Cutaneous Rosai-Dorfman Disease – A Clinical Case

Abstract

Introduction. Rosai-Dorfman disease is a benign condition, characterized by self limiting proliferation of non-Langerhans histiocytes inside lymph nodes but also in extranodal areas. Clinical case. A 54-year-old patient, diagnosed with arterial hypertension since 2011, is hospitalised in the Dermatology Department of the Emergency County Hospital, Craiova, Romania, in March 2016, for a polymorphous rash composed of violet-erythematous papulonodules with sizes ranging between 0.2 cm and 2 cm, slightly itchy, located in the latero-thoracic region, lumbar region and in the area around the menton area. The lesions appeared 18 months prior to hospitalization. Clinical and paraclinical investigations as well as interdisciplinary consultations revealed no associated pathologies. Histopathological and immunohistochemical examinations confirmed the diagnostic of cutaneous Rosai-Dorfman disease, imaging investigations excluding other localizations. Treatment with Doxycycline, in combination with 20 mg of Rutoside and 50 mg of ascorbic acid, Pentoxifylline and Levocetirizine resulted in remission of the skin lesions. Discussion. Rosai-Dorfman disease is a rare condition, having a reported incidence rate of 1:200.000. It generally affects males, being more frequent in young males and people of African descent. The pathogenesis of Rosai-Dorfman disease is still not fully understood, although immunological and infectious factors may play an important role. Foucar has divided this condition into three types: nodal, extranodal and mixed. Cutaneous lesions have been described in 3% of cases of Rosai-Dorfman disease of the extranodal type. It is more frequent in females (sex ratio of 2/1) and in adults of Asian descent, with a mean age at onset of 45 years. Treatment options include: oral and intralesional corticosteroids, alfa-interferon, retinoids, dapsone, methotrexate, thalidomide, cryo-and radiotherapy. Conclusions. The presented case is an atypical one because of the three locations of the skin lesions. Although we obtained remission of the disease, the patient would remain in direct observation because of the risk of recurrence.