Cutaneous Rosai-Dorfman disease
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Synopsis
The median age of onset for CRDD is 40 years, and it displays a 2:1 predilection for females. Individuals of Asian and Northern European descent are also more commonly affected.
Patients with CRDD generally appear well and lack constitutional symptoms. They may have minor cutaneous symptoms (pain, ulceration, tenderness) associated with localized growth but are generally asymptomatic. The etiology is thought to be an exaggerated immunologic reaction triggered by infectious or autoimmune stimuli. CRDD has been described in the presence of human herpesvirus 6, Epstein-Barr virus, parvovirus B19, and human immunodeficiency virus (HIV), as well as post-vaccination. Polyclonal infiltration further supports the notion of a reactive versus a neoplastic process.
CRDD is a benign and self-limited disease. In case series and reviews, the disease does not progress to systemic involvement. The time to resolution is variable. In a review of 65 cases occurring on the face, 37.5% resolved spontaneously after an average 10-month observation period.
Codes
ICD10CM:D76.3 – Other histiocytosis syndromes
SNOMEDCT:
402883006 – Non-Langerhans cell histiocytic dermatosis
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Differential Diagnosis & Pitfalls
- Langerhans cell histiocytosis – histiocytes will stain positive for CD1a and have Birbeck granules on electron microscopy
- Non-Langerhans cell histiocytosis:
- Juvenile xanthogranuloma – a single or few lesions, pediatric patients
- Benign cephalic histiocytosis – multiple lesions, pediatric patients
- Generalized eruptive histiocytosis – multiple lesions, adult patients, self-limited
- Necrobiotic xanthogranuloma – adult patients, multisystem disease, ophthalmologic complications, hepatosplenomegaly, leukopenia, increased erythrocyte sedimentation rate (ESR), associated with immunoglobulin G (IgG) monoclonal gammopathy
- Reticulohistiocytosis – multicentric and solitary, adult patients, can be associated with destructive arthritis, increased ESR, fever, anemia, solid organ malignancy
- Systemic Rosai-Dorfman disease – bilateral cervical lymphadenopathy, constitutional symptoms, increased ESR, polyclonal hypergammaglobulinemia
- Xanthoma disseminatum – cutaneous xanthomas, mucosal xanthomas, diabetes insipidus
- Sarcoidosis
- Granuloma annulare
- Pityriasis lichenoides et varioliformis acuta (PLEVA)
- Pityriasis lichenoides chronica (PLC)
- Leukemia cutis
- Cutaneous lymphomas
- Kaposi sarcoma
- Dermatofibrosarcoma protuberans
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References
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Last Reviewed:08/13/2019
Last Updated:09/02/2019
Last Updated:09/02/2019
Cutaneous Rosai-Dorfman disease