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

Relapsing polychondritis, an underestimated dermatological urgency: case report and literature review

“…In all groups, the most frequent manifestations were purpura and nodules (41%), followed by urticarial papules (32%), phlebitis (27%), and livedo reticularis (23%). In global terms, cutaneous manifestations were more related to MDS and vasculitis . In our patient, skin manifestations were not found (See Table ) …”

“…In global terms, cutaneous manifestations were more related to MDS and vasculitis. 5,12,14,24,26,28,29,36 In our patient, skin manifestations were not found (See Table 2). 1-3,14,33-40 The histological findings help to guide the diagnosis in nonclassic cases.…”

“…Relapsing polychondritis (RPC) is an autoimmune multisystemic disease with recurrent inflammation of three types of cartilage: hyaline, elastic, and fibrous. However, it possibly involves another system, such as the ear, nose, airway, eyes, blood vessels, heart, and skin . RPC was first described by Wartenhorst in 1923, but Pearson and colleagues, in 1960, described its recurrence.…”

Hypocomplementemic urticarial vasculitis occurring in a patient with relapsing polychondritis

“…Forty percent of patients with RP develop dermatological manifestations, oral aphthosis being the most frequent . Recently, annular urticarial lesions affecting the upper part of the trunk, with no angioedema, and showing lymphocytic vasculitis, have been characterized as specific to RP . Our patient, however, had urticarial lesions mainly located in the upper and lower limbs, demonstrating leukocytoclastic vasculitis, associated with angioedema and related to HUV.…”

“…Corticosteroids improve both RP and HUV flares and decrease their frequency . Despite the good response of HUV to hydroxychloroquine, its efficiency on RP is uncertain . The use of immunosuppressants and biological agents should be restricted to severe or relapsing cases …”