Annular eruption preceding relapsing polychondritis: case report and review of the literature

Abstract

Relapsing polychondritis (RP) is a rare multisystem disorder affecting cartilaginous tissues, primarily of the ears, nose, larynx, and trachea. RP shows dermatologic manifestations in 35-50% of cases, and may show skin findings as the first sign of disease in 12% of affected patients. There have been reports of urticarial-like lesions in RP, typically associated with vasculitis, as well as a few reports of erythema multiforme-like lesions. There has also been one previous report of erythema annulare centrifugum associated with RP, presenting with a histology suggestive of a drug eruption. Our patient presented with urticarial and erythema annulare centrifugum-like plaques preceding the diagnosis of RP by over 1 year, with an initial histology suggestive of a drug eruption.

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

“…Two cases of patients with RP and annular urticarial eruption preceding chondritis have been reported in the literature. 6,7 Interestingly, their skin lesions were very similar to those of patients from the J AM ACAD DERMATOL current study. These two male patients presented with annular and urticarial lesions of the trunk, which occurred 18 and 30 months before the onset of chondritis, respectively (Table II).…”

“…Histologic analysis showed a dermal perivascular infiltrate of small lymphocytes with endothelial swelling, sparse keratinocyte necrosis, and a negative DIF finding, suggestive of a drug reaction. These two patients had an anemia and one had a RP-associated myelodysplasia 6,7 (Table II). The clinical and histologic features described in the current series and in these two case reports are completely different from the infiltrated purpura corresponding to a leukocytoclastic vasculitis, which has been previously reported in patients with RP.…”

“…Interestingly, corticosteroid and/or thalidomide were also reported to be effective in the two cases of RP-associated annular urticarial eruption reported in the literature. 6,7 Overall, these annular urticarial eruptions seem to represent a unique and early skin manifestation in patients with RP. Their particular clinical features allowed us to suspect the diagnosis of RP before the occurrence of chondritis in the last 3 patients in this series.…”

Laryngotracheal Reconstruction at Relapsing Polychondritis

“…7 Because of destruction of cartilaginous structures, respiratory problems occur in RP. Meanwhile, necrosis and fibrosis lead to a narrowing tracheal ring or destruction of the trachea.…”

Relapsing Polychondritis: Systemic and Ocular Manifestations, Differential Diagnosis, Management, and Prognosis

“…20,21 The type II collagen antibodies are not specific to RP, as they are also present in rheumatoid arthritis, ankylosing spondylitis, systemic lupus erythematosus, and psoriatic arthritis. 22,23 It is thought that RP bears many of the hallmarks of a TNF-α-mediated disease. T-cell clones reactive to type II collagen have been identified, suggesting a Th1-type autoimmune response, producing TNF-α.…”