Woman with Nodules on the Cheek and Arm

Evan Farmer, M.D.

Specimen Type:

Skin

History:

The patient is a 41-year-old woman with tender nodules on the left arm and cheek of several months duration. She has no systemic symptoms or other abnormal physical findings. The biopsy was from her left arm.

Pathologic Features:

The specimen has a diffuse nodular dermal infiltrate composed of prominent, large, polygonal, histiocytic cells with fine granular cytoplasms associated with numerous lymphocytes, plasma cells and rare eosinophils (Fig. 1). No neutrophils are identified. The large histiocytic cells are positive for S-100 protein (Fig. 2) and CD68 (Fig. 3). Some of the histiocytic cells contain lymphocytes and plasma cells within the cytoplasms (emperipolesis). Bundles of sclerotic collagen are also present.

Differential Diagnosis:

  • Indeterminate cell histiocytosis
  • Langerhans’ cell histiocytosis
  • Reticulohistiocytoma
  • Rosai-Dorfman Disease
  • Xanthoma

Diagnosis:

Rosai-Dorfman Disease

Rosai-Dorfman Disease is a rare, usually self-limited disease of the skin that may present with or without systemic symptoms including lymphadenopathy. It is characterized by the presence of large histiocytic cells which engulf other inflammatory cells (emperipolesis) and with the histiocytic cells having the following immunoprofile: S-100 protein+, CD1a- and CD68+. The clinical lesions in the skin are usually papules or nodules and may coalesced into larger lesions. The diagnosis is established by the clinical context and a biopsy with the large histiocytes expressing the characteristic immunoprofile. Older lesions tend to develop fibrosis and sclerotic collagen such as the current case.

The lesions tend to spontaneously resolve over several months to years. Multiple therapies to hasten the resolution have been tried with surgical excision seeming to be the most effective. The underlying cause of Rosai-Dorfman Disease is unknown, but there is some suggestion that HHV-6 may be playing a role.

The other entities in the differential diagnosis list can generally be separated by patient context and the following immunoprofile of the histiocytic cells:

Disease S-100 Protein CD1a CD68
Rosai-Dorfman Disease + - +
Indeterminate Cell Histiocytosis + + +
Langerhan's Cell Histiocytosis + + -
Reticulohistiocytoma - - +
Xanthoma - - +

References:

  1. Brenn T, Calonje E, Granter SR, et al. Cutaneous rosai-dorfman disease is a distinct clinical entity. Am J Dermatopathol. Oct 2002;24(5):385-391.
  2. 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. Mar 2007;31(3):341-350.
  3. 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. Feb 2006;154(2):277-286.