Rosai–Dorfman disease (RDD) is a rare idiopathic histiocytic proliferative disorder.1 The skin is the most frequently involved extranodal organ. Nevertheless, primary cutaneous manifestation of RDD (CRDD) without systemic involvement is rare.2 We present herein a case of CRDD showing excellent remission after low-dose oral thalidomide therapy.
A 43-year-old man (height, 178 cm; weight, 80 kg) was referred to our department for evaluation of a 12-month history of painless, enlarging papules and crusts involving the facial and pectoral areas and upper extremities. The papules were moderately tender and occasionally pruritic (Figure 1A). Findings of physical examination, comprehensive laboratory tests, and whole-body radiography were all within normal limits. Two skin biopsy specimens were taken, one from the right mandibular region and the other from the right upper arm. Histopathologic examination revealed that intact lymphocytes, plasma cells, and even neutrophils were readily found within the cytoplasm of histiocytes (emperipolesis). Immunohistochemically, the histiocytes were positive for S-100, CD68, and CD20 and negative for CD1a, CK, and CD21. Periodic acid–Schiff and Giemsa staining results were negative. Based on the clinical and histopathologic findings, a diagnosis of CRDD was made.
A, Erythematous plaques with reddish-yellow nodules on his face. B, After 8 months of thalidomide treatment, the nodules are nearly resolved, and most of erythematous plaques are lightened or even cleared.
The patient had been treated with 36 mg/d of methylprednisone for 3 months and received 10 fractionated local radiation doses of 2 Gray with a total dose of 20 Gray to the face. One lesion in the neck was intralesionally injected once with combination betamethasone dipropionate/betamethasone disodium phosphate (Diprospan; Merck Sharp & Dohme [Malaysia] Sdn Bhd).3,4 All treatments failed to demonstrate clinical efficacy. After providing his informed consent, the patient was given oral thalidomide, 50 mg/d for 2 weeks with slight improvement and no significant adverse effects. Therefore, the dose of thalidomide was increased to 100 mg/d. After 8 months of treatment, the nodules on his face and limbs had nearly resolved, and most of the erythematous plaques had lightened and partially cleared (Figure 1B). At 2-year follow-up, the lesions had not recurred.
Various treatment options, including administration of high-dose thalidomide, steroids, chemotherapy, or surgical excision, have been reported with variable success.5- 8 Prior to thalidomide treatment, our patient received systemic steroid treatment, local radiotherapy, and an intralesional injection of steroid in a nodule without clinical improvement. Instead, the lesions progressively enlarged. Our treatment with oral low-dose thalidomide for this benign proliferative disorder resulted in excellent clinical outcome. The most challenging adverse effect of thalidomide is neuropathy, which might be persistent at a cumulative dose of over 20 g.9 Although the cumulative dose of thalidomide was greater than 20 g in our patient, there were no signs of peripheral neuropathy or other adverse effects.
Both successful and failed treatments of RDD with oral thalidomide have been reported,5,6,10 suggesting that there may exist different genetic and etiologic factors in the disease. Those patients with RDD who responded to thalidomide were given doses ranging from 200 to 300 mg/d, and amenorrhea occurred after 3 weeks of treatment (cumulative dose, approximately 6 g).5,10 The present case demonstrates the effectiveness of low-dose oral thalidomide in treatment of CRDD.
Corresponding Authors: Xing-Hua Gao, MD, PhD, and Hong-Duo Chen, MD, Department of Dermatology, No. 1 Hospital of China Medical University, 155 N Nanjing St, Shenyang 110001, People’s Republic of China (firstname.lastname@example.org).
Published Online: June 19, 2013. doi:10.1001/jamadermatol.2013.4399.
Conflict of Interest Disclosures: None reported.
Funding/Support: This work was supported in part by the Program for Innovative Research Team in Universities, Liaoning Province (LT2012012), National Natural Science Foundation of China (30972659).
Role of the Sponsors: The sponsors had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; and preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.
Additional Contributions: Written informed consent was obtained from the patient for submission and publication of this case report and accompanying images.
Li X, Hong Y, An Q, Chen J, Wei H, Chen H, Gao X. Successful Treatment of Rosai-Dorfman Disease With Low-Dose Oral Thalidomide. JAMA Dermatol. 2013;149(8):992-993. doi:10.1001/jamadermatol.2013.4399