Facial Cutaneous Rosai-Dorfman Disease Mimicking Sporotrichosis: A Case Report and Discussion
Abstract:
This case report describes a 64-year-old woman with facial Cutaneous Rosai-Dorfman Disease (CRDD) initially misdiagnosed as sporotrichosis. The patient presented with a solitary erythematous nodule on the right cheek, which gradually enlarged and ulcerated. Histopathological examination revealed epidermal pseudoepitheliomatous hyperplasia and granulomatous inflammation in the dermis, with dense inflammatory infiltrates composed predominantly of lymphocytes, histiocytes, and neutrophils. The diagnosis of CRDD was established based on these findings, and the patient underwent complete surgical excision with no recurrence observed during follow-up. This case highlights the importance of careful differential diagnosis to avoid misdiagnosis and ensure appropriate treatment.
Keywords: Rosai-Dorfman disease, sporotrichosis, histiocytic diseases, sinus histiocytosis with massive lymphadenopathy, emperipolesis
Introduction:
Rosai-Dorfman disease (RDD) is a rare form of sinus histiocytosis with massive lymphadenopathy, characterized by the accumulation of histiocytes within lymph node sinuses and various extranodal tissues. Cutaneous Rosai-Dorfman disease (CRDD) is a rare cutaneous variant confined to the skin. This report describes an uncommon presentation of CRDD that initially misdiagnosed as sporotrichosis, a manifestation rarely reported in the literature.
Case Report:
A 64-year-old woman presented with a solitary erythematous nodule on the right cheek, which had developed four months earlier without an identifiable precipitating factor. The nodule gradually enlarged, forming an erythematous plaque that ulcerated and developed a thick, greasy, yellowish-white crust. Histopathological examination revealed epidermal pseudoepitheliomatous hyperplasia and granulomatous inflammation in the dermis, with dense inflammatory infiltrates composed predominantly of lymphocytes, histiocytes, and neutrophils. Based on the histopathological findings, the diagnosis of CRDD was established.
Discussion:
CRDD can develop at any age, with a mean onset of approximately 45 years, and exhibits a slight female predominance. Its clinical manifestations are remarkably diverse, presenting as papules, nodules, plaques, or other morphologic variants. The diagnosis relies primarily on histopathologic evaluation, which typically reveals a dense dermal infiltrate consisting of histiocytes, lymphocytes, plasma cells, and neutrophils. The hallmark histological feature is emperipolesis: the engulfment of intact inflammatory cells within histiocytes. However, emperipolesis is not universally observed and might only be detected through serial sectioning and meticulous microscopic examination, making diagnosis occasionally challenging.
Conclusion:
In clinical practice, when CRDD is suspected, thorough evaluation is essential to ensure diagnostic accuracy and appropriate management. A detailed medical history, assessment for potential comorbidities, and prompt histopathologic and immunohistochemical examinations are critical for establishing the correct diagnosis. Such vigilance helps prevent misdiagnosis or delayed recognition, thereby avoiding inappropriate treatment and potential disease progression.
