Multicentre reticulohistiocytosis. Saba R, Case Rep Rheumatol (2013). Open

Definition

Reticulohistiocytosis is a rare form of histiocytosis. The symptoms vary from some skin changes (limited form) to severe disease in many organs (multi-centric type).

Limited reticulo-histiocytosis

Only the skin is attacked

• Synonyms: "solitary reticulohistiocytosis" or "solitary histicytoma"
• Surgical treatment is most often curative

Multicentre reticulohistiocytosis

Synonym: "lipoid dermatoarthritis", "giant cell reticulo-histocytosis" which is an aggressive disease. Adults around the age of 50X often become ill. Only a few hundred cases are described.

• 50% starts with Arthritis (Joint inflammation), often in the fingers, knees and shoulders. Often destructive (mutilating)
• Knots (nodules) are present in the skin and under the skin and can clump into larger masses, most commonly on the upper body including the face
  • Tissue sample (biopsy). This shows reticulohistiocytic granulomas with mono- and multinuclear giant cells with PAS positive cytoplasm. CD68 positive cells.
  • Radiological surveys that can show joint damage as with mutilating (destructive) arthritis
  • The cause of the disease is unknown. Some (about 25%) turn out to have underlying cancer, while others may have autoimmune disease such as Vasculitis or connective tissue diseases

Differential diagnosis

• Dermatomyositis
• Farber disease
• Leprosy
• Lymphoma
• Psoriasis arthritis, merratic type
• Rheumatoid arthritis (RA)
• Sarcoidosis
• Tofous uric acid
• Urbach-Wiethe disease
• Xanthogranuloma

Treatment

Immunosuppressants like Prednisone, Methotrexate, Azathioprine or TNF inhibitors (Biological drug)

Prognosis

Spontaneous recovery may occur, but relapse occurs

Literature

• Tariq S, 2016

Histiocytosis, BINDEVESSYKDOMMER.no

• Bindevevssykdommer.no
• Vaskulitt.no