(Last Updated On: May 18, 2021)

Authors: Joland A. San Juan, MD, Juan Antonio D. Cervantes, MD, Johannes F. Dayrit, MD, FPDS, FDSP, Ricky H. Hipolito, MD, FPDS, Ma. Teresita G. Gabriel, MD, FPDS


INTRODUCTION : Rosai-Dorfman disease is a rare disease that manifests with painless cervical lymphadenopathy, fever, anemia,
an elevated erythrocyte sedimentation rate (ESR), and hypergammaglobulinemia. Extranodal lesions occur in 1/3 of patients, and
the skin is involved in more than 10% of cases. Purely cutaneous disease is uncommon and only about more than 100 cases have
been reported. Cutaneous Rosai-Dorfman Disease (CRDD) appears to be a distinct entity with different age and race predilection
from cases with lymph node involvement.

CASE REPORT : This is a case of a 40-year-old Filipino female who presented with multiple erythematous papules and plaques with
pustules on the cheeks. Skin punch biopsy showed a dense dermal infiltrate of polygonal histiocytes with abundant cytoplasm
and vesicular nuclei. Emperipolesis was also present. The histiocytes were highlighted by the immunohistochemical stains S-100
and CD68 and was CD1a negative. Complete blood count and ESR were normal. Cervical lymphadenopathy was absent. Findings
were consistent with Cutaneous Rosai-Dorfman disease. The patient was started on methotrexate at 15mg/week with folic acid
supplementation. Mild soap, benzoyl peroxide 5% gel and tretinoin 0.05% cream once daily were maintained during the treatment
course. There was significant decrease in erythema and size of existing lesions after 2 months. The patient was referred to a
hematologist for monitoring of possible future systemic involvement.

CONCLUSION : Because of its rarity, clinicopathological correlation is always mandatory to establish a diagnosis of CRDD. Immunohistochemical stains are required to differentiate this entity form other forms of Langerhans cell histiocytosis. Multidisciplinary referral is required to rule out concomitant systemic involvement






Cutaneous Rosai-Dorfman Disease, Non-langerhans cell histiocytosis, lymphadenopathy

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