Rosai-Dorfman disease presenting with extensive cutaneous manifestation - Case report

Abstract

Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been reported in 43% of cases. In this study, we report an atypical case of Rosai-Dorfman disease in a female with massive cutaneous manifestation on the thigh, associated with a minimal lymphadenopathy limited to the regional inguinal lymph nodes.

Cutaneous Rosai‐Dorfman disease in a 3‐year‐old boy

“…3 Rarely, CRDD can present with pustules, acneiform lesions, panniculitis-or vasculitis-like lesions, macular erythema, annular lesions, breast tumors, or resemble vascular lesions. 3,[18][19][20] Ulcerative nodules, as seen in our patient, are unusual. We believe that this atypical presentation may be due to the fact that some lesions were located in previous chickenpox scars, which could have weakened the underlying tissue, allowing ulceration.…”

“…Solitary or multiple erythematous‐brown to yellow papules, nodules, or plaques usually affecting the face (eyelids and malar region), back, anterior chest, thighs, abdomen, and shoulders are most frequently described 3 . Rarely, CRDD can present with pustules, acneiform lesions, panniculitis‐ or vasculitis‐like lesions, macular erythema, annular lesions, breast tumors, or resemble vascular lesions 3,18–20 . Ulcerative nodules, as seen in our patient, are unusual.…”

“…Emperipolesis (presence of intact inflammatory cells within histiocytes) is a specific finding of the disease 3,19 . Classic immunohistochemistry is positive for S‐100, CD4, factor XIIIa, CD68, and negative for CD1a 3,15,20 …”

Isolated intracranial Rosai-Dorfman disease in an adult man: Report of a rare case

“…6 RDD has mainly been reported in childhood and young adults in the second and third decade of life. 7 Constitutional symptoms are generally absent in the patients with CNS involvement. Neurological symptoms vary depending on the size, location and number of lesions; seizure and headache are the most common complaint in RDD patients.…”

Rosai‐Dorfman disease successfully treated with thalidomide: A case report

“…The main causes for RDD remain uncertain, however, its association with viral (such as human herpes virus 6 and Epstein-Barr virus infections) and immune causes have been suggested (Gaul & Chang, 2019). In the classic pattern, affected patients manifest with bilateral cervical lymphadenopathy and frequently with intermittent fevers, night sweats, and weight loss (di Dio et al, 2016;Fang & Chen, 2015;Leal et al, 2013) which did not treat with thalidomide but later responded to methotrexate (Nadal, Kervarrec, Machet, Petrella, & Machet, 2015). Numerous cases of cutaneous RDD have been reported that their lesions were solitary without good response to treatment (Fang & Chen, 2015;Nadal et al, 2015;Sun, Shi, Su, Zhang, & Lu, 2018).…”