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Related Experiment Videos

[Multiple cutaneous reticulohistiocytosis].

F Loche1, F Lucas, P Bayle-Lebey

  • 1Service de Dermatologie, CHU Purpan, place du Dr-Baylac, 31059 Toulouse Cedex.

Annales De Dermatologie Et De Venereologie
|June 23, 2000
PubMed
Summary

This case presents a rare instance of multicentric reticulohistiocytosis (MR) with extensive, plaque-like skin lesions. Early treatment with corticosteroids and cyclophosphamide proved effective in managing this unique presentation of MR.

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Area of Science:

  • Dermatology
  • Rheumatology
  • Histiocytosis

Background:

  • Multicentric reticulohistiocytosis (MR) is a rare non-Langerhans cell histiocytosis.
  • Characterized by skin papules/nodules and destructive polyarthritis, with potential multisystem involvement.
  • Diffuse cutaneous involvement, particularly plaque-type lesions, is exceptionally rare.

Observation:

  • A 65-year-old woman presented with papulonodular lesions and destructive hand arthritis.
  • Histological analysis confirmed MR; initial investigations for visceral disease or neoplasia were negative.
  • Rapid progression to diffuse, infiltrated cutaneous plaques on trunk, face, and limbs occurred.

Findings:

  • The case is the first reported instance of MR presenting with diffuse, plaque-like cutaneous lesions.

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  • Histological findings were consistent across all lesions, including the plaques.
  • Treatment with corticosteroids and cyclophosphamide resulted in successful disease management.

    • Implications:

    • Diffuse cutaneous lesions in MR may be associated with a higher incidence of visceral involvement.
    • The potential association between MR and neoplasia (up to 25%) warrants consideration.
    • Standard treatment involves initial corticosteroids combined with alkylating agents or methotrexate.