Multicentric reticulohistiocytosis

A rare histiocytic proliferative disorder, first described in 1954 by Goltz and Laymon, in which joints, skin and mucous membranes are affected. Other organs may be involved and 20% of patients have an internal malignancy.

This chapter is set out as follows:

• Women are affected more than men
• Tends to affect middle-aged individuals
• Joint involvement usually preceded skin and mucosal involvement

• Distribution
• Extensor surfaces, especially of the hands and forearms
• The face, ears and scalp are often affected
• Involvement of the lower trunk and legs is rare

• Morphology
• Non-tender, firm, brown-yellow papules
• Lesions vary in size from 1-10 mm and can coalesce to form plaques with a cobblestone surface. This may give a characteristic “coral-bead” appearance, which is considered pathognomonic. The nodules grow slowly, and rarely ulcerate
• Nail involvement can lead to dystrophic change
• Lesions on the ears and nose may cause destruction of cartilage resulting in a disfigured appearance

• Other clinical features
• Joint involvement is characterised by a destructive and deforming symmetrical polyarthritis with a predilection for the distal interphalangeal joints
• 50% have mucosal involvement, especially the lips and tongue
• 30% have lipid abnormalities
• 25% have pruritus
• Internal organs can be affected

• Internal malignancy
  • Affects 20% of patients
  • The most common tumours are gastric, ovarian, breast and uterine carcinomas, myeloma, melanoma and lymphoma

• Because up to 20% of cases may stem from an underlying malignancy, evaluation for a possible malignancy is essential. Thorough history taking and physical examination with age-appropriate cancer screening is recommended

• If there is no systemic malignancy the disease will often remit within 7-8 years, however, by this time considerable joint destruction may have occurred

• Management is in Secondary Care, although no treatment appears to be of consistent value