Cutaneous Rosai-Dorfman Disease

Landim, Fábio Machado; Rios, Helena de Oliveira; Costa, Carolina Oliveira; Feitosa, Roney Gonçalves Fechine; Rocha Filho, Francisco Dário; Costa, Adriana Alencar Araújo

doi:10.1590/S0365-05962009000300010

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Anais Brasileiros de Dermatologia

On-line version ISSN 1806-4841

An. Bras. Dermatol. vol.84 no.3 Rio de Janeiro July 2009

http://dx.doi.org/10.1590/S0365-05962009000300010

CASE REPORT

Cutaneous Rosai-Dorfman Disease

Fábio Machado Landim^I; Helena de Oliveira Rios^II; Carolina Oliveira Costa^III; Roney Gonçalves Fechine Feitosa^IV; Adriana Alencar Araújo Costa^V

^ISurgeon of the Skin Tumors Service of Instituto do Câncer do Ceará, General Medical Residence Coordinator of Instituto do Câncer do Ceará – Fortaleza (CE), Brazil
^IIDermatologist of Instituto do Câncer do Ceará – Fortaleza (CE), Brazil^IIIMedical Student of Universidade Estadual do Ceará – Fortaleza (CE), Brazil
^IVPathologist of Instituto do Câncer do Ceará – Fortaleza (CE), Brazil
^VPathologist of Instituto do Câncer do Ceará – Fortaleza (CE), Brazil

Mailing Address

ABSTRACT

Rosai-Dorfman disease is a self-limited benign disease. Rosai and Dorfman first described it in 1969, and the etiology of the disease remains unknown. Main manifestations are cervical adenopathy associated with fever, leukocytosis with neutrophilia and polyclonal gamaglobulinemia. Although the skin is the most common site of extra nodal disease, Rosai-Dorfman disease restricted to the skin is very rare with only a few cases described in the literature. This paper reports a rare case of cutaneous Rosai-Dorfman with skin as the sole site, and reviews the controversies of diagnosis and treatment.

Keywords:Histiocytosis, Non-langerhans-cell; Histiocytosis, sinus; Skin

INTRODUCTION

Sinus histiocytosis with massive lymphadenopathy, described in 1969 by Rosai and Dorfman,¹ is a benign and self-limited condition, characterized by cervical lymphadenopathy usually accompanied by fever, leukocytosis with neutrophilia and polyclonal gamaglobulinemia. Lymphadenopathy, is generally bilateral, painless and massive, and may also affect other lymphatic chains. It is seen at any age, but there is a preference for young male adults (1. 4:1).² The disease is more common in white and black individuals, and is rare in Asian descendent individuals.

Purely cutaneous manifestations occur in only 3% of cases, usually as red-brown or red-yellow, restricted or disseminated, papules or nodules, and without a particular site. Regarding disease restricted to the skin, the average age most frequently affected is around 49 years,³ with a slight predominance in females and Asian individuals,^2-4 unlike classical Rosai-Dorfman Disease. Despite most cases having been observed in poorer areas of the globe, no etiological agent has been isolated.

The authors of the present study present a case of skin Rosai-Dorfman Disease with a literature review on the subject, focusing on the different aspects of diagnosis and treatment of the condition.

CASE REPORT

Black, male, 27 year-old, single, Brazilian, nurse technician sought medical care with a history of painless, non erythematous, nodules on his back, without previous draining, maintaining same skin coloration, and that one year before had increased rapidly into nodules in the central region of his back, leading him to pursue medical care, occasion on which an incisional biopsy was performed. The result of the pathology exam was a lesion compatible with sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease). After diagnosis, the patient was sent to a skin tumor reference service for diagnosis and treatment. At the reference service, the patient referred absence of fever, peripheral adenopathy or weight loss. He denied alcohol consumption, smoking or use of illicit substances.

The patient’s physical examination was uneventful, and there were no peripheral lymphadenomegalies or oral mucosa lesions. BP=130x80mmHg; RR=18rpm; HR= 88bpm; T= 36°C. There was a transverse scar on the medium region of the back, where the incision biopsy had been performed; and five adjacent intradermal nodules, without changes in skin color, not adhered to the deep plane, hardened, the largest measuring about 1.5 cm, in its largest diameter. The examination of lungs, heart, abdomen and extremities was uneventful.

