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 w...

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Autores principales: Heather Grace Dulnuan, Carlo Victorio Garcia, Agnes Tirona-Remulla
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Publicado: Philippine Society of Otolaryngology-Head and Neck Surgery, Inc. 2021
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spelling oai:doaj.org-article:ffc9833737eb4bce936c1a591daf32bb2021-11-15T21:14:38ZPyoderma Gangrenosum Initially Presenting as an Ulceration of the Ear Lobule1908-48892094-1501https://doaj.org/article/ffc9833737eb4bce936c1a591daf32bb2021-11-01T00:00:00Zhttps://pjohns.pso-hns.org/index.php/pjohns/article/view/1813https://doaj.org/toc/1908-4889https://doaj.org/toc/2094-1501 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 Heather Grace DulnuanCarlo Victorio GarciaAgnes Tirona-RemullaPhilippine Society of Otolaryngology-Head and Neck Surgery, Inc.articlePyoderma gangrenosumearpediatricneutrophilic dermatitisOtorhinolaryngologyRF1-547ENPhilippine Journal of Otolaryngology Head and Neck Surgery, Vol 36, Iss 2 (2021)
institution DOAJ
collection DOAJ
language EN
topic Pyoderma gangrenosum
ear
pediatric
neutrophilic dermatitis
Otorhinolaryngology
RF1-547
spellingShingle Pyoderma gangrenosum
ear
pediatric
neutrophilic dermatitis
Otorhinolaryngology
RF1-547
Heather Grace Dulnuan
Carlo Victorio Garcia
Agnes Tirona-Remulla
Pyoderma Gangrenosum Initially Presenting as an Ulceration of the Ear Lobule
description 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
format article
author Heather Grace Dulnuan
Carlo Victorio Garcia
Agnes Tirona-Remulla
author_facet Heather Grace Dulnuan
Carlo Victorio Garcia
Agnes Tirona-Remulla
author_sort Heather Grace Dulnuan
title Pyoderma Gangrenosum Initially Presenting as an Ulceration of the Ear Lobule
title_short Pyoderma Gangrenosum Initially Presenting as an Ulceration of the Ear Lobule
title_full Pyoderma Gangrenosum Initially Presenting as an Ulceration of the Ear Lobule
title_fullStr Pyoderma Gangrenosum Initially Presenting as an Ulceration of the Ear Lobule
title_full_unstemmed Pyoderma Gangrenosum Initially Presenting as an Ulceration of the Ear Lobule
title_sort pyoderma gangrenosum initially presenting as an ulceration of the ear lobule
publisher Philippine Society of Otolaryngology-Head and Neck Surgery, Inc.
publishDate 2021
url https://doaj.org/article/ffc9833737eb4bce936c1a591daf32bb
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AT carlovictoriogarcia pyodermagangrenosuminitiallypresentingasanulcerationoftheearlobule
AT agnestironaremulla pyodermagangrenosuminitiallypresentingasanulcerationoftheearlobule
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