PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 PhiliPPine Journal of otolaryngology-head and neck Surgery 5352 PhiliPPine Journal of otolaryngology-head and neck Surgery PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 FEATURED GRAND ROUNDS 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. Pyoderma Gangrenosum Initially Presenting as an Ulceration of the Ear Lobule Correspondence: Dr. Agnes Tirona - Remulla Department of Otolaryngology - Head and Neck Surgery College of Medicine - Philippine General Hospital University of the Philippines Manila Ermita, Manila 1000 Philippines Phone: (632) 8526 4360 Email: antironaremulla@up.edu.ph The authors declared that this represents original material that is not being considered for publication or has not been published or accepted for publication elsewhere, in full or in part, in print or electronic media; that the manuscript has been read and approved by all authors, that the requirements for authorship have been met by each author, and that each author believes that the manuscript represents honest work. Disclosures: The authors signed disclosures that there are no financial or other (including personal) relationships, intellectual passion, political or religious beliefs, and institutional affiliations that might lead to a conflict of interest. Heather Grace Dulnuan, MD Carlo Victorio Garcia, MD Agnes Tirona-Remulla, MD Department of Otolaryngology - Head and Neck Surgery Philippine General Hospital University of the Philippines Manila Philipp J Otolaryngol Head Neck Surg 2021; 36 (2): 52-54 c Philippine Society of Otolaryngology – Head and Neck Surgery, Inc. Creative Commons (CC BY-NC-ND 4.0) Attribution - NonCommercial - NoDerivatives 4.0 International PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 PhiliPPine Journal of otolaryngology-head and neck Surgery 5352 PhiliPPine Journal of otolaryngology-head and neck Surgery PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 FEATURED GRAND ROUNDS DISCUSSION Pyoderma gangrenosum is rare in childhood, with approximately 4% of PG cases seen in infants and children usually affecting the head and perineal area.4 Studies have also shown that there is a female preponderance because reactive neutrophilic dermatoses are known to affect females.5 The disease arising from a neutrophilic process is the well-accepted etiology. With advances in biologic therapies, it has been found that it may be related to overexpression of growth factors IL-8, IL-18, IL-16, and TNF-α. Metabolic oscillations and aberrant neutrophil trafficking is also observed.6 Clinically, it begins as a painful nodule that may be seen after episodes of trauma. Pain is a prominent feature in the pediatric population. In the classic lesion, the pustule progresses to a necrotic ulceration with irregular, red-purplish inflammatory borders and a purulent or bloody exudate.7 They may present anywhere in the body, usually in multiples sites, as compared to the adult population where lesions are solitary and observed in the extremities.8 Aside from the classic presentation, other PG subtypes are pustular, bullous, vegetative, peristomal, genital, infantile and extracutaneous.9 In most cases, a biopsy is warranted to rule out other causes for the cutaneous lesion. Specimens can also be sent for bacterial and fungal cultures. Around 20% of patients present with pathergy, where inciting trauma (such as a biopsy or venipuncture) forms a new lesion.10 The histopathologic findings seen in the classic lesion is ulcerative associated with dense neutrophilic infiltrates. Su et al.10 proposed a diagnostic criterion for PG, where the major criteria is a rapidly enlarging, painful ulceration in the absence of any other cause for the lesion. Since then, modifications to this criterion have been made; however, PG remains to be a diagnosis of exclusion.10 The key to diagnosis is a thorough history with emphasis on a history of pathergy, associated pain, and the presence of associated systemic diseases. When present in the head and neck, differential diagnoses such as infected preauricular cysts or sinuses, dissecting folliculitis, ulcerating basal cell carcinoma, and trigeminal trophic syndrome should be considered.11 PG often occurs in isolation but may be associated with other systemic conditions in up to 50% of cases.3 In pediatric patients, it is most commonly associated with Inflammatory Bowel Disease (IBD), warranting investigation of gastrointestinal symptoms and further examination through endoscopy.8 It is also associated with hematologic disorders, such as myelodysplastic syndromes, leukemias and lymphoma.8 In the pre-adolescent population, PG is often idiopathic or associated with hematologic disorders, while