Which skin lesion findings are characteristic of relapsing polychondritis (RP)?

Updated: Dec 06, 2018
  • Author: Nicholas Compton, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Skin lesions are found in 17%-39% of patients with relapsing polychondritis. Specific lesions are limited to erythema and edema overlying the inflamed cartilaginous structures. See the image below.

Severe auricular edema and inflammation. Courtesy Severe auricular edema and inflammation. Courtesy of the University of Washington, Division of Dermatology.

Various nonspecific skin lesions have been reported, as follows:

  • Aphthous ulcers are the most common.

  • Limb nodules, purpura, papules, sterile pustules, superficial phlebitis, livedo reticularis, limb ulceration, and distal necrosis have been reported.

  • Rarer findings include Sweet syndrome, urticarial vasculitis, and Kaposi sarcoma.

  • Some findings likely represent the skin manifestations of the many conditions associated with relapsing polychondritis rather than specific manifestations of relapsing polychondritis itself.

  • Cutaneous vasculitis: The prevalence of biopsy-proven cutaneous (small vessel) leukocytoclastic vasculitis is approximately 10%, while the prevalence of systemic (including skin) medium-to-large vessel vasculitis ranges from 11%-56%. It may appear as in its typical form of palpable purpura or as hemorrhagic bullae, typically on the lower extremities or other dependent areas.

  • Erythema elevatum diutinum: This has been described in two patients with relapsing polychondritis. [32, 33]

  • Cutaneous polyarteritis nodosa: A patient with relapsing polychondritis presented with relapsing painful red nodules from 1-3 cm in size, occurring on the entire skin and accompanied by arthralgias and myalgias.

Other cutaneous lesions reported in patients with relapsing polychondritis and vasculitis included the following:

  • Palpable purpura

  • Acute febrile neutrophilic dermatosis (Sweet syndrome) [34, 35]

  • Subcutaneous inflammatory nodules resembling erythema nodosum

  • Localized ulcerating neutrophilic conditions resembling pustules, furuncles, abscesses, and ulcerating abscesses

  • Panniculitis: This is characterized by 5- to 10-cm tender erythematous nodules showing septal and lobular inflammation. [36]


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