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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
    • 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
  • Sj√∂gren Syndrome
  • Biliary cirrhosis
  • Pregnancy

Prognosis

  • Rarely leads to death unless there is an associated malignancy

multicentric reticulohistiocytosis

Multicentric Reticulohistiocytosis

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