PMC5192348_01
Erythema on the cheeks and ears at initial onset (4 years old) (A) and at relapse (12 years old) (C). Keratotic papules and purplish erythema on the dorsal of the left hand at initial onset (B) and at relapse (D).
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Physical examination found erythema on the cheeks and ears, keratotic papules and purplish erythema on the dorsa of the hands, and scaly erythema on the knees (Fig 1, A and B). At 12 years of age, the patient presented to us with similar symptoms affecting the skin and proximal muscles but without preceding infectious episodes within the previous 3 months (Fig 1, C and D).
Vacuolar changes at the dermoepidermal junction of the epidermis, and deposition of mucin, pigment incontinence, and infiltration of lymphocytes in the papillary dermis are observed in the biopsy specimen of the left cheek at initial onset (4 years old) (A) and of the right knee at relapse (12 years old) (C). At the initial onset (T2) (B) (orange arrows) and at relapse (STIR) (D) (yellow arrows), magnetic resonance imaging shows high-intensity areas in the proximal muscles of the thighs, which suggests edema caused by inflammation. (C, Hematoxylin-eosin stain; original magnification: x200.)
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The histopathology of the left knee showed vacuolar changes in the epidermis, deposition of mucin, pigment incontinence, and infiltration of lymphocytes in the papillary dermis (Fig 2, A). The clinical, histopathologic, and radiologic findings were virtually identical to those observed 8 years before (Fig 2, C and D).