Indian Journal of Dermatology
: 2021  |  Volume : 66  |  Issue : 2  |  Page : 223-

A case of cutaneous rosai-dorfman disease treated by intralesional injections of glucocorticoid

Yan-xia Yuan, Chun-xing Xu, Ru-zhi Zhang 
 Department of Dermatology, The Third Hospital of Soochow University, Changzhou, Jiangsu, China

Correspondence Address:
Ru-zhi Zhang
185 Juqian Road, Changzhou - 213003, Jiangsu


A 45-year-old male presented with painless reddish-brown plaques and nodules that had infiltrated his shoulder and back for 3 months. From the clinical manifestations and histopathological findings, the patient was diagnosed with cutaneous Rosai-Dorfman disease. Intralesional injections of betamethasone (trade name: diprospan) were recommended and the lesions improved significantly after three treatments.

How to cite this article:
Yuan Yx, Xu Cx, Zhang Rz. A case of cutaneous rosai-dorfman disease treated by intralesional injections of glucocorticoid.Indian J Dermatol 2021;66:223-223

How to cite this URL:
Yuan Yx, Xu Cx, Zhang Rz. A case of cutaneous rosai-dorfman disease treated by intralesional injections of glucocorticoid. Indian J Dermatol [serial online] 2021 [cited 2021 Dec 8 ];66:223-223
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The purely cutaneous form of the disease, namely cutaneous RDD (CRDD), is a rare benign non-Langerhans histiocytosis of unknown cause. The phenomenon of emperipolesis is suggestive but not totally specific. Various kinds of treatment have been applied with different outcomes. Here, we report a case of CRDD cured by intralesional injections of glucocorticoid.

 Case Report

A 45-year-old Chinese male with no identifiable past medical history consulted us for cutaneous lesions located on his shoulder and back. A red nodule had appeared 3 months earlier and grew progressively in size with no pain or pruritus. Subsequently, several papules and nodules appeared nearby and merged to form a dusky-red infiltrated plaque. A month later, similar red nodules appeared on his shoulder and waist and gradually enlarged. The patient denied any local trauma or infection. He had no symptoms of fever, malaise, or weight loss and had no history of hypertension, diabetes, food, and/or drug allergies. There were no similar patients in his family.

Physical examination revealed two dark red plaques that infiltrated his back [Figure 1]a and a peanut-size red nodule with a smooth surface on his right shoulder [Figure 1]b. The boundaries of the two plaques were clear and irregular. There was no enlargement of lymph nodes palpated in his neck, axillary fossa, or groin.{Figure 1}

Laboratory investigations indicated normal results for routine tests of blood and urine, liver, and kidney functions, and hematological examination of sexually transmitted diseases was normal. A skin biopsy specimen showed an intradermal infiltration of lymphocytes, histiocytes, multinucleated giant cells, and a few plasma cells [Figure 2]a. A high-power view showed emperipolesis [Figure 2]b. From those clinical manifestations and histological features, the diagnosis of cutaneous Rosai-Dorfman disease (CRDD) was made. Treatment with acitretin at 20 mg daily for 3 weeks did not show significant improvement and was terminated because of the obvious dryness of his lips. An intralesional injection of betamethasone (trade name: Diprospan) was then prescribed, once every 3 weeks, three times in total. After 3 weeks of the first treatment, the lesions had a shrunken surface and were dim in color. At the 9-week visit, the patient was satisfied with the improvement of his skin lesions [Figure 3], which further improved during the next 6 months of follow-up [Figure 4]. During that period, the patient did not complain of any discomfort.{Figure 2}{Figure 3}{Figure 4}


Rosai-Dorfman disease (RDD), first reported by Rosai and Dorfman in 1969, is also known as sinus histiocytosis with massive lymphadenopathy.[1] It is a rare benign non-Langerhans histiocytosis of unknown cause. Its etiology and pathogenesis remain unknown, but numerous studies demonstrating a polyclonal infiltrate and the presence of functionally activated macrophages support a reactive process, which may be related to possible infections or following an immune challenge. A possible viral etiology of RDD has also been suggested by several studies.[2],[3]

Clinically, RDD is frequently characterized by a febrile illness associated with massive cervical lymphadenopathy, skin lesions, hyperglobulinemia, leukocytosis and an elevated sedimentation rate. Lymphadenopathy is the main clinical manifestation. Extranodal involvement has been observed in diverse organs, with the skin being the most affected. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without involvement of other organs or tissues.[4]

The purely cutaneous form of the disease, namely CRDD, is a rare and distinct entity, usually presenting as erythematous to brown papules, plaques, or nodules with no predilection for site. In the literature, CRDD mainly manifests as papulonodular-type and infiltrating plaque-type lesions, and tumor-like lesions are rare. Skin lesions not only involve the face but also occur on the trunk, limbs, neck, and so on.[5]

The diagnosis of CRDD mainly depends on histopathological examination, and its pathological features are characterized by a dense cellular infiltration involving the dermal and subcutaneous tissues. The phenomenon of emperipolesis (phagocytosis of lymphocytes, neutrophil polymorphonuclear cells, or plasma cells), which is suggestive but not totally specific, is the key point for differentiating CRDD from other diseases. Immunohistochemically, the histiocytes show reactivity for S-100 protein and CD68.[6]

Because of the normal self-limited course and mild clinical symptoms, aggressive therapies are not recommended for CRDD. The skin lesions may even regress spontaneously in several months. Thus, some patients with CRDD will not require treatment. Because it is so rare, standard therapies for CRDD have not been established. For patients requiring treatment, local and/or systemic therapy can be selected depending on individual circumstances, including topical or intralesional corticosteroids, cryotherapy, radiotherapy, corticosteroids, dapsone, thalidomide, isotretinoin, acitretin, α-interferon, and imatimib. These treatments have been reported to have variable efficacies.[7],[8],[9],[10] Surgical excision should be an appropriate option for single nodal or localized primary lesions. However, there was a report of a regrowth of a skin lesion after it had been completely removed.[8] Fumerton et al. reported a case of CRDD that was refractory to both topical and intralesional corticosteroid therapies but showed a rapid and remarkable response to cryotherapy.[10] Radiotherapy may provide an effective approach for treating CRDD. Our patient showed a good response to an intralesional injection of glucocorticoid, but long-term follow-up is needed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


The authors are very grateful to Professor V.J. Hearing (Laboratory of Cell Biology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA) for help with the English language editing. The work was supported by the National Natural Science Foundation of China (Grant No. 81673078) and the Science and Technology Foundation of Jiangsu, China (Grant No.BL2014036).

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


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