Skin manifestations of relapsing polychondritis (RP) are usually nonspecific.
We report a series of patients with RP who presented with annular skin lesions.
The clinical and histologic features and follow-up data of patients with RP and an annular urticarial eruption were retrospectively reviewed.
Ten patients (9 male, 1 female) (mean age 63.7 years) were included. All patients had tense, fixed,
... [Show full abstract] 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.
Retrospective case series design is a limitation.
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.
