The Journal of Dermatology | 2021
Generalized pruritic erythema with neutrophils in a patient with relapsing polychondritis
Abstract
Dear Editor, An 84yearold Japanese man presented with a 3month history of generalized rash, swelling of the ears, fever and cough. He had noticed recurrent episodes of pruritic erythema, general malaise and fever (37– 38.4°C) from around 1 year before this admission to our department. Physical examinations showed reddish, swollen and tender auricles (Figure 1a). His eyes showed conjunctival and/ or ciliary hyperemia (Figure 1b). In addition, widespread, irregularly shaped patches of itchy erythema leaving brown pigmentation were observed on the trunk and extremities (Figure 1c,d). No arthralgia was noted. Histopathological examinations of the auricle revealed dense cellular infiltrates comprising lymphocytes and a small number of neutrophils surrounding the auricular cartilages (Figure 1e). Itchy erythema showed diffuse inflammatory cell infiltration with lymphocytes and neutrophils in the upper dermis (Figure 1f,g). Laboratory findings were: red blood cell count, 3.14 × 106/μl; white blood cell coutn, 9800/μl (neutrophils, 77.1%; lymphocytes, 15.9%; eosinophils, 2.6%; monocytes, 4.0%); platelets, 26.5 × 104/μl; and Creactive protein, 7.4 mg/dl. Antitype II collagen antibody was weakly positive (26.5 EU/ml; normal, <25). Ophthalmic examination revealed bilateral uveitis. Computed tomography of the lungs showed atelectasis of the right S10 segment. No disturbances of auditory or vestibular functions were observed. The patient was diagnosed with relapsing polychondritis (RP) and treatment was initiated with oral prednisolone (0.5 mg/kg/day), leading to improvements in systemic, cutaneous, ocular and respiratory symptoms. Approximately 35% of RP patients show dermatological symptoms, and skin symptoms in some patients precede polychondritis by anywhere from 10 days to 20 years.1 Dermatological manifestations include aphthosis, purpura with or without livedo, erythema nodosumlike lesions, Sweet’s syndrome, superficial phlebitis and pyoderma gangrenosum.1– 3 Neutrophil activation status may thus be involved in the development of some of the skin symptoms of RP. Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome,