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Dermatology Practical & Conceptual

ISSN 2160-9381

April 2012 | Volume 2, No. 2

Sinus histiocytosis (Rosai-Dorfman disease) presenting with solitary cutaneous nodule: a very rare clinical entity

Emilia Duarte-Williamson, M.D.1, Fiona Antony, M.D.2, Radu Rotarescu, M.D.3 1 Dermatology Department, Kent and Canterbury Hospital, Canterbury, Kent, UK 2 Dermatology Department, Frimley Park Hospital, Frimley, Surrey, UK 3 Dermatology Department, Stafford General Hospital, Stafford, Staffordshire, UK

Keywords: histiocytosis; sinus histiocytosis; Rosai-Dorfman; nodule;

Citation: Duarte-Williamson E, Antony F, Rotarescu R. Sinus histiocytosis (Rosai- Dorfman disease) presenting with solitary cutaneous nodule: a very rare clinical entity. Dermatol Pract Conc. 2012;2(2):4. http://dx.doi.org/10.5826/dpc.0202a04.

History: Received: January 22, 2012. Accepted: February 25, 2012. Published: April 30, 2012.

Copyright: ©2012 Duarte-Williamson et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Funding: None.

Competing interests: The authors have no conflicts of interest to disclose.

Corresponding Author: Radu Rotarescu, , Staff Residences, Block 1, Flat/House 9, Room 1, Cowan Drive, Stafford, ST16 3RS, United Kingdom. Tel. 07405137128; Fax. 01543576134. Email: radur30@gmail.com

Abstract

Sinus histiocytosis is a rare inflammatory disease mainly affecting the cervical lymph nodes, presenting with skin lesions in 10% of cases. Our patient had a solitary nodule on the trunk without any other clinical signs. The histology reported a dermal neoplasm composed mainly of macrophages and lymphocytes. Macrophages were aggregated in clusters resembling lymph node sinuses. Lymphophagocytosis or emperipolesis (the presence of an intact cell within the cytoplasm of another cell) was noted and the diagnosis of sinus histiocytosis established.

Introduction

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, is a very rare inflammatory entity. It is generally self-limited and confined mainly to the cervical lymph nodes. Cutaneous manifestations are present in approximately 10% of patients [1]. It can be accompanied by fever, neutrophilia, polyclonal hypergammaglobulinemia and an elevated erythrocyte sedimentation rate.

Sinus histicytosis is part of the non-Langerhans cell histiocytosis or cutaneous non-histiocytosis X, in which proliferation of macrophages always lack Langherhans granules. This group of disorders is usually nonaggressive, self-healing and can affect both children and adults [2-7].

Report

We report a very rare case of sinus histiocytosis, which presented with only a solitary cutaneous nodule and no other clinical manifestations or lymphadenopathy.

A 75-year-old man was referred to the Dermatology Department with a six-month history of an asymptomatic solitary nodule on the trunk. His general practitioner had diagnosed a possible pyogenic granuloma, but could not exclude a malignant lesion. His medical history was unremarkable and he was otherwise well. Examination revealed a 1.2 cm purple, soft, shiny nodule with a violaceous peripheral rim in the interscapular area (Figure 1). Dermoscopy was featureless.

Figure 1. Purple, soft, shiny nodule with a violaceous peripheral rim in the interscapular area. [Copyright: ©2012 Duarte-Williamson et al.]

The lesion was excised immediately with the clinical suspicion of a probable amelanotic melanoma, squamous cell carcinoma, or Merkel cell carcinoma in mind. The pathology reported a dermal lesion composed of an infiltrate of cells expanding and filling the papillary dermis and spreading into the deep reticular dermis (Figure 2a). The infiltrate included prominent macrophages with pale, vacuolated cytoplasm, some multinucleate macrophages of similar staining characteristics, other small, untransformed macrophages and a large number of lymphoid cells. Lymphocytes were predominant, but a modest number of plasma cells with a smaller number of other inflammatory cells were seen. The large pale staining macrophagic cells were positive for CD 68. They also expressed S100 protein, but HMB45 was negative. The large pale staining cells were PAS negative. No microorganisms were seen in a section stained by the Ziehl-Neelsen method.

Figure 2a. Dermal tumor composed of an infiltrate of cells expanding and filling the papillary dermis and spreading into the superficial reticular dermis showing a diffuse dermal infiltrate. Haematoxylin and eosin stain, original magnification x10. [Copyright: ©2012 Duarte-Williamson et al.]

Macrophages were aggregated in clusters resembling lymph node sinuses. Lymphophagocytosis or emperipolesis (the presence of an intact cell within the cytoplasm of another cell) was noticed and the diagnosis of sinus histiocytosis established (Figure 2b).

Figure 2b. The infiltrate included macrophages, large number of lymphoid cells, modest number of plasma cells and smaller number of other inflammatory cells. Macrophages were aggregated in clusters resembling lymph node sinuses. Lymphophagocytosis or emperipolesis (the presence of an intact cell within the cytoplasm of another cell) was noticed. Haematoxylin and eosin stain, original magnification x40. [Copyright: ©2012 Duarte-Williamson et al.]

With this surprise diagnosis the patient was seen in clinic and a thorough physical examination was carried out. No lymphadenopathy or other clinical abnormality was found. The laboratory investigations (full blood count, C reactive protein, erythrocyte sedimentation rate, coagulation screen, electrolytes, renal and liver function tests) were all normal. Levels of immunoglobulins G, A, M and serum protein electrophoresis were also normal.

Discussion

Approximately 365 cases of sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, have been described in the literature [2]. Sometimes the cutaneous manifestations may be the initial and sole presenting feature of this disease [2]. Massive bilateral cervical lymphadenopathy is usually the hallmark of this condition; however, our patient presented with only a solitary cutaneous nodule with no lymph node enlargement, making the diagnosis more difficult.

Cutaneous lesions are reported to be polymorphic with yellowish patches and macules, reddish-brown papules, plaques and nodules that can become eroded or ulcerated [2]. The aetiology of the disease remains unclear, but a possible disturbance of cell-mediated immunity or a primary infection hypothesis with Epstein-Barr virus have been suspected [2].

The differential diagnosis is very broad and includes both proliferative and inflammatory diseases. In our case the initial suspicion was of a cutaneous malignancy.

Most of the sinus histiocytoses are self-healing with spontaneous regression over a period of months to years. Very rarely when an internal organ is involved, for example, liver, spleen, upper respiratory tract, then glucocorticoids or other chemotherapeutic regimens may be required. Solitary cutaneous lesions can be excised. Widespread cutaneous disease has been treated successfully with dapsone or thalidomide [1].

Our patient is undergoing close clinical follow-up, as it is possible that the solitary cutaneous nodule is the precursor to more extensive sinus histiocytosis (Rosai-Dorfman disease) [1]. However, even after a long follow-up of 12 months, there are no signs of disease progression.

Acknowledgement

Many thanks to Dr. Fiona Antony for the histopathological diagnosis and for the assistance with the case.

References

1. Burgdorf WHC, Plewig G, Wolff HH, et al. Sinus histiocytosis with massive lymphadenopathy. In: Burgdorf WHC, Plewig G, Wolff HH, et al. Braun-Falco’s Dermatology 3rd ed. Heidelberg: Springer Medizin Verlag, 2009:1528-9.

2. Freedberg IM, Eisen AZ, Wolff K, et al. Sinus histiocytosis with massive lymphadenopathy. In: Freedberg IM, Eisen AZ, Wolff K, et al. Fitzpatrick’s Dermatology in General Medicine. 6th ed., New York: McGraw Hill Professional, 2003:1593-4.

3. Weitzman, Jaffe R. Uncommon histiocytic disorders: the non-Langherhans cell histiocytoses. Pediatr Blood Cancer. 2005;45(3):256-64.

4. Flater JL, Maddox JS, Obadiah JM, Hurley MY. Cutaneous Rosai-Dorfman disease: comprehensive review of cases reported in the medical literature since 1990 and presentation of an illustrative case. J Cutan Med Surg. 2006;10(6):281-90.

5. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7(1):19-73.

6. Kong YY, Kong JC, Shi DR, et al. Cutaneous Rosai-Dorfman disease: a clinical and histopathologic study of 25 cases in China. Am J Surg Pathol. 2007;31(3):341-50.

7. Wang KH, Chen WY, Liu HN, Huang CC, Lee WR, Hu CH. Cutaneous Rosai-Dorfman disease: clinicopathological profiles, spectrum of evolution of 21 lesions in six patients. Br J Dermatol 2006;154(2):277-86.