Cutaneous Rosai-Dorfman Disease – A Case Report and Review of Literature

  • Shalu Thomas Department of Pathology Believers Church Medical College and Hospital, Thiruvalla, Kerala
  • Dahlia Joseph Department of Pathology Believers Church Medical College and Hospital, Thiruvalla, Kerala
  • Renny Napolean Department of Surgery, Believers Church Medical College and Hospital, Thiruvalla, Kerala
  • Elizabeth Joseph Department of Pathology Believers Church Medical College and Hospital, Thiruvalla, Kerala
Keywords: Cutaneous, Rosai-Dorfman disease, Emperipolesis, S-100


Rosai–Dorfman disease (RDD), or Sinus Histiocytosis with Massive Lymphadenopathy (SHML) is a self-limiting, rare benign proliferative disorder of histiocytes in the lymph nodes with occasional extra-nodal involvement of the skin. Isolated Cutaneous Rosai-Dorfman disease(C-RDD) without node involvement is an exceedingly rare occurrence. Despite its unique characteristics, the diagnosis of Cutaneous Rosai Dorfman disease is hampered by its variable clinical presentation, misleading histopathological patterns, and the absence of lymphadenopathy. Herein we present a case report of Cutaneous Rosai-Dorfman disease without any lymph node involvement.


Wang KH, Chen WY, Liu HN, Huang CC, Lee WR, Hu CH. Cutaneous Rosai–Dorfman disease: clinicopathological profiles, spectrum, and evolution of 21 lesions in six patients. British Journal of Dermatology. 2006 Feb; 154: 277–286.

Khoo JJ, Rahmat BO. Cutaneous Rosai-Dorfman disease. Malaysian J Pathol.2007 Jun; 29(1): 49 – 52.

Farooq U, Chacon AH, Vincek V, Elgart GW. Purely cutaneous rosai-dorfman disease with immunohistochemistry. Indian J Dermatol. 2013; 58:447-50.

Lu C I, Kuo TT, Wong W R, Hong HS. Clinical and histopathologic spectrum of cutaneous Rosai-Dorfman disease in Taiwan. J Am Acad Dermatol 2004;51(6):931-939.

Bazyluk AB, Agnieszka B. Serwin, Puza AP, Iwona F. Cutaneous Rosai – Dorfman disease in a patient with late syphilis and cervical cancer – case report and a review of literature. BMC Dermatology. 2020; 20:19.

Krumpouzos G, Demierre MF. Cutaneous Rosai–Dorfman disease: histopathological presentation as inflammatory pseudotumor. A literature reviews. Acta Dermatol Venereol.2002; 82(4):292–296.

Hinojosa T et al. Cutaneous Rosai -Dorfman disease: A separate clinical entity. Journal of Dermatology and Dermatologic Surgery. 2017;21(2): 107-109.

Ahmed A, Crowson N, Margo CM. A comprehensive assessment of cutaneous Rosai-Dorfman disease. Ann Diagn Pathol. 2019 Jun; 40:166–173.

Noguchi S, Yatera K, Shimajiri S, Inoue N, Nagata S, Nishida C et al. Intrathoracic Rosai-Dorfman disease with spontaneous remission: a clinical report and a review of the literature. Tohoku J Exp Med. 2012; 227:231–325.

Xu Y, Han B , Yang J , Ma J, Chen J, Wang Z. Soft tissue Rosai–Dorfman disease in child .A case report and literature review .Medicine(Baltimore). 2016 Jul; 95(29): e4021.

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 Diagn Pathol. 2010; 14:309–16.

Rajib RC, Pillai R, Sulaiman IA, Haddabi IA . Soft Tissue Rosai-Dorfman Disease: Case report. Sultan Qaboos Univ Med J. 2017 Nov; 17(4): e452–e454.

Case Report