Enfermedad de Rosai-Dorfman cutánea, reporte de un caso / Cutaneous Rosai-Dorfman disease, a case report

RESUMEN La enfermedad de Rosai-Dorfman cutánea (ERDC) es una rara proliferación reactiva de histiocitos, que cursa con lesiones cutáneas sin compromiso generalizado. Las manifestaciones clínicas cutáneas son diversas y la variante clínica "tipo tumoración" es la forma más infrecuente. Se describe el caso de un paciente con ERDC que se presentó con esta forma inusual.

ABSTRACT Cutaneous Rosai-Dorfman disease (CRDD) is a rare reactive histiocytic proliferation, which presents with skin lesions without systemic involvement. The clinical cutaneous manifestations are diverse and the "tumor-like" clinical variant is the most infrequent. We describe the case of a patient with CRDD who presented with this unusual form.

Cutaneous Rosai-Dorfman disease in a 40-year-old female: A case report

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.

Cutaneous Rasai-Dorfman disease / 中华皮肤科杂志

Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder.The etiology and pathogenesis of RDD are still unclear,and may be associated with the activation of signal transduction pathways and inflammatory reactions induced by infection and immune dysfunction.CRDD is defined as the RDD involving only the skin.Histopathological examination of CRDD lesions shows that a large amount of histiocytes,lymphocytes and plasma cells infiltrate the dermis,and intact endocytosed lymphocytes can be observed in the cytoplasm of histiocytes,which is called emperipolesis.Immunohistochemical study shows that histiocytes are stained positive for S-100 and CD68,but negative for CD1a.The treatment of CRDD includes surgical excision,local therapy and systemic therapy.Lesions can subside spontaneously in some patients,and it usually has a favorable prognosis.

Enfermedad de Rosai-Dorfman cutánea: a propósito de un caso / Cutaneous Rosai-Dorfman disease: a propos of a case

Se presenta una paciente de 50 años de edad con lesión tumoral redondeada, no dolorosa en región inguinal, con crecimiento progresivo y formación de una placa eritematosa, con áreas de pigmentación e infiltración de tejidos profundos, así como aparición de otras lesiones de aspecto tumoral. El estudio anatomopatológico evidenció un infiltrado histiocitario con fenómenos de linfohistiofagocitosis (emperipolesis) y el inmunohistoquímico mostró positividad para vimentina, S-100, CD68 y lisozima. Se revisó la evidencia científica disponible en la literatura mundial para determinar las manifestaciones clínicas más frecuentes, así como los medios diagnósticos más empleados en la actualidad y el tratamiento de esta enfermedad. Es importante considerar a la enfermedad de Rosai-Dorfman cutánea como un posible diagnóstico para lo que es indispensable un profundo estudio de la biopsia, así como la realización de estudios de inmunohistoquímica, dada la benignidad y buen pronóstico que la caracterizan

A 50 year-old patient with a painless round tumoral lesion on inguinal region with progressive growth and formation of an eritematous plaque with areas of pigmentation, infiltration of deep tissues and apparition of other lesions with tumoral aspect is described. The anatomopathological study showed a hystiocitic infiltrate with linfohystiofagocitosis phenomenon (Emperipolesis) and the immunohystochemical made evident positive for Vimentine, S-100, CD68 and Lisozime. The available scientific evidence of the international literature was reviewed to determine the most frequent clinical manifestations of this disease, as well as most updated means of diagnosis employed and the treatment of this disease. It is important to consider the cutaneous Rosai-Dorfman disease as one of the possible diagnosis so a profound study of the biopsy is essential, as well as the immunohystochemical studies due to the mildness and good prognosis of this disease

Doença de Rosai-Dorfman cutânea: relato de caso e revisão de literatura / Cutaneous Rosai-Dorfman disease: case report and review of literature

Relata-se o caso de uma paciente com doença de Rosai-Dorfman cutânea que apresentava na região glútea direita uma placa eritemato-acastanhada infiltrada com áreas amareladas e pápulas eritemato-acastanhadas perilesionais. O exame anatomopatológico revelou infiltrado inflamatório predominantemente de linfócitos e histiócitos de citoplasma amplo, claro e contendo células inflamatórias íntegras (emperipolese). Nenhuma outra alteração ao exame físico e laboratorial foi encontrada. A doença de Rosai-Dorfman é uma forma rara de proliferação histiocitária e o acometimento estritamente cutâneo é ainda mais raro. Sua etiologia é desconhecida e a correlação clinicopatológica permitiu o diagnóstico dessa enfermidade.

We report a patient with cutaneous Rosai-Dorfman disease that presented on the right buttock, a red brown plaque with yellowish areas and red-brown satellite papulas. The histopathologic exam revealed inflammatory infiltrate predominantly of lymphocytes and histiocytes with wide and clear cytoplasm containing intact inflammatory cells (emperipolesis). Any other alteration in the physical exam and laboratorial was not found. The Rosai-Dorfman disease is a rare form of histiocytic proliferation and the strictly cutaneous form is still rarer. The aetiology is unknown and the clinical and histologic correlation enables the correct diagnosis.