(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

Abstract

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

 

 

Citation

 

Keywords

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

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