An unusual presentation of cutaneous Rosai Dorfman Disease i
Cutaneous RDD is now considered to be a distinct entity with isolated skin involvement without any lymphadenopathy or systemic symptoms. Authors report a case of cutaneous RDD in a child presenting with multiple subcutaneous nodules.

A 6-year-old girl presented to us with multiple asymptomatic subcutaneous nodules over bilateral lower eyelids, left cheek, upper abdomen, bilateral buttocks, and left anterior thigh. The nodules developed insidiously, were progressive in nature and were gradually increasing in size for the past eight months. On cutaneous examination, the nodules were nontender, varied in size from 1 to 3 cm in diameter, were discrete, well defined and immobile, firm in consistency, and not fixed to the overlying skin. The overlying skin appeared normal without any change in color or texture. No lymphadenopathy was found. History of fever or constitutional symptoms was absent.

Routine blood, urine, and biochemical investigations were normal, except for microcytic, hypochromic anemia, and a raised erythrocte sedimentation rate (ESR) (65 mm). Chest X-ray and abdominal ultrasonography were normal. Mantoux test was negative. Fine-needle aspiration cytology from the subcutaneous nodule in the gluteal area from the lesion in the buttock predominantly showed histiocytes with plenty of plasma cells and lymphocytes. Few of the histiocytes showed emperipolesis.

Histopathological examination was done after the excision of the subcutaneous nodule over the left anterior thigh which showed an unencapsulated mass consisting of numerous lymphoid follicles. Surrounding areas showed sheets of histiocytes having bland nuclei and abundant foamy cytoplasm along with fibrocollagenous tissue and vascular fragments. Occasional histiocytes showed the presence of intact lymphocytes and plasma cells in its cytoplasm suggesting emperipolesis. The presence of numerous foci of plasma cell aggregates was also noted. Periodic acid-Schiff stain, Gram stain, and Fite stain were all negative. Immunohistochemistry studies could not be done due to affordability issues. Thus, taking into consideration, the cutaneous, systemic, laboratory, and histopathological findings, a diagnosis of cutaneous RDD was made.

The patient was started on oral prednisolone (0.5 mg/kg) with a significant decrease in the size of the lesions after one month. Thereafter, the patient was lost to follow-up.