Cutaneous Rosai-Dorfman disease in a 40-year-old female: A case report
- Author:
Joland A. San Juan
1
;
Juan Antonio D. Cervantes
1
;
Johannes F. Dayrit
1
;
Ricky H. Hipolito
1
;
Ma. Teresita G. Gabriel
1
Author Information
- Publication Type:Case Reports
- Keywords: Cutaneous Rosai-Dorfman Disease; Non-langerhans cell histiocytosis
- MeSH: Lymphadenopathy
- From: Journal of the Philippine Dermatological Society 2021;30(1):53-56
- CountryPhilippines
- Language:English
-
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. Immu- nohistochemical stains are required to differentiate this entity form other forms of Langerhans cell histiocytosis. Multidisci- plinary referral is required to rule out concomitant systemic involvement. - Full text:jpds11.pdf
