Abstract

Rosai-Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy, is a rare disease. Cutaneous RDD (CRDD) is an extremely rare form of RDD, which is limited to the skin. The present study examined a case of purely CRDD in a 25-year-old female patient who presented with a two-month history of red plaques on her face. In addition, a review of the literature was conducted, where the etiology, pathology, clinical characteristics and treatment of the disease were discussed. From a dermatological perspective, the current study aimed to emphasize the histological features and clinical morphology of cutaneous RDD. Clinicians should have sufficient knowledge to be able to recognize and manage this rare condition. The present study found that the presence of reddish-yellow nodules on the face without any particular sensitivity may be useful in the diagnosis of CRDD. Treatment with topical steroids was found to be beneficial in alleviating CRDD.

Highlights

  • Rosai‐Dorfman disease (RDD), originally known as sinus histiocytosis with massive lymphadenopathy, is a non‐Langerhans cell histiocytosis that was first described in 1965 by Destombes [1] and subsequently recognized as a distinct entity by Rosai and Dorfman in 1969 [2]

  • The term cutaneous RDD (CRDD) is used to describe the forms of RDD that only involve the skin

  • CRDD differs from RDD in that RDD

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Summary

Introduction

Rosai‐Dorfman disease (RDD), originally known as sinus histiocytosis with massive lymphadenopathy, is a non‐Langerhans cell histiocytosis that was first described in 1965 by Destombes [1] and subsequently recognized as a distinct entity by Rosai and Dorfman in 1969 [2]. CRDD presents as solitary or numerous papules, nodules, plaques or as a combination of these. A dermatological examination showed indurated erythematous plaques with a number of reddish‐yellow nodules on the left side of the patient's face (Fig. 1). The biopsy specimen obtained from the afflicted area showed that the epidermis was normal, but revealed dense inflammatory infiltrates, composed of neutrophils, plasma cells, lymphocytes and histiocytes, in the dermis. Laboratory examination revealed no abnormality in routine tests, including the blood count, urinalysis, erythrocyte sedimentation rate, C‐reactive protein levels, liver and kidney function tests and muscle enzymes. The plaque treated with local injections of the steroid showed improvement. The patient declined to continue with the injection therapy due to side‐effects, including increased blood pressure and weight gain, from the systematic use of corticosteroids. The patient showed slight improvement in FANG and CHEN: CUTANEOUS ROSAI-DORFMAN DISEASE. Written informed consent was obtained from the patient prior to participation in the present study

Discussion
Rosai J and Dorfman RF
Chan CC and Chu CY
13. Mannan AA and Karak AK
17. Weitzman S and Jaffe R
Findings
19. Silvestre JF and Aliaga A
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