Rowell's syndrome: 7 cases and literature review / 中华风湿病学杂志

Objective To leam the clinical features and management of Rowell's syndrome (RS).Methods 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.Results 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.Conclusion RS is a rare clinical syndrome with relative special immunological presentations.No specific therapy strategy has developed for this disease.Glucocorticosteroids tapering should be slower than in those who with lupus erythematosus,and the prognosis is variable.

Rowell's syndrome: a clinical observation and a follow-up analysis / 中华皮肤科杂志

Objective To investigate the characteristics,treatment and prognosis of Rowell's syndrome.Methods Clinical data were collected from 6 inpatients with Rowell's syndrome from July 2009 to July 2015,and retrospectively analyzed.Results 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 hauds 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-U 1RNP 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.Conclusions 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.

Necrolisis epidérmica tóxica y lupus eritematoso sistémico / Toxic epidermal necrolysis and systemic lupus erythematosus

La necrolisis epidérmica tóxica es el resultado de una reacción mucocutánea aguda severa. La frecuencia con que ocurre esta enfermedad es superior en estados patológicos, cuando existe una respuesta inmune aberrante, incluida enfermedad injerto contra huésped, cáncer, infección por VIH y enfermedades autoinmunes. Se describe un caso que cumple con los criterios diagnósticos de lupus eritematoso sistémico y necrolisis epidérmica tóxica

Toxic epidermal necrolysis is the result of severe acute mucocutaneous reaction. The frequency of this disease is higher in the pathological states, when there is aberrant immune response including disease, graft against host, cancer, HIV infection and autoimmune diseases. A case that met the diagnostic systemic lupus erythematosus and toxic epidermal necrolysis criteria was described

A Case of Rowell's Syndrome / 대한피부과학회지

Rowell's syndrome is a rare entity consisting of lupus erythematosus with associated erythema multiforme-like lesions and characteristic immunological findings, and it was first described by Rowell et al. in 1963. Because of the clinical similarities between subacute cutaneous lupus erythematosus and Rowell's syndrome, it is difficult to clinically distinguish between the annular-polycyclic type of subacute cutaneous lupus erythematosus and Rowell's syndrome. We report a case of 18-year-old woman with systemic lupus erythematosus who presented erythema multiforme-like eruptions on both thighs. Simultaneously, she developed urticarial-like rashes on her trunk consistent with atypical skin lesion of lupus erythematosus. She also developed a positive antinuclear antibody with speckled pattern and positive anti-Ro antibody.

A Case of Rowell Syndrome / 대한피부과학회지

In 1963, Rowell et al. reported a new syndrome characterized by lupus erythematosus with erythema multiforme-like lesions and specific immunological abnormalities, termed Rowell syndrome. Herein, we report a case meeting the criteria for Rowell syndrome. A 30-year-old woman presented with annular, targetoid, erythematous, scaly papules and plaques on her face and the whole body. She also showed abnormal immunoserologic findings. A biopsy specimen confirmed lupus erythematosus and erythema multiforme; therefore, she was diagnosed with Rowell syndrome.

A Case of Rowell's Syndrome / 대한피부과학회지

Rowell's syndrome is a rare and distinct clinical entity that has been described as lupus erythematosus associated with erythema multiforme-like lesions with the immunological findings of speckled antinuclear antibodies and positive tests for anti-Ro/La antibodies and rheumatoid factor. We report here on a case of discoidlupus erythematosus in a 39-year-old woman with targetoid eruptions on both palms, the lower legs and soles and she also showed abnormal immunoserologic findings. Most of these features were consistent with Rowell's syndrome.

A Case of Rowell Syndrome with Kikuchi Disease / 대한피부과학회지

A 24-year-old woman presented with an erythema multiforme-like eruption on face, scalp and both extremities, intermittent fever, and cervical lymphadenopathy. The clinical and unusual laboratory findings were consistent with a diagnosis of Rowell's syndrome. The histopathology of cervical lymph node showed histiocytic necrotizing lymphadenitis, Kikuchi disease.

A Case of Rowell's Syndrome / 대한피부과학회지

Rowell's syndrome is described as erythema multiforme(EM)-like lesions in the presence of lupus erythematosus(LE) associated with immunoserological characteristics of speckled antinuclear antibodies and positive tests for anti-Ro/La antibodies and rheumatoid factor. We report a case of Rowell's syndrome in a 28-year-old woman with systemic lupus erythematosus for 13 years, who presented with targetoid eruptions on the right arm and with positive immunoserologic findings as above.

A Case of Rowell's syndrome / 대한피부과학회지

Rowell's syndrome is believed to be a distinct and rare clinical entity originally descrived as lupus erythematosus associated with erythema multiforme-like skin lesions, immunologic findings of speckled antinuclear antibodies, anti-La antibodies and a positive test for rheumatoid factor. We report a case of systemic lupus erythematosus in a 31-year-old woman with annular erythematous patches on both hands, feet and back. Also, she presented abnormal serologic and immunologic findings. We believed that our patient meets the criteria for this rarely reported entity.