Lab tests, such as blood count and electrolytes were normal. Chest and cervical CT was normal, with no lymphadenopathy.

Despite a self-limited condition, we discussed management with the patient, and, due to progressive growth, lack of comfort, and aesthetical reasons, the patient preferred surgical excision of the lesion.

An ample resection was performed resulting in a 6.0 x 5.5 x 2.0 cm surgical specimen, showing a flat and undulated, dark-brown, symmetrical lesion, with irregular edges, measuring 4.0 x 1.7 cm, distant 0.1cm from the closest lateral margin. Slices showed the lesion surface was light-brown and compact, measuring 1.4cm deep, 4.3 cm long and 0.2 cm distant from the deep margin. Microscopy showed an infiltrate consisting of countless lymphocytes, plasmocytes and hystiocytes displaying a nodular arrangement affecting the dermis (Figure 1). Emperipolesis was observed in certain fields (Figures 1 and 2). Immunohistochemistry revealed CD45+, CD68+, S100+ protein (Figure 2), CD1a-. Rotation of a fasciocutaneous flap was performed. There was a small dehiscence in the flap during the post-operative, for which management was conservative. The patient evolved without developing disease in the past six months of outpatient follow-up.

DISCUSSION

Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman Disease is a proliferative histiocyte disorder, which is rare, benign and of unknown etiology. It is classified as a non-Langerhans cell histiocytosis or type II histiocytosis.

It was described for the first time in 1965 and acknowledged as a clinical-pathological entity in 1969 after the publication by Rosai and Dorfman, who described the clinical manifestations of the condition.1,5 The disease may affect any age, but is more common in the first and second decades. It has a slight preference for males (1.4:1) and the most frequently affected ethnical groups are black and white individuals, being rarely reported in Asians.² It is, in general, characterized as extensive, bilateral and painless lymphadenopathy (most commonly cervical), frequently associated with fever, anemia, leucocytosis with neutrophilia, high blood sedimentation rate and polyclonal hipergamaglobulinemia. ^2,4

Extra-nodal involvement is observed in 25-43% of cases.^6,7 Although skin is the most common extra-nodal site (11%),8 the purely cutaneous variant, that is, without lymphonode involvement, of Rosai-Dorfman Disease is very rare. Only about 3% of patients have disease limited to the skin. ⁸

Cutaneous Rosai-Dorfman Disease was described for the first time in 1978, by Thawerani et al⁹ who reported a case in a 48 year-old man with a single shoulder nodule and hypergammaglobulinemia.

Rosai-Dorfman Disease skin lesions may be single or multiple. They present as pápules,¹⁰ nodules,¹⁰ or plaques,^11,12 or a combination of these clinical variants,¹³ with or without change in the color of adjacent skin.

Optical microscopy of skin lesions show dense dermis infiltrate with histiocytes mixed to plasmocytes and lymphocytes. Histiocytes generally have a foamy aspect, and may be multinucleate and result in emperipolesis. In emperipolesis, unlike lympho- phagocytosis, lymphocytes are trapped by histiocytes, but not digested, remaining intact inside the latter. Emperipolesis is a useful criterion, albeit not specific for diagnosing Rosai-Dorfman Disease restricted to skin, given it may also be seen in neoplastic and inflammatory processes.14 Emperipolesis was observed in the microscopy of the present patient’s skin lesion (Figures 1 and 2).

Lymph nodes present capsular fibrosis and dilated nodular sinuses filled by foamy histiocytes, therefore the condition also being known as sinus histiocytosis with massive lymphadenopathy.⁴

The classical immuno-histochemistry and ultramicroscopic profile is characterized by S-100+, CD1a-, without Birbeck granules. This type of immuno-histochemistry spectrum leads to believe that sinus histiocytosis with massive lymphadenopathy is a proliferation of lymph node antigen processing interdigitating dendrite cells.²

The clinical-pathological diagnosis of Rosai-Dorfman Disease limited to the skin may sometimes be difficult due to the clinical profile and undifferentiated histopathological characteristics of the skin lesions of Rosai-Dorfman Disease affecting lymph nodes.²

Differential diagnosis of Rosai-Dorfman Dissease skin lesions is complex and very important, given that the malignant lesion variety should be ruled out, as should the association with Langerhans cells histiocytosis, which is extremely rare and fatal.¹⁵

Cutaneous Rosai-Dorfman Disease generally has a benign development with low risk of systemic involvement. Patients who do not have an association with Langerhans cell histiocytosis, but do have histiocyte pleomorphism with little mitoses, generally resolve spontaneously, or do not need surgical exeresis. Surgical excision of the skin lesion is the exclusive effective treatment for disease limited to the skin, especially for single or located lesions.

