Papular and annular fixed urticarial eruption: a characteristic skin manifestation in patients with relapsing polychondritis.

BACKGROUND: Skin manifestations of relapsing polychondritis (RP) are usually nonspecific. OBJECTIVE: We report a series of patients with RP who presented with annular skin lesions. METHODS: The clinical and histologic features and follow-up data of patients with RP and an annular urticarial eruption were retrospectively reviewed. RESULTS: Ten patients (9 male, 1 female) (mean age 63.7 years) were included. All patients had tense, fixed, urticarial papules with an annular configuration predominantly located on the upper part of the trunk. Skin lesions occurred before the chondritis in 7 of 10 cases with a mean delay of 23 ± 13 months. Histologic examination consistently showed a lymphocytic vasculitis with no leukocytoclastic vasculitis, even when biopsies were repeated during the evolution (n = 7). Hematologic abnormalities were found in all cases. A myelodysplastic syndrome was found in 4 patients. Oral corticosteroids were effective in all cases, although skin lesions recurred during the decrease of corticosteroid doses in 4 cases. Five patients died during the evolution. LIMITATION: Retrospective case series design is a limitation. CONCLUSION: Annular and papular fixed urticarial eruption may represent a characteristic skin manifestation of RP. It is frequently associated with hematologic abnormalities and may auger a poor prognosis.