Diverse cutaneous manifestations of pyoderma gangrenosum with triple therapeutic trial in a series of 53 cases
Keywords:Pyoderma gangrenosum, Neutrophilic dermatosis, Triple therapy, Etiopathogenesis
AbstractBackground: Pyoderma gangrenosum is a chronic auto-inflammatory neutrophilic skin reaction to an underlying systemic disease characterized by small, red bumps or blisters that eventually erode to form ulcerations where in around 50% of cases are associated with underlying systemic disease. Objective: To characterize the demographic and the cutaneous features of pyoderma gangrenosum in large series of patients. Patients and methods: This is a case series cross sectional descriptive study with therapeutic trial where 53 patients with pyoderma gangrenosum were seen during the period from September 2013-January 2023.All patients were fully investigated and diagnosed as cases of pyoderma gangrenosum. Still full history and examination were carried out to characterize the clinical pictures. All patients were fully investigated to exclude any associated disease in doubtful diagnosis. Skin biopsy was performed when needed as exclusive measure. Triple therapeutic trial was tried using oral azathioprine, oral dapsone and oral prednisolone. Results: Fifty-three patients were included, their ages ranged from 10-60 years with a mean 45 years, with 39(73.58%) males and 14(26.41%) females with ratio 2.78:1. The duration of lesions ranged from 4-12 weeks. The location of these lesions was mostly lower limbs in 31(58.49%) cases. For the rest of the cases, lesions appeared on the trunk including the groin and neck in 14(26.41%), breast of the female in 3(5.66%) cases, upper limbs in 2 (3.77%) cases, male genitalia in 2(3.77%) cases and face in one (1.88%) case. The lesions were single in 36(67.9.21%) patients while multiple in 17(32%) cases. The rash had mostly painful ulcerative yellowish surface with undermined edges. The ulcerative subtype was the most frequently recorded in 48(90.56%) patients. No associated underlying triggering pathologies were noticed during follow up except in seven (13.2%) patients. The therapeutic response was noticed after two weeks but complete clearance of ulcers took several weeks to months according to the size of ulceration. Conclusion: This study showed that pyoderma gangrenosum is a disease of middle-aged males with no well-defined etiological triggering factors except in 13.2% of cases that have been associated with miscellaneous underlying diseases. Accordingly, the etiopathogenesis of pyoderma gangrenosum remains idiopathic, still long follow up might discover many other concealed etiologies. Effective triple therapy was tried and proved its effectiveness.
Ahronowitz I, Harp J, Shinkai K. Etiology and management of pyoderma gangrenosum: a comprehensive review. Am J Clin Dermatol. 2012; 13:191-211.
Ruocco E, Sangiuliano S, Gravina AG, et al. Pyoderma gangrenosum: an updated review. J Eur Acad Dermatol Venereol. 2009; 23:1008-1017.
Davis MDP, Moschella SL. Neutrophilic dermatoses. In: Bolognia J, Schaffer J, Cerroni L, editors. Dermatology. 4th ed. Philadelphia; Elsevier; 2017. p. 453–71.
Kubaisi T. A neglected pyoderma gangrenosum following COVID-19: A case report. J Pak Assoc Dermatol [Internet]. 2023;33(1):345-9.
Skopis M, Bag-Ozbek A. Pyoderma Gangrenosum: A Review of Updates in Diagnosis, Pathophysiology and Management. Multidisciplinary Scientific Journal. 2021;4(3):367-375.
Wang EA, Steel A, Luxardi G et al. Classic ulcerative pyoderma gangrenosum is a T cell-mediated disease targeting follicular adnexal structures: A hypothesis based on molecular and cinicopathologic studies. Frontiers in Immunology. 2018 Jan 15; 8:1980.
Cugno M, Borghi A, Marzano AV. PAPA, PASH and PAPASH Syndromes: Pathophysiology, Presentation and Treatment. Am J Clin Dermatol. 2017 Aug;18(4):555-562.
Ortega-Loayza AG, Nugent WH, Lucero OM, et al. Dysregulation of inflammatory gene expression in lesional and nonlesional skin of patients with pyoderma gangrenosum. Br J Dermatol. 2018;178(1): e35-e36
McKenzie F, Arthur, M, Ortega-Loayza. Pyoderma Gangrenosum: What Do We Know Now? Wound Care and Healing. 2018; 18:555-562.
Maverakis E, Ma C, Shinkai K, et al. Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts. JAMA Dermatol