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Successful Treatment of Rosai-Dorfman Disease With Low-Dose Oral Thalidomide FREE

Xiaodong Li, MD, PhD1,2; Yuxiao Hong, MD2; Qian An, MD2; John Chen, MD3; Huachen Wei, MD, PhD4; Hong-Duo Chen, MD2; Xing-Hua Gao, MD, PhD2
[+] Author Affiliations
1Fengtian Hospital Affiliated with Shenyang Medical College, Shenyang, China
2Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang
3Sheftel Associates Dermatology, Tucson, Arizona
4Department of Dermatology, Dermatology Research Laboratories, Mount Sinai Medical Center, New York, New York
JAMA Dermatol. 2013;149(8):992-993. doi:10.1001/jamadermatol.2013.4399.
Text Size: A A A
Published online

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.

REPORT OF A CASE

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.

Place holder to copy figure label and caption
Figure 1.
Clinical Images

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.

Graphic Jump Location

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.

DISCUSSION

Various treatment options, including administration of high-dose thalidomide, steroids, chemotherapy, or surgical excision, have been reported with variable success.58 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.

ARTICLE INFORMATION

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 (gaobarry@hotmail.com).

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.

REFERENCES

Rosai  J, Dorfman  RF.  Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol. 1969;87(1):63-70.
PubMed
Chu  P, LeBoit  PE.  Histologic features of cutaneous sinus histiocytosis (Rosai-Dorfman disease): study of cases both with and without systemic involvement. J Cutan Pathol. 1992;19(3):201-206.
PubMed   |  Link to Article
Saboo  SS, Jagannathan  JP, Krajewski  KM,  et al.  Symptomatic extranodal Rosai-Dorfman disease treated with steroids, radiation, and surgery. J Clin Oncol. 2011;29(31):e772-e775.
PubMed   |  Link to Article
Hinduja  A, Aguilar  LG, Steineke  T, Nochlin  D, Landolfi  JC.  Rosai-Dorfman disease manifesting as intracranial and intraorbital lesion. J Neurooncol. 2009;92(1):117-120.
PubMed   |  Link to Article
Tjiu  JW, Hsiao  CH, Tsai  TF.  Cutaneous Rosai-Dorfman disease: remission with thalidomide treatment. Br J Dermatol. 2003;148(5):1060-1061.
PubMed   |  Link to Article
Satter  EK, Graham  BS, Steger  JW.  Response of cutaneous Rosai-Dorfman disease to topical and intralesional steroids. Br J Dermatol. 2003;149(3):672-674.
PubMed   |  Link to Article
Gebhardt  C, Averbeck  M, Paasch  U,  et al.  A case of cutaneous Rosai-Dorfman disease refractory to imatinib therapy. Arch Dermatol. 2009;145(5):571-574.
PubMed   |  Link to Article
Chang  HS, Son  SJ, Cho  KH, Lee  JH.  Therapeutic challenge of dapsone in the treatment of purely cutaneous Rosai-Dorfman disease. Clin Exp Dermatol. 2011;36(4):420-422.
PubMed   |  Link to Article
Chen  M, Doherty  SD, Hsu  S.  Innovative uses of thalidomide. Dermatol Clin. 2010;28(3):577-586.
PubMed   |  Link to Article
Kroumpouzos  G, Demierre  MF.  Cutaneous Rosai-Dorfman disease: histopathological presentation as inflammatory pseudotumor: a literature review. Acta Derm Venereol. 2002;82(4):292-296.
PubMed   |  Link to Article

Figures

Place holder to copy figure label and caption
Figure 1.
Clinical Images

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.

Graphic Jump Location

Tables

References

Rosai  J, Dorfman  RF.  Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol. 1969;87(1):63-70.
PubMed
Chu  P, LeBoit  PE.  Histologic features of cutaneous sinus histiocytosis (Rosai-Dorfman disease): study of cases both with and without systemic involvement. J Cutan Pathol. 1992;19(3):201-206.
PubMed   |  Link to Article
Saboo  SS, Jagannathan  JP, Krajewski  KM,  et al.  Symptomatic extranodal Rosai-Dorfman disease treated with steroids, radiation, and surgery. J Clin Oncol. 2011;29(31):e772-e775.
PubMed   |  Link to Article
Hinduja  A, Aguilar  LG, Steineke  T, Nochlin  D, Landolfi  JC.  Rosai-Dorfman disease manifesting as intracranial and intraorbital lesion. J Neurooncol. 2009;92(1):117-120.
PubMed   |  Link to Article
Tjiu  JW, Hsiao  CH, Tsai  TF.  Cutaneous Rosai-Dorfman disease: remission with thalidomide treatment. Br J Dermatol. 2003;148(5):1060-1061.
PubMed   |  Link to Article
Satter  EK, Graham  BS, Steger  JW.  Response of cutaneous Rosai-Dorfman disease to topical and intralesional steroids. Br J Dermatol. 2003;149(3):672-674.
PubMed   |  Link to Article
Gebhardt  C, Averbeck  M, Paasch  U,  et al.  A case of cutaneous Rosai-Dorfman disease refractory to imatinib therapy. Arch Dermatol. 2009;145(5):571-574.
PubMed   |  Link to Article
Chang  HS, Son  SJ, Cho  KH, Lee  JH.  Therapeutic challenge of dapsone in the treatment of purely cutaneous Rosai-Dorfman disease. Clin Exp Dermatol. 2011;36(4):420-422.
PubMed   |  Link to Article
Chen  M, Doherty  SD, Hsu  S.  Innovative uses of thalidomide. Dermatol Clin. 2010;28(3):577-586.
PubMed   |  Link to Article
Kroumpouzos  G, Demierre  MF.  Cutaneous Rosai-Dorfman disease: histopathological presentation as inflammatory pseudotumor: a literature review. Acta Derm Venereol. 2002;82(4):292-296.
PubMed   |  Link to Article

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