Pyoderma Gangrenosum Initially Presenting as an Ulceration of the Ear Lobule

Pyoderma Gangrenosum (PG) was first described in 1916 as “phagedenisme geometrique”, after a French dermatologist observed rapidly progressing, cutaneous necrotic lesions with sharp borders.1 In 1930, Brunsting and his colleagues at the Mayo Clinic coined the term Pyoderma Gangrenosum, because it was initially thought to arise from staphylococcal and streptococcal infections which were observed in 5 of their patients.2 The exact etiology and pathogenesis is still unknown. To date, only a few cases of PG have been shown to affect the ears, all showing no gender or age predilection.3 We report another such case.   CASE REPORT A three-year-old girl presented at the emergency room with a non-healing, erythematous papule over her left ear lobule, allegedly following an ear piercing one month prior. She was initially treated at another institution with oral antibiotics. Despite treatment, her mother noted rapid worsening of the lesion, eventually developing into a painful ulceration and affecting the left eyelid as well. At the time of examination, the patient presented with a painful, necrotic plaque around the left eyelid with serpiginous borders (Figure 1) and ear lobule with erythematous, advancing borders (Figure 2A, B). There were no systemic co-morbidities noted. The working diagnosis then was necrotizing fasciitis and she was immediately started on systemic intravenous antibiotics which she did not respond to. Laboratory tests showed elevated CRP, but procalcitonin, C-ANCA and ANA were all normal. Tissue cultures of both eyelid and earlobe, as well as blood cultures, revealed no growth. Wedge biopsy of the eyelid ulceration revealed neutrophilic dermatitis. Biopsy of the ear lobule revealed suppurative granulomatous dermatitis with secondary leucocytoclastic vasculitis. Further workups for infection and possible systemic diseases were all unremarkable. A pathergy test was negative. A diagnosis of pyoderma gangrenosum was made after excluding systemic and infectious causes. The patient was started on systemic prednisone at a dose of 1mg/kg/day which she slowly responded to. Surgical reconstruction of the earlobe was to be planned once the ulceration completely healed; unfortunately, this patient was lost to follow-up