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Year : 2013  |  Volume : 56  |  Issue : 1  |  Page : 60-61
Cutaneous Rosai-Dorfman Disease: Report of a case


Department of Pathology, Al Jahra Hospital, Kuwait

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Date of Web Publication6-Aug-2013
 

How to cite this article:
Singh NG, Mannan AA. Cutaneous Rosai-Dorfman Disease: Report of a case. Indian J Pathol Microbiol 2013;56:60-1

How to cite this URL:
Singh NG, Mannan AA. Cutaneous Rosai-Dorfman Disease: Report of a case. Indian J Pathol Microbiol [serial online] 2013 [cited 2020 Jul 4];56:60-1. Available from: http://www.ijpmonline.org/text.asp?2013/56/1/60/116154


A 37-year-old man presented in the surgical outpatient department with one month history of a progressively enlarging painless right neck swelling. On examination, the lesion revealed a 1 × 1 cm reddish nodule on the right posterior triangle of the neck. It was non-tender and freely mobile. There was no associated cervical lymphadenopathy. His other systemic and vital records were noncontributory. The laboratory investigation revealed no significant abnormality. The skin lesion was excised under general anesthesia. In the Pathology Department, we received a small, partially skin covered, well circumscribed nodule, measuring 1 cm in diameter. Cut section was grey-white and firm in consistency. Microscopic examination revealed a well circumscribed dermal lesion [Figure 1]a composed of diffuse sheets of histiocytes with abundant pale cytoplasm admixed with polymorphs, lymphocytes, plasma cells and few scattered eosinophils [Figure 1]b. There was prominent emperipolesis of lymphocytes, polymorphs and plasma cells [Figure 1]c. No atypical cells or epethelioid cell granuloma was seen. Immunological staining showed that the histiocytes were positive for CD68, S-100 protein [Figure 1]d and were negative for CD1a and CD20. Thus, a diagnosis of cutaneous Rosai-Dorfman disease was entertained.
Figure 1: (a) Photomicrograph of a well circumscribed cutaneous nodular lesion (H and E, ×200). (b) Exhibiti ng diff use sheets of histi ocytes admixed with lymphocytes, plasma cells, polymorphs and few scatt ered eosinophils (H and E, ×100). (c) There is prominent emperipolesis of lymphocytes (arrows) (H and E, ×400). (d) Immunohistochemistry reveals positi vity for S-100 protein in the histi ocyti c cells (avidine and bioti n, ×400)

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Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML) is characterized by massive painless bilateral lymph node enlargement in the neck. [1] This lesion usually affects males in their first to second decade of life. Most patients present with massive cervical lymphadenopathy, but about 40% of patients will have extra-nodal manifestations involving the skin, nasal cavity and paranasal sinus, eye and ocular adnexa, bone, central nervous system, liver and heart. [1] In our present case there was no associated cervical lymphadenopathy. The exact etiology of SHML is not known. An underlying immune dysfunction has been postulated. Possibility of viruses like Human Herpes Virus 6, Klebsiella, Epstein-Barr virus, Cytomegalovirus or Brucella as a causative agent has been postulated as well. Patient may manifest hematologic abnormalities, such as normocytic or microcytic anemia and polyclonal hypergammaglobulinemia. [2]

Although SHML is relatively rare, purely cutaneous RDD is even less common, accounting for approximately 3% of SHML cases in one large study.Patients with cutaneous RDD seem to have an older age of onset of disease (43.5 years in one series) and higher rates of disease in women, Asians and whites. [3],[4] Most patients with cutaneous RDD do not have associated systemic symptoms such as fever, malaise or night sweats, although a few have been reported to have fatigue and weight loss. Clinically it may present as single or multiple macules, papules, plaques, or nodules ranging in color from red-brown to yellow. [3],[4] The lesions may be indurated and can show central atrophy or ulceration.The lesions of cutaneous RDD show no predilection for any specific location. Sometimes it could be localized in the soft tissue and manifest as subcutaneous mass or panniculitis. [4] Cutaneous RDD has been clinically mistaken for other dermatologic disorders including vasculitis, acne vulgaris, hidradenitis suppurativa, granuloma annulare, lupus vulgaris, sarcoidosis, and other histiocytoses.

Although histopathologic examination is often the key to diagnosis, especially in the absence of lymphadenopathy or other systemic symptoms, it is worth noting that the difficulty in obtaining a pathological diagnosis is not uncommon.The hallmark of the cutaneous RDD is the presence of a variable number of histiocytes with abundant pale cytoplasm exhibiting prominent emperipolesis of lymphocytes, polymorphs and plasma cells. Those histiocytes may be intimately admixed with mature lymphocytes, plasma cell, polymorphs and eosinophils. Immunological staining showed that the histiocytes were positive for CD68, S-100 protein and were negative for CD1a and CD20. [2] However many reported cases were initially confused with juvenile xanthogranuloma, malignant histiocytosis, reticulohistiocytoma cutis, hemophagocytic syndrome, Langerhan's cell histiocytosis and T cell lymphoma. [3],[4]

Several pitfalls may also complicate pathologic diagnosis. The variable amounts of cellular reactive fibrosis displayed in storiform pattern in cutaneous lesions of RDD may be confused with a dermatofibroma. Fibrosis and foamy histiocytes may be especially prominent in older lesions. Epidermal changes including pseudoepitheliomatous hyperplasia may be present. Tissue cultures, special stains for infection, immunohistochemical studies, and possibly even electron microscopy to evaluate for Birbeck granules may be required before the diagnosis of RDD is reached.

When RDD is limited to the skin and soft tissue, the prognosis is usually good, and there is only a small risk of developing systemic disease. [3],[4] Most of the cutaneous lesions generally do not require any further treatment, although roentgen therapy has been described as a useful therapeutic modality when the cutaneous disease runs a prolong course or when the lesions are aesthetically unacceptable to the patient. Cutaneous lesions have been reported to respond to topical and oral corticosteroids, high-dose thalidomide and cryotherapy. [5]

To conclude, cutaneous RDD should be one of the uncommon differential diagnoses for any lesion that is manifested as cutaneous nodule. Purely cutaneous RDD is very rare and diagnosis is difficult without associated lymphadenopathy. However a combined clinical evaluation and histopathological examination of the lesion may enable us to impart a correct diagnosis.

 
   References Top

1.Foucar E, Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Review of the entity. Semin Diagn Pathol1990;7:19-73.  Back to cited text no. 1
    
2.McClain KL, Natkunam Y, Swerdlow SH. Atypical cellular disorders. Hematology Am Soc Hematol Educ Program 2004:283-96.  Back to cited text no. 2
    
3.Frater JL, Maddox JS, Obadiah JM, Hurley MY. CutaneousRosai-Dorfman disease: Comprehensive review of cases reported in the medical literature since 1990 and presentation of an illustrative case. J Cutan Med Surg2006;10:281-90.  Back to cited text no. 3
    
4.Lu CI, Kuo TT, Wong WR, Hong HS. Clinical and histopathologic spectrum of cutaneous Rosai-Dorfman disease in Taiwan. J Am Acad Dermatol 2004;51:931-9.  Back to cited text no. 4
    
5.Fumerton R, Ball N, Zhou Y. Refractory cutaneousRosai-Dorfman disease responsive to cryotherapy. Cutis 2011;87:296-9.  Back to cited text no. 5
    

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Correspondence Address:
Naorem G Singh
Department of Pathology, Al Jahra Hospital, P.O. Box 62276, Jahra
Kuwait
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.116154

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