Rosai Dorfman disease

Rosai-Dorfman disease
Classification and external resources
ICD-10	D76.3 (ILDS D76.360)
DiseasesDB	31419

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2] Kiran Singh, M.D. [3]

Synonyms and keywords: Sinus histiocytosis with massive lymphadenopathy, SHML, Destombes-Rosai-Dorfman syndrome, familial Rosai–Dorfman disease, familial SHML, sinus histiocytosis

Overview

Rosai–Dorfman disease is a rare disorder of unknown etiology that is characterized by abundant histiocytes in the lymph nodes throughout the body.

Historical Perspective

This condition has been named after Ronald F. Dorfman and Juan Rosai. The alternative eponym of this condition is known as Destombes-Rosai-Dorfman syndrome, part of which is named after Pierre-Paul Louis Lucien Destombes.

Pathophysiology

Lymphadenopathy of the neck is the most common place of histiocyte accumulation, although accumulation outside of lymph nodes may occur, as well. The most common sites of accumulation outside of the lymph nodes are skin, upper respiratory tract, and the sinuses. In approximately 25% of the situations salivary glands, spleen, liver and eyelids can be involved in the disease process.

Genetics

Rosai–Dorfman disease is inherited in an autosomal recessive pattern.

Microscopic Pathology

Micrograph of a lymph node involved by Rosai-Dorfman disease showing emperipolesis. H&E stain.

Differentiating from other diseases

The histological features of Rosai-Dorfman disease (RDD) resemble those of Faisalabad histiocytosis. Faisalabad histiocytosis can be differentiated from RDD by the presence of joint problems, sensorineural hearing loss and rubbery swellings over the eyelids.

Epidemiology and Demographics

• The incidence of this disease is <10/100 000.
• It is a disease of the childhood.

Natural History, Complications and Prognosis

The symptoms of this disease vary with the site of accumulation similar to other regional tumors. For instance, accumulation in closed spaces such as the cranium can lead to poor outcomes compared to growth in the dermis of an extremity where surgical excision is possible.

Diagnosis

Symptoms

• Fever

Physical Examination

Skin

Extremities

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  Rosai-dorfman disease. Adapted from Dermatology Atlas.

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  Rosai-dorfman disease. Adapted from Dermatology Atlas.

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  Rosai-dorfman disease. Adapted from Dermatology Atlas.

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  Rosai-dorfman disease. Adapted from Dermatology Atlas.

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  Rosai-dorfman disease. Adapted from Dermatology Atlas.

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  Rosai-dorfman disease. Adapted from Dermatology Atlas.

• 

  Rosai-dorfman disease. Adapted from Dermatology Atlas.

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  Rosai-dorfman disease. Adapted from Dermatology Atlas.

• 

  Rosai-dorfman disease. Adapted from Dermatology Atlas.

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  Rosai-dorfman disease. Adapted from Dermatology Atlas.

Head

• Massive cervical lymph node enlargement

Ear

• Sensorineural hearing loss may be present in some patients.

Abdomen

• Hepatosplenomegaly

Laboratory Findings

• Leukocytosis
• Elevated erythrocyte sedimentation rate (ESR)
• Polyclonal hypergammaglobulinemia

References

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