(BMJ)—A 14-year-old girl presented with a 6-month hx of a rash on her face and ears. Exam: alopecia; hyperkeratotic scaly lesions on eyebrows; hyperpigmented plaques in ears; painless ulcers on hard palate. Labs: CBC normal; ESR 30 mm/hr; UA normal; ANA positive, 1:160; double-stranded DNA and extractable nuclear antigen negative; complements C3 C4 borderline low. Positive direct Coombs test. What’s the dx?
You are correct. The patient was diagnosed with cutaneous discoid lupus. "Lupus" is the Latin word for "wolf"; it was thought that the cutaneous damage caused by the disease resembled the bite of a wolf. Mucocutaneous manifestations can occur in 60% to 85% of children with juvenile lupus. The typical malar rash is present in 44% to 85% of children with cutaneous disease. Discoid lupus is rare and occurs in <10% of children with skin disease. These lesions are commonly found in the ear lobe, concha, nose, chin, and cheeks but may also be present on the scalp and neck. They initially present as red dry patches that may evolve to hyperpigmented plaques with scale.
Tx includes topical and/or systemic steroids, hydroxychloroquine, and sun protection. Calcineurin inhibitors are used in steroid-sensitive areas such as the face.
The patient was treated with topical steroids, short course of PO steroids, hydroxychloroquine, azathioprine, and sun protection. At 3-month follow-up, there was partial resolution of eyebrow lesions and 0.1% topical tacrolimus was added to her tx. The rashes, oral ulcers, and alopecia had resolved completely at 6-month follow-up.
Archives of Disease in Childhood 2021;106:920
