressure leaving a raw surface. Oral lesions are frequently persistent and may cause difficulty in eating. Nikolsky's sign is positive.

Secondary infection and disturbance of fluid and electrolyte balance are common in the untreated patient, and were largely responsible for the high mortality associated with the disease before the introduction of corticosteroid therapy.

Painful mouth and genital ulcers develop early and may be followed by the development of fragile skin blisters. Lesions usually begin in the mucous membranes and extend to involve the skin after several weeks or months (1). The lesions rarely itch but are quickly denuded by friction or pressure leaving a raw surface. Due to the thin roof which covers the vesicle, more erosions are present compared to intact blisters. These erosions may take some time to heal (weeks or months) (2).

Oral lesions are frequently persistent and may cause difficulty in eating.

Nikolsky's sign is positive (when lateral pressure is put on the skin with the thumb, the epidermis appears to slide over the underlying dermis.) (1).

Secondary infection and disturbance of fluid and electrolyte balance are common in the untreated patient, and were largely responsible for the high mortality associated with the disease before the introduction of corticosteroid therapy.

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