IBD and PAPA (Pyogenic Arthritis, Pyoderma gangrenosum and Acne) are seen in the adolescent population.8 In the Philippines, one of the cases of PG published in literature is that of an adult female suffering from acute myelogenous leukemia who presented with the bullous type of the disease.11 PG may also be the initial presentation of vasculitis such as Behcet disease and Takayasu’s arteritis, rheumatoid arthritis, and neoplasms.12 Due to the rarity of the disease, there is no definite effective therapy for PG, and treatment has mainly been empiric. Treatment goals include relieving pain, controlling the inflammatory process and managing the underlying disease.9 Oral corticosteroids with a usual dose of 0.5-1mg/ Figure 1. Ulcerative lesions around an erythematous margin and serpiginous borders involving the upper eyelid Figure 2. Rapidly progressive ulceration with areas of necrosis around the left A. preauricular; and B. post auricular area. PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 PhiliPPine Journal of otolaryngology-head and neck Surgery 5554 PhiliPPine Journal of otolaryngology-head and neck Surgery PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 36 no. 2 July – december 2021 FEATURED GRAND ROUNDS REFERENCES 1. Farhi D. The clinical and histopathological description of geometric phagedenism (pyoderma gangrenosum) by Louis Brocq one century ago.  Arch Dermatol.  2008 Jun;144(6):755. DOI:10.1001/archderm.144.6.755 PubMed PMID: 18559764. 2. Brunsting LA, Goeckerman WH, O’Leary PA. Pyoderma (echthyma) gangrenosum: clinical and experimental observation in five cases occurring in adults. Arch Dermatol Syphilol. 1930 Oct; 22(4):655–680. DOI: 10.1001/archderm.1930.01440160053009. 3. Dos Santos Sousa MC, Lima Lemos, EF, Oliveira De Morais O, Silva Leite Coutinho AS, Martins Gomes C. Pyoderma gangrenosum leading to bilateral involvement of ears. J Clin Aesthet Dermatol. 2014 Jan;7(1):41–43. PubMed PMID: 24563696. PubMed Central PMCID: PMC3930540. 4. Powell FC, Schroeter AL, Su WP. Pyoderma gangrenosum: a review of 86 patients. Q J Med. 1985 May;55(217):173–186. PubMed PMID: 3889978. 5. Von Den Driesch P. Pyoderma gangrenosum: a report of 44 cases with follow-up. Brit J Dermatol. 1997;137(6);1000–1005. DOI:10.1046/j.1365-2133.1997.20022083.x. 6. Adachi Y, Kindzelskii AL, Cookingham G, Shaya S, Moore EC, Todd RF 3rd, Petty HR. Aberrant neutrophil trafficking and metabolic oscillations in severe pyoderma gangrenosum. J Invest Dermatol. 1998 Aug;111(2):259-68. DOI: 10.1046/j.1523-1747.1998.00311.x. PubMed PMID: 9699727. 7. Schoch JJ, Tolkachjov SN, Cappel JA, Gibson LE, Davis DM. Pediatric pyoderma gangrenosum: A retrospective review of clinical features, etiologic associations, and treatment. Pediatr Dermatol. 2017 Jan;34(1):39-45. DOI: 10.1111/pde.12990. PubMed PMID: 27699861. 8. Kechichian E, Haber R, Mourad N, El Khoury R, Jabbour S, Tomb R. Pediatric pyoderma gangrenosum: a systematic review and update. Int J Dermatol. 2017 May;56(5):486-495. DOI: 10.1111/ijd.13584. PubMed PMID: 28233293. 9. Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti G. Pyoderma gangrenosum: an updated review. J Eur Acad Dermatol Venerol. 2009 Sep;23(9):1008-17. DOI: 10.1111/j.1468- 3083.2009.03199.x. PubMed PMID: 19470075. 10. Su WPD, Davis MDP, Weenig RH, Powell FC, Perry HO. Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria. Int J Dermatol. 2004 Nov;43(11):790–800. DOI: 10.1111/j.1365-4632.2004.02128.x PubMed PMID: 15533059. 11. Snyder RA. Pyoderma gangrenosum involving the head and neck. Arch Dermatol. 1986 Mar;122(3):295-302. PubMed PMID: 2869733. 12. Ramos VME, Chamberlin CVS, Dofitas BL. A rare case of bullous pyoderma gangrenosum in a patient with acute myelogenous leukemia. Acta Medica Philippina. 2020;54(3):336-339. DOI: https://doi.org/10.47895/amp.v54i3.1687. 13. Powell FC, Perry HO. Pyoderma gangrenosum in childhood.  Arch Dermatol 1984;120(6):757– 761. DOI:10.1001/archderm.1984.01650420067018. kg/day have been widely used in the pediatric and adult population due to the rapid response to treatment.9 Other reported systemic treatments are immunosuppressive drugs, thalidomide, minocycline, sulphasalazine, and dapsone. Infliximab and other biologic treatments have also been reported to provide clinical improvement after one infusion.8 Local wound care and pain control are also necessary. In summary, pediatric pyoderma gangrenosum may present in multiple sites, and in atypical areas such as the head and neck. As a diagnosis of exclusion, a thorough workup is imperative to diagnose other etiologies and evaluate co-morbidities associated with the disease. Once diagnosed, aggressive immunosuppression should be started to facilitate complete recovery.