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
  • Multiple nodules
• 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

• Dermatomyositis
• Gout
  • Asymmetric
• Soft tissue nodules do not calcify
• Leprosy
• Lymphoma
• Rheumatoid arthritis
  • Does not frequently involve DIP joints
• Features Periarticular demineralization
• Sarcoidosis
  

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%
  • Melanoma
• Sarcomas
• Leukemia
• Lymphoma
• Carcinomas (most often adenocarcinomas) of
• Breast
• Ovary
• Cervix
• Stomach
• Colon
• Bronchus
• Endocrinopathies
• Diabetes
• Hypothyroidism
• Rheumatoid arthritis
• Lupus
• Sjogren 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