reticulohistiocytosis 4.25/5 (4)

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

Definition

Reticulohistiocytosis is a rare form of histiocytosis (non-Langerhan's histiocytosis). The symptoms vary from individual skin changes (limited form) to serious disease in many organs (multicentric type). The disease was described in 1937 by Weber and Freudenthal in 1937 (Weber FP, 1937). The condition, which most often attacks women aged 50-60, is very rare, but can be confused with Rheumatoid arthritis . Limited and multicentric (widespread) forms exist.

Limited reticulohistiocytosis

Only the skin is affected. Synonyms: "solitary reticulohistiocytosis" or "solitary histicytoma". Surgical treatment is usually 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.

Disease Cause is unknown. Some (approx. 25%) turn out to have an underlying cancer, while others may have an autoimmune disease such as Vasculitis or connective tissue diseases.

Symptoms. Joint pain for up to a few years is common before approximately 50% start with joint inflammation (Arthritis), often in fingers, knees and shoulders.

Skin abnormalities with small knots in pearl string formation can be seen on fingers. Fat (lipids) under the skin (xanthomas) is seen in 30%.

Clinical signs of damage to the joints are detected (destructive / mutilating course). Nodules (noduli) present in the skin and under the skin and can clump together into larger masses, most commonly on the upper body including the face.

Blood tests may be inconspicuous. "Rheuma tests" such as anti-CCP are normal.

Imaging can show joint damage as in destructive arthritis

Tissue sample (biopsy). This is important for the diagnosis and shows reticulohistiocytic granulomas with mono- and multinuclear giant cells with PAS positive cytoplasm. CD68 positive cells.

Similar diseases, differential diagnoses

Treatment

Immunosuppressants like Prednisone, Methotrexate, Azathioprine or TNF inhibitors (Biological drug) (reference; Tariq S, 2016).

Prognosis

Spontaneous recovery may occur, but relapses also occur.

Literature


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