Multicentre reticulohistiocytosis. Saba R, Case Rep Rheumatol (2013). Open
Contents
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 eller 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
Histiocytosis, BINDEVESSYKDOMMER.no
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