[Cutaneous vasculitis, myelodysplasia and relapsing polychondritis].
F. Nemeth-Normand, L. Machet, L Vaillant, V. Fontes, Thierry Lefrancq, G Lorette
Abstract
F. Nemeth-Normand, L. Machet, L Vaillant, V. Fontes, Thierry Lefrancq, G Lorette
Abstract
BACKGROUND: Relapsing polychondritis is a rare autoimmune disease, characterized by recurrent inflammation of cartilaginous tissues. In some cases, many other tissues can be involved. CASE REPORT: We describe the case of a 64 year-old man with relapsing polychondritis, whose first symptoms were papulonodular and mucosal aphthous lesions. The skin biopsy revealed vasculitis without leukocytoclasic features. The diagnostic of relapsing polychondritis was made only two years later, thanks to recurrent auricular chondritis. Since the beginning, the disease was associated with a myelodysplasia. DISCUSSION: Dermatological manifestations are noticed in less than 50 p. 100 of cases during the evolution of relapsing polychondritis. An association with a myelodysplasia has already been reported, and it could be a particular form of relapsing polychondritis. Myelodysplasic syndromes are more frequently associated with relapsing polychondritis than with other vasculitis. It must be sought especially when cutaneous and mucosal manifestations exist.
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BACKGROUND: Relapsing polychondritis is a rare autoimmune disease, characterized by recurrent inflammation of cartilaginous tissues. In some cases, many other tissues can be involved. CASE REPORT: We describe the case of a 64 year-old man with relapsing polychondritis, whose first symptoms were papulonodular and mucosal aphthous lesions. The skin biopsy revealed vasculitis without leukocytoclasic features. The diagnostic of relapsing polychondritis was made only two years later, thanks to recurrent auricular chondritis. Since the beginning, the disease was associated with a myelodysplasia. DISCUSSION: Dermatological manifestations are noticed in less than 50 p. 100 of cases during the evolution of relapsing polychondritis. An association with a myelodysplasia has already been reported, and it could be a particular form of relapsing polychondritis. Myelodysplasic syndromes are more frequently associated with relapsing polychondritis than with other vasculitis. It must be sought especially when cutaneous and mucosal manifestations exist.
Key concepts: Relapsing polychondritis, Chondritis, Medicine, Chondropathy, Vasculitis, Dermatology, Pathology, Systemic disease