Other documented treatment options for Cutaneous Rosai-Dorfman Disease are: radiation therapy, chryotherapy, chemotherapy and isotretionin. 3 High doses of thalidomide (300 mg/dl) may be used to control extensive skin disease. ³ Treatment options depend on the extension of the disease, acceptance by the patient and adverse effects of treatment.

We conclude that, in tumors located exclusively in a single skin anatomical site, with feasible surgical resection, surgery should be considered: 1) in tumors that persist or recur; 2) when histopathology findings are inconclusive; 3) when there are symptoms; 4) if it is the patient’s wish. Surgery should aim for complete resection, with free margins, which is a very good option for loco-regional control. Patient follow-up with skin restricted disease is long (17 months on average), aimed at excluding systematic spread of the disease or local recurrence. ²

REFERENCES

1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathologic entity. Arch Pathol. 1969;87:63-70 [ Links ]

2. Brenn T, Calonje E, Granter SR, Leonard N, Grayson W, Fletcher CDM, et al. Cutaneous Rosai-Dorfman disease is a distinct clinical entity. Am J Dermatopathol. 2002;24:385-91 [ Links ]

3. Kong Y, Kong J, Shi D, Lu H, Zhu X, Wang J, et al. Cutaneous Rosai-Dorfman disease a clinical and histopathologic study of 25 cases in China. Am J Surg Pathol. 2007;31:341-50 [ Links ]

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 [ Links ]

5. Destombes P. Adenites avec surcharge lipidique, de l’enfant ou de l’adulte jeune, observe'es aux Antilles et au Mali (quatre observations). Bull Soc Pathol Exot. 1965;58:1169-75 [ Links ]

6. Mebazaa A, Trabelsi S, Denguezli M, Sriha B, Belajouza C, Nouira R. Extensive purely cutaneous Rosai-Dorfman disease responsive to acitretin. Int J Dermatol 2007;46:1208-10 [ Links ]

7. 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. Br J Dermatol. 2006;154:277–86 [ Links ]

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

9. Thawerani H, Sanchez RL, Rosai J, Dorfman RF. The cutaneous manifestations of sinus histiocytosis with massive lymphadenopathy. Arch Dermatol. 1978;114:191-7 [ Links ]

10. Tsang WY, Chan JK, Yu HC, Chow LT. Extranodal Rosai-Dorfman disease: an uncommon cause of persistent nodule in the ear. J Laryngol Otol. 1992;106:249-51 [ Links ]

11. Kang JM, Yang WI, Kim SM, Lee MG. Sinus histiocytosis (Rosai-Dorfman disease) clinically limited to the skin. Acta Derm Venereol. 1999;79:363-5 [ Links ]

12. Scheel MM, Rady PL, Tyring SK, Pandya AG. Sinus histiocytosis with massive lymphadenopathy: presentation as giant granuloma annulare and detection of human herpes virus 6. J Am Acad Dermatol. 1997;37:643-6 [ Links ]

13. Annessi G, Gianetti A. Purely cutaneous Rosai- Dorfman disease. Br J Dermatol. 1996;134:749-53 [ Links ]

14. Motta L, Mc Menamin ME, Thomas MA, Calonje E. Crystal deposition in a case of cutaneous Rosai-Dorfman disease. Am J Dermatopathol. 2005;27:339-42 [ Links ]

15. Wang KH, Cheng CJ, Hu CH, Lee WR. Coexistence of localized Langerhans cell histiocytosis and cutaneous Rosai-Dorfman disease. Br J Dermatol. 2002;147:770-4 [ Links ]

Mailing Address:
Roney Gonçalves Fechine Feitosa
Alameda das Verbenas n°142, Cidade 2000
60190-330 - Fortaleza CE
Tel./fax: (85) 88285458 / (85) 32490080
email: roneyfechine@gmail.com

Conflict of interest: None
Financial funding: None
How to cite this article: Landim FM, Rios HO, Costa CO, Feitosa RGF, Rocha Filho FD, Costa AAA. Doença de Rosai-Dorfman cutânea. An Bras Dermatol. 2009;84(3):275-8.