Dermatology Online Journal

Cutaneous Rosai-Dorfman disease
JoEllen Van Zander MD
Dermatology Online Journal 10 (3): 12

From the Ronald O. Perelman Department of Dermatology, New York University

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Abstract

Presented is a case of a 31-year-old woman with cutaneous Rosai-Dorfman disease, which is a rare, benign, generally self-limited histiocytic proliferative disorder that is characterized by painless lymphadenopathy, fever, neutrophilia, an elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. It primarily involves lymph nodes but can affect extranodal sites such as the skin, where it typically appears as indistinct erythematous papules and nodules. On histopathologic examination, emperipolesis is a consistent finding. The etiology is unknown, and treatment is based on clinical manifestations.

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History.—The patient initially presented to the Dermatology Clinic at Bellevue Hospital Medical Center for evaluation of eczema. She was noted to have a growth over her right scapula. She claimed the lesion had been present for approximately 6 months and had been slowly expanding. She complained of a mild itch but was otherwise asymptomatic. She denied fevers, chills, or myalgias.

Physical Examination.—A cluster of erythematous papules that coalesced into a plaque 2 cm in diameter was present overlying the right scapula. No lymphadenopathy or hepatosplenomegaly was present.

Figure 1	Figure 2

Laboratory Data.—The complete blood count was normal. The erythrocyte sedimentation rate was elevated to 34 mm/hr. Serum protein electrophoresis showed diffuse IgG hypergammaglobulinemia. No lymphadenopathy was present on chest, abdomen, or pelvis computed-tomography scans.

Histopathology.—Throughout the reticular dermis, there is a multinodular infiltrate of lymphocytes, plasma cells, and histiocytes with abundant finely vacuolated eosinophilic cytoplasm. Within their cytoplasm, occasional histiocytes contain intact lymphocytes (emperipolesis). These histiocytes stain positively with antibodies to S-100 protein. A Fite stain for acid-fast bacilli, a periodic-acid-Schiff stain for fungi, and a Steiner stain for spirochetes are negative. Polarization fails to show foreign material.

Diagnosis.—Cutaneous Rosai-Dorfman disease.

Comment:

Rosai-Dorfman disease, or sinus histiocytosis with lymphadenopathy, is an uncommon, often protracted, histiocytic proliferative disorder. It usually appears in patients in the first or second decades of life as a febrile illness that is accompanied by extensive but painless lymphadenopathy (most commonly cervical), fever, anemia, an elevated erythrocyte sedimentation rate, neutrophilia, and polyclonal hypergammaglobulinemia. Extranodal involvement occurs in 43 percent of the cases with skin being the most common site. About 10 percent of patients have skin lesions, and 3 percent of patients have disease detectable only in the skin. [1] Cutaneous lesions are characterized by isolated or disseminated, yellow-brown papules or nodules or macular erythema. Panniculitis and acneiform lesions have also been described. Most patients with skin lesions are about 40 years old at presentation. [2] Immune disorders occur in approximately 13 percent of patients with Rosai-Dorfman disease, with antierythrocyte autoantibodies and joint disease being the most common findings. Patient with associated immunologic abnormalities have a less favorable prognosis. [3]

Cutaneous lesions demonstrate a dense, dermal infiltrate of histiocytes with scattered lymphocytes, plasma cells, and neutrophils. The histiocytes have large vesicular nuclei, small nucleoli, and abundant, foamy, eosinophilic cytoplasm with feathery borders. Emperipolesis, which is the presence of intact lymphocytes (and rarely plasma cells, neutrophils, and red blood cells) within histiocytes, is common. Infiltrating histiocytes stain positively for S100, CD11c, CD14, CD68, laminin 5, and lysozyme. No Birbeck granules are present on electron microscopy. [4]

The etiology of Rosai-Dorfman disease is unknown although a viral pathogenesis has been postulated. Numerous reports have identified human herpesvirus 6 (HHV-6) in visceral and cutaneous lesions. [5] However, HHV-6 has frequently been found in many reactive and infectious disorders of lymphoid tissue, and its presence in Rosai-Dorfman disease is non-specific.

Treatment should be based on clinical manifestations. Many lesions are asymptomatic, heal spontaneously, and do not require treatment. When destructive or disseminated disease is present, radiotherapy, surgical excision, systemic glucocorticoids and alkylating agents may be used. [6]

References

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

2. Carrington PR, et al. Extranodal Rosai-Dorfman disease (RDD) of the skin. Int J Dermatol 1998; 37:271.

3. Foucar E, et al. Sinus histiocytosis with massive lymphadenopathy: an analysis of 14 deaths occurring in a patient registry. Cancer 1984; 54:1834.

4. Paulli M, et al. Immunophenotypic characterization of the cell infiltration five cases of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Hum Pathol 1992; 23:647.

5. Levine PH, et al. Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Infect Dis 1992; 166:1694.

6. Schee MM, et al. Sinus histiocytosis with massive lymphadenopathy. J Am Acad Dermatol 1997; 37:643.

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