IgG4-Related Skin Disease Successfully Treated by Thalidomide: A Report of 2 Cases With Emphasis on Pathological Aspects

• Autores: Saskia Ingen-Housz-Oro, Nicolas Ortonne, Muriel Elhai
• Localización: JAMA Dermatology, ISSN 2168-6068, Vol. 149, Nº. 6, 2013, págs. 742-747
• Idioma: inglés
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• Resumen

  • Importance In IgG4-related disease (IgG4-RD), skin involvement is rare and associated especially with systemic disease. We report 2 cases of isolated skin IgG4-RD successfully treated with thalidomide and investigated their phenotypic characteristics.

    Observations Two men had cephalic nodules. Skin biopsies revealed dense lymphocytic infiltrates with numerous plasma cells and fibrosis. IgG4-RD was confirmed by very high IgG4+ to IgG+ plasma cells ratios of 76% (patient 1) and 100% (patient 2). The serum IgG4 level was normal. There was no other organ involvement. Thalidomide therapy was introduced. After 6 months, lesions were in remission. Patient 1 required long-term, low-dose thalidomide, whereas patient 2 stopped treatment and showed no relapse. Immunostaining revealed numerous FoxP3+ cells in the interfollicular areas, which decreased with treatment in patient 2, and numerous follicular helper T lymphocytes (TFH) within the follicular germinal centers. There were numerous mast cells; some stained for interleukin (IL)-6, and expression of phospho-Smad2/3 was demonstrated.