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Year : 2021  |  Volume : 66  |  Issue : 2  |  Page : 223
A case of cutaneous rosai-dorfman disease treated by intralesional injections of glucocorticoid


Department of Dermatology, The Third Hospital of Soochow University, Changzhou, Jiangsu, China

Date of Web Publication16-Apr-2021

Correspondence Address:
Ru-zhi Zhang
185 Juqian Road, Changzhou - 213003, Jiangsu
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_508_18

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   Abstract 


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.


Keywords: Intralesional Compound Betamethasone, Rosai-Dorfman disease, skin leisions


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

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 May 6];66:223. Available from: https://www.e-ijd.org/text.asp?2021/66/2/223/313788





   Introduction Top


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 Top


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: (a) Two reddish-brown plaques infiltrating the right scapular region of the patient's back. (b) A peanut-size red nodule on the patientfs right shoulder

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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: (a) Infiltration of dense multinodular cells in the dermis. Most histiocytes had an abundant eosinophilic cytoplasm and lymphocytes, as well as occasional plasma cells. (H and E, ×40). (b) Emperipolesis of histiocytes. (H and E, ×200)

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Figure 3: After three treatments, the lesions displayed a shrunken surface and were dim in color

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Figure 4: At the 6-month follow-up visit, the lesions had become flat and tended to fade

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   Discussion Top


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.

Acknowledgments

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63-70.  Back to cited text no. 1
    
2.
Sarwal R, Tu E, Mendelblatt FI, Sugar J, Gross SA, Pulido JS, et al. Atypical ocular presentations of Rosai-Dorfman disease. Ocul Immunol Inflamm 2008;16:9-15.  Back to cited text no. 2
    
3.
Al-Daraji W, Anandan A, Klassen-Fischer M, Auerbach A, Marwaha JS, Fanburg-Smith JC. Soft tissue Rosai-Dorfman disease: 29 new lesions in 18 patients, with detection of polyomavirus antigen in 3 abdominal cases. Ann of Diag Pathol 2010:14:309-16.  Back to cited text no. 3
    
4.
Kong YY, Kong JC, Luo Yan LU. Clinicohistopathologic features of cutaneous Rosai-Dorfman disease: A study of 14 cases. J Clin Dermatol 2009;38:137-40.  Back to cited text no. 4
    
5.
Vuong V, Moulonguet I, Cordoliani F, Crickx B, Bezier M, Vignon-Pennamen MD, et al. Cutaneous revelation of Rosai-Doffman disease: 7 cases. Ann Dermatol Venereol 2013;140:83-90.  Back to cited text no. 5
    
6.
Juskevicius R, Finley JL. Rosai-Dorfman disease of the parotid gland: Cytologic and histopathologic findings with immunohistochemical correlation. Arch Path Lab Med 2001;125:1348-50.  Back to cited text no. 6
    
7.
Nadal M, Kervarrec T, Machet MC, Petrella T, Machet L. Cutaneous Rosai-Dorfman disease located on the breast: Rapid effectiveness of methotrexate after failure of topical corticosteroids, acitretin and thalidomide. Acta Derm Venereol 2015;95:758-9.  Back to cited text no. 7
    
8.
Fening K, Bechtel M, Peters S, Zirwas M, Darabi K. Cutaneous Rosai-Dorfman disease persisting after surgical excision: Report of a case treated with acitretin. J Clin Aesthet Dermatol 2010;3:34-6.  Back to cited text no. 8
    
9.
Chan CC, Chu CY. Dapsone as a potential treatment for cutaneous Rosai-Dorfman disease with neutrophilic predominance. Arch Dermatol 2006;142:428-30.  Back to cited text no. 9
    
10.
Fumerton R, Ball N, Zhou Y. Refractory cutaneous Rosai-Dorfman disease responsive to cryotherapy. Cutis 2011;87:296-9.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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