Facial necrotic ulcerative lesions in an adolescent female with granulomatosis with polyangiitis (GPA)

Excerpt

A 15-year-old female was referred with forehead lesions starting 3 months before, initially treated as acne. She was also suffering from recurrent otitis media, treated with 3 courses of antibiotics over the last 45 days. Four weeks ago, she was admitted to another institution with symptoms of arthralgias, myalgias, and low-grade fever up to 37.8 °C. Her physical exam revealed ulcerative lesions of the forehead (Fig. 1) and pre-auricular area bilaterally, muscle tenderness, hemorrhagic lesions of the nasal mucosa, aphthous ulcers of the buccal mucosa, gum hemorrhagic lesions, right side perforated tympanic membrane, and otorrhea. No sinusitis, rhinitis, or chronic cough was ever reported. Blood pressure was 115/78 mmHg. Chest X-ray revealed nodular lesions and CT chest confirmed the presence of 2 large nodular lesions up to 1.5 cm of diameter as well as multiple smaller nodules (Fig. 2). The paranasal sinuses imaging revealed mastoid cells, sphenoid sinus, and the ethmoid cells filled with secretions, with demineralization of the septations of the latter. Forehead skin lesion biopsy revealed ulcerative lesions with fibroinflammatory exudate and granulomatous reaction of the dermis, but no lesions suggestive of vasculitis. Blood testing revealed WBC 11360/μL (Ne 65.5%, Ly 23%, Mo 8.1%, Eo 3.3%), Hb 11.3 g/dl, PLT 374 K/μL, CRP 91.8 mg/L, ESR 140 mm/h, Ur 24 mg/dL, Cr 0.5 mg/dL, AST 10 U/L, ALT 7 U/L, and CPK 23 U/L. Urine protein/creatinine ratio was < 0.2, and urinalysis was negative for hematuria. ANCA indirect immunofluorescence was positive (c-ANCA) with PR-3 specificity (300 U/mL) by ELISA. The diagnosis of granulomatosis with polyangiitis (GPA) was established and she was treated with IV methylprednisolone pulses (1 g × 3 days) followed by 60 mg/day with a tapering dose and rituximab 500 mg × 4 doses.

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