Background. Relapsing polychondritis is a rare systemic disease. Skin invol
vement occurs in 20 to 50 % of cases. Cutaneous signs are most often relate
d to a leukocytoclastic vasculitis. Association of relapsing polychondritis
with neutrophilic dermatosis have also been reported. We report the first
case of an erythema annulare centrifugum-like dermatosis associated with re
lapsing polychondritis, with a two years delay between both conditions.
Case report. A 74 year-old man was seen for papulo-erythematous centrifugal
annular lesions that appeared 18 months earlier in a context of bad genera
l conditions. Biological tests were normal. Several skin biopsies were perf
ormed, showing at the beginning features of drug reaction and then of lupus
-lichen. Treatment with hydroxychloroquine, topical corticosteroids, dapson
e and thalidomide were unsuccessful. In the following months, the patient d
eveloped fever and relapsing bronchitis. Suddenly, a chondritis of the ears
appeared, leading to the diagnosis of relapsing polychondritis. All the cu
taneous, chondritic and respiratory signs disappeared with oral steroid the
rapy. Two years after the diagnosis of relapsing polychondritis the patient
developed refractory anemia.
Discussion; Cutaneous signs of relapsing polychondritis are frequent and ma
y occur several months or years before the chondritis. They are polymorphou
s, but to the best of our knowledge, a clinical aspect of erythema annulare
centrifugum has never been described. Our observation recalls the sometime
s long delay between the cutaneous and the chondritic signs of relapsing po
lychondritis and the high frequency of dysmyelopoiesis in relapsing polycho
ndritis with cutaneous involvement.