To learn the clinical features and management of Rowell's syndrome (RS).

The clinical information of patients with RS who were admitted to the First Affiliated Hospital of Zhengzhou University from December 2010 to December 2016 was retrospectively analyzed.

Seven cases, 6 women, 1 man; with the average age at onset of (40±11) years were reported. Only one patient had clear history of seafood intake. Skin lesions presented as generalized targeted lesions, coupling with some papules. Histopathological examination revealed hyperkeratinization, partial dyskeratocytes, focal areas of basal cell liquefaction, and perivascular infiltration of lymphocytes in the upper dermis. Six of them were treated with prednisone at a dose of 0.7-1.1 mg·kg^-1·d^-1, one was treated with methylprednisolone at a dose of 80 mg/d while in hospital and at a dose of 1.2 mg·kg^-1·d^-1 after discharge. During the process of glucocorticoid tapering, hydroxychloroquine was administered at a dose of 0.2-0.4 g/d in 4 patients, and 1 was treated with cyclophosphamide at 0.4 g per week. Glucocorticoid could be tapered once the symptoms were relieved, 4 patients without relapse while others relapsed.

RS is a rare clinical syndrome with relative special immunological presentations. No specific therapy strategy has developed for this disease. Glucocortico-steroids tapering should be slower than in those who with lupus erythematosus, and the prognosis is variable.

Rowell's syndrome; Lupus erythematosus, cutaneous; Erythema multiforme