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ParaneoplasticHistiocytosisRareICD-10 D76.3 / D76.1

Multicentric reticulohistiocytosis

MRH · lipoid dermatoarthritis · giant-cell reticulohistiocytoma syndrome

Multicentric reticulohistiocytosis (MRH) is a rare non-Langerhans systemic histiocytosis with cutaneous, articular and systemic involvement. The skin shows skin-coloured to red-brown papulonodules in a characteristic coral-bead pattern around the nail folds. A destructive symmetric erosive polyarthritis frequently progresses to arthritis mutilans. Approximately 25-30% of cases are paraneoplastic; the most common associated malignancies are breast, colorectal, gastric and lung carcinomas.

CurrentLast reviewed 16 May 2026

Epidemiology

  • Adults >40 years; female:male ~3:1.
  • Approximately 25-30% have an underlying malignancy at presentation or develop one within several years.
  • ~15-20% have an underlying autoimmune disorder (rheumatoid arthritis, SLE, Sjögren, scleroderma).

Clinical features

  • Cutaneous: 2-10 mm skin-coloured to yellow-brown / pink papulonodules; predilection for hands (especially periungual — "coral-bead" sign), face, scalp, ears, mucosae. Cobblestone perinasal nodules ("string of pearls").
  • Articular: destructive symmetric polyarthritis affecting DIP and PIP joints; can progress rapidly to arthritis mutilans (opera-glass hand).
  • Mucosal: lips, tongue, oropharynx, nasal mucosa, conjunctiva.
  • Systemic: fever, weight loss, fatigue; rare cardiac, pulmonary, GI, endocrine involvement.
  • Associated xanthelasma in up to a third.

Histopathology

  • Diffuse dermal infiltrate of mononuclear and multinucleated giant histiocytes with eosinophilic ground-glass cytoplasm.
  • IHC: CD68+, CD163+, S100−, CD1a−, Langerin (CD207)−. PAS-positive cytoplasm.
  • Distinguishes from Langerhans-cell histiocytosis, xanthogranuloma family, reticulohistiocytoma solitarium.

Workup for paraneoplastic causes

  • Full malignancy screen at diagnosis: history, examination, FBC, U&E, LFT, ESR, CRP, urinalysis.
  • Age-appropriate cancer screening: mammography, cervical screening, colonoscopy / FIT, gastroscopy if symptoms.
  • CT chest / abdomen / pelvis in patients ≥40; PET-CT if extracutaneous features.
  • Autoimmune screen — ANA, RF, anti-CCP, anti-Ro / La, complement.
  • Joint imaging — radiographs and / or MRI — for documentation and progression monitoring.

Management

  • Treat underlying malignancy if present — cutaneous and articular features often regress.
  • Methotrexate (first-line steroid-sparing), prednisolone, cyclophosphamide, leflunomide, hydroxychloroquine.
  • Increasing use of TNF inhibitors (etanercept, adalimumab, infliximab), IL-6 inhibitors (tocilizumab), bisphosphonates for joint preservation.
  • Hand-therapy / occupational therapy input for arthritis mutilans risk.
  • Long-term cancer surveillance even after remission.

References

  1. West KL et al. Multicentric reticulohistiocytosis: a multicenter case series and review of literature. J Clin Rheumatol. 2017;23:67-72.
  2. Trotta F et al. Multicentric reticulohistiocytosis. Best Pract Res Clin Rheumatol. 2004;18:759-772.
  3. Rapini RP, Warner NB. Relapsing multicentric reticulohistiocytosis. J Am Acad Dermatol. 2006;55:712-714.
  4. Goto H et al. A case of multicentric reticulohistiocytosis associated with breast cancer. J Dermatol. 2020;47:e84-e85.

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