To investigate the characteristics, treatment and prognosis of Rowell′s syndrome.

Clinical data were collected from 6 inpatients with Rowell′s syndrome from July 2009 to July 2015, and retrospectively analyzed.

All the patients were female, of them, 2 developed skin lesions after sun exposure, 2 had medication history, 1 had Staphylococcus aureus infection before occurrence of skin lesions, and 1 had no obvious precipitating factor. Skin lesions spread from the face to the whole body in 4 cases, from the dorsal hands to the whole body in 1 case, and were confined to the face, both hands and feet in 1 case. All the 6 patients had chilblain-like lesions in the digit tips, and 5 patients had fever. Among these patients, 6 were positive for antinuclear antibodies (granular pattern) , 4 for anti-Sm antibodies, 5 for anti-U1RNP antibodies, 4 for anti-SSA antibodies, 2 for anti-dsDNA antibodies, and 1 was positive for both anti-SSA and anti-SSB antibodies. Histopathological examination revealed hyperkeratinization, mild hyperplasia, partial necrosis, many dyskeratocytes, liquifaction degeneration of basal cells in the epidermis, and perivascular infiltration of lymphocytes in the superficial dermis. After admission, 1 patient was finally diagnosed with subacute cutaneous lupus erythematosus (SCLE) , and 5 with systemic lupus erythematosus (SLE) . All the 6 patients were treated with prednisone at a dose of 0.6-1.0 mg·kg^-1·d^-1, and 1 was also treated with gamma globulin (200 mg/d) . During the process of glucocorticoid tapering, hydroxychloroquine was administered at 0.2 g twice a day. During 1-year follow-up, neither erythema multiform nor chilblain-like lesions recurred in 5 patients, and 1 patient was lost to follow-up.

Rowell′s syndrome frequently occurs in women. Glucocorticoids are a mainstay of its treatment, and their tapering should be slower in patients with Rowell′s syndrome than in those with lupus erythematosus.

Lupus erythematosus, systemic; Erythema multiforme; Glucocorticoids; gamma-Globulins; Rowell′s syndrome