[Necrobiotic xanthogranuloma without monoclonal gammopathy and with a rapidly fatal outcome].

Abstract

BACKGROUND:

Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years.

CASE REPORT:

A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset ofcorticosteroid dependency, iatrogenic Cushing's syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient.

DISCUSSION:

Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.

• Authors: A Bernez, M Abdallah-Lotf, M D'Incan, A De Muret, P Souteyrand, G Lorette, L Machet
• Journal: Annales de dermatologie et de venereologie (Ann Dermatol Venereol) Vol. 133 Issue 3 Pg. 246-9 (Mar 2006) ISSN: 0151-9638 [Print] France
• Vernacular Title: Xanthogranulome nécrobiotique sans gammapathie monoclonale d'évolution rapidement fatale.
• PMID: 16800175 (Publication Type: Case Reports, English Abstract, Journal Article)

Topics

• Aged
• Fatal Outcome
• Granuloma (diagnosis)
• Humans
• Male
• Necrobiotic Disorders (diagnosis)
• Shock, Septic (etiology)
• Ulcer (etiology)
• Xanthomatosis (diagnosis)