Five cases of cutaneous Rosai-Dorfman disease

Abstract

Rosai-Dorfman disease (RDD), previously known as sinus histiocytosis with massive lymphadenopathy, is a benign, idiopathic histiocytic proliferative disorder. It commonly affects lymph nodes, but any organ of the body may be involved. Histological findings include characteristic large, pale, histiocytic cells (Rosai-Dorfman cells) exhibiting cytophagocytosis. Immunohistochemically, these histiocytes are positive for S-100 protein and CD68, but stain negatively for CD1a. On electron microscopy, Birbeck granules are absent. RDD limited to the skin is rare, less than 30 cases having been reported to date. We present five further cases of purely cutaneous RDD. Three presented as solitary nodules and one as a large, well-circumscribed plaque. The fifth patient, who was HIV positive, had a rosacea-like facial eruption.

Doença de Rosai-Dorfman cutânea: relato de caso

“…4,7,9,10 Various affected sites have been described such as the nasal and paranasal cavities, eyes and retro-orbital tissue, central nervous system, bones and skin, among others. 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] …”

“…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.…”

“…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. 1,3,4,6,11,12 Clinically, it presents with papules, nodules, plaques, masses or tumors of a brownish-or yellowish-erythematous color, varying in size from less than 1 cm to 30 cm, either localized or disseminated.…”

“…[1][2][3][4][5][6][7][8] It is characterized clinically by a massive, bilateral, painless cervical lymphadenopathy, usually in association with fever, weight loss, night sweats, leukocytosis with neutrophilia, increased erythrocyte sedimentation rate and polyclonal gammopathy. 1,2,[8][9][10] Although less common, other lymph node chains such as the axillary, inguinal, paraaortic or mediastinal chains may be affected. 8,11 Extranodal involvement occurs in 43% of cases, either in association with lymph node involvement or otherwise.…”

Rosai Dorfman disease- A rare entity: Case report in North Indian male

“…1 Patients with this disease presents with massive lymphadenopathy with systemic symptoms like fever, neutrophilia, leucocytosis, raised ESR and polyclonal gammopathy. 2,3 The Etiology of this disease entity is still unclear. Less than 1000 cases have been reported in literature worldwide with young adult males being most affected.…”