Reticulohistiocytosis multicenter D76.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 16.03.2023

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benign reticulohistiocytosis; Giant cell histiocytosis; Histiocytosis giganto-cellularis; lipoid dermato-arthritis; Lipoid dermatoarthritis; Lipoid rheumatism; multicenter reticulohistiocytosis of the skin and synovia; multicentric reticulohistiocytosis; multiple reticulohistiocytomas; Reticulohistiocytomas multiple; Reticulohistiocytosis benign; reticulohistiocytosis disseminata; Reticulohistiocytosis of the skin and synovia multicenter

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Goltz and Laymon, 1954

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Rare, polytopically occurring, systemic non-Langerhans cell histiocytosis affecting the skin, joints, muscles, heart and lungs.

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Unknown. Associations with malignant tumors such as colon carcinomas, breast carcinomas, cervical carcinomas exist in about 28% of cases. Occurring in hypothyroidism, also after injection of X-ray contrast agents. Association with tuberculosis has been observed.

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Occurring in middle age. The ratio of women to men is about 3:1, rarely described in children.

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Occurrence of skin changes, especially periarticular on fingers, back and wrists, face (especially nasal entrance and lips and ears). Frequent (> 50%) involvement of oral mucosa and tongue as well as synovia, bones and, more rarely, internal organs (muscles, heart, lungs).

Clinical features
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Skin lesions: Symmetrically distributed, multiple, pinhead- to pea-sized, mostly dermal-consistent, skin-colored but also copper-brown, sometimes slowly growing, sometimes eruptively exanthematous, solitary, also confluent papules and nodules, possibly with atrophic, possibly also excoriated, mostly smooth surface. Typical (>50% of cases) is a pearl-like involvement of the nail folds of the fingers (coral bead sign). Itching is not infrequently reported. Xanthelasma is present simultaneously in 25% of cases.

Arthropathies: swelling, painful immobilization, mutilating arthritis of the primary chronic polyarthritis type (rheumatoid arthritis), especially of the fingers and metacarpals, also of the vertebral joints and sacroiliac joints.

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In early lesions, cellular inflammatory responses of histiocytes and lymphocytes are seen in the upper and middle dermis. In older lesions, the typical substrate consists of large mononuclear and multinuclear giant cells (see Fig.). PAS-reactive, diastase-resistant, and lipid-soluble substances are present in the cytoplasm.

In the final stage, there is fibrosis.

Granulomatous tissue reactions with accumulations of large, MS-I (high molecular weight protein) positive histiocytes, cytoplasm-rich macrophages, and giant cells with PAS-reactive, lipid-soluble substances are present. Stabilin-1: positive, S100 and CD1a: negative.

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Tumor search and cleanup.

External therapy
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Experiment with intrafocal injection of triamcinolone crystal suspension(e.g. Volon A diluted 1:4 with LA, e.g. mepivacaine).

Internal therapy
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Symptomatic antirheumatic treatment with non-steroidal anti-inflammatory drugs such as diclofenac (e.g. Voltaren) 2 times 50 mg/day. The disease is self-limited in most cases (healing after an average of 8 years).

A few severe courses have been described with severe manifestation of skin symptoms, persistence of the clinical picture and organ involvement. In these cases, immunosuppressive therapy may be necessary. Try glucocorticoids initially 100 mg prednisolone equivalent/day (e.g. Decortin H), slowly tapering off.

Approaches with cytostatics (cyclophosphamide 50 mg/day p.o. or methotrexate up to 25 mg/week i.m. or i.v.) have been described in isolated cases with moderate success. When using cytostatics, a strict indication is necessary, since aggravations of the clinical picture are possible.

Operative therapie
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Circumscribed foci can be excised, if necessary also removed with laser.

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Chronic course (inactive in 8-10 years), possible deformities.

Case report(s)
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Case 1. A 57-year-old female patient complained of itching that had been present for 4 months and the "sudden" appearance of numerous, disseminated, 0.2-0.5 cm large, firm, brown-red, constantly growing papules. These conglomerated in several places (trunk and lower extremities) to form several plaques up to 10.0 cm in size. The skin lesions showed a certain "light accentuation" on the trunk. In addition, the distal and proximal interphalangeal joints of both hands were dominated by coarse, confluent papules and nodules with simultaneous painful joint swelling and movement restrictions. Except for an adenoma of the thyroid gland with euthyroid metabolism there were no secondary diseases.
  • Histology: dermal infiltrate with large polygonal cells characterized by an eosinophilic milky glassy cytoplasm and large clumsy nuclei. These cells expressed CD68 and were 10% positive for the proliferation marker Ki-67, while the infiltrate reacted negatively with S100 and CD1a.
  • Other test results: No evidence of autoimmune diseases and no indication of malignancies. Laboratory: o.B.
  • Therapy: Symptomatic antirheumatic treatment with the non-steroidal anti-inflammatory drug Diclofenac (2 times 50 mg/day). As this therapy was unsuccessful, a follow-up treatment with prednisolone 0.5 mg/kg bw was initiated in descending order and additional hydroxychloroquine 200mg was given. Below this, there was a very slow (not convincing) slight improvement of the findings so far (after 6 months).

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  13. Utikal J et al (2003) Cutaneous non-Langerhans' cell histiocytoses. J Dtsch Dermatol Ges 1: 471-491.
  14. Yasar S et al (2011) Xanthomas at the nail fold: reevaluation of the coral bead sign. JDDG 9: 387-388.


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Last updated on: 16.03.2023