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[Multicenter histiocytosis]

A M Schmitt1, P Grolleau-Rochiccioli, G Laplanche

  • 1Service de Dermatologie, CHR Purpan, Toulouse.

Annales De Dermatologie Et De Venereologie
|January 1, 1994
PubMed
Summary
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Multicentric histiocytosis, a rare systemic disease, was effectively treated in a 38-year-old man using alkylating agents. This case highlights successful management despite cyclophosphamide-induced hepatitis, showing the benefits of immunosuppressors.

Area of Science:

  • Rheumatology
  • Dermatology
  • Oncology

Background:

  • Multicentric histiocytosis is a rare systemic disorder characterized by active phases and remissions.
  • Alkylating agents are established immunosuppressors for managing multicentric histiocytosis.

Observation:

  • A 38-year-old male presented with multicentric histiocytosis, initially manifesting as spontaneous hemarthrosis, mediastinal lymphadenopathy, and pruritus preceding skin lesions.
  • Treatment with cyclophosphamide (100 mg/day) was initiated but discontinued due to drug-induced hepatitis.
  • Subsequent treatment with chlorambucil (0.1 mg/kg/day for 6 months) resulted in no relapse after 14 months of follow-up.

Findings:

  • This case underscores the systemic nature of multicentric histiocytosis.
  • Successful management was achieved with alkylating agents, specifically chlorambucil, following an adverse reaction to cyclophosphamide.

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  • The presentation included unusual initial symptoms such as spontaneous hemarthrosis and mediastinal lymph nodes.

    • Implications:

    • Alkylating agents remain a beneficial therapeutic option for multicentric histiocytosis.
    • Physicians should be vigilant for potential adverse effects like drug-induced hepatitis during immunosuppressive therapy.
    • This case expands understanding of the diverse clinical presentations of multicentric histiocytosis.