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Multicentric Reticulohistiocytosis (MRH)
General Considerations
- Rare disease with proliferation of histiocytes characterized by skin lesions and arthritis
- Erosive symmetrical polyarthritis
- Multiple cutaneous nodules
- Primarily middle-aged adult women with a mean age of 43
Clinical Findings
- Inflammatory joint disease
- Skin lesions
- Most often in hands, especially at base of nail
- Grow slowly and may ulcerate
- Weight loss
- Fever
Imaging Findings
- Hand
- Predilection for DIP joints
- Symmetrical well-circumscribed marginal erosions at joint surface
- Lesions may resemble gout
- Uncalcified soft tissue nodules
- Mild osteopenia
- Marked resorption of subchondral bone with foreshortening of fingers
- Arthritis mutilans
Differential Diagnosis
Treatment
- No effective treatment
- Steroids, antimalarials, aledronate have been used
Complications
- Deformities of the hands
- Malignancies with no particular predilection for site or type in as many as 25%
- Sarcomas
- Leukemia
- Lymphoma
- Carcinomas (most often adenocarcinomas) of
- Breast
- Ovary
- Cervix
- Stomach
- Colon
- Bronchus
- Endocrinopathies
- Diabetes
- Hypothyroidism
- Rheumatoid arthritis
- Lupus
- Sjögren Syndrome
- Biliary cirrhosis
- Pregnancy
Prognosis
- Rarely leads to death unless there is an associated malignancy
Multicentric histiocytosis. Characteristic findings of a rare disease. Note the lack of osteoporosis, the marked destruction of not only the carpals but of all the interphalangeal joints, the non-calcified soft tissue masses (white arrows) and the symmetrical distribution in both hands.
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Multicentric reticulohistiocytosis eMedicine Callen, JP, MD
Gentili.net Multicentric reticulohistiocytosis
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