Cutaneous Rosai-Dorfman Disease: Comprehensive Review of Cases Reported in the Medical Literature since 1990 and Presentation of an Illustrative Case

Abstract

Purely cutaneous disease without the characteristic lymphadenopathy is rare but has been increasingly reported in the literature. Compared with patients with systemic Rosai-Dorfman disease, patients with primary cutaneous Rosai-Dorfman disease are older, women are more commonly affected, and whites are more likely than blacks to be afflicted.

Doença de Rosai-Dorfman cutânea: relato de caso

“…Investigators have also attempted to demonstrate this association in small series of cases in the cutaneous forms of the disease; however, results have been varied, with the majority testing negative. 5,13 The finding of polyclonal gammopathy strengthens the hypothesis of a reactive process against infectious agents. 2 Contrary to the systemic form, which affects principally children and young adults and which shows no preference for gender or ethnic group, the purely cutaneous form of the disease is slightly more common in older age-groups, in women and in nonblack ethnic groups.…”

“…2,4,13,15 Extranodal forms occur in 43% of cases, the skin being the most common site. Around 10% of patients have skin lesions and in 3% the disease is limited exclusively to the skin.…”

“…The most common sites affected are, in order of decreasing frequency, the trunk, head, neck, lower and upper extremities. 13 The lesions may occasionally resemble psoriasis or acne. 11 Histopathology shows a proliferation of histiocytic cells of large cytoplasm and vesicular nuclei, sometimes with evident nucleoli.…”

“…Therapeutic options include corticosteroids, thalidomide, ·-interferon, cryosurgery, radiotherapy, chemotherapy and surgical excision, among others. 7,12,13 As a consequence of the progressive growth, the distressing appearance of the lesion and the absence of any systemic involvement, the patient was submitted to surgical excision and is currently being followed-up to monitor any possible recurrence or progression to systemic disease. …”

“…2,6 Purely extranodal cutaneous forms have already been reported; however, they occur rarely, with only 85 cases having been described up to 2006 in reports or short series, thus justifying the present case report. [10][11][12][13][14][15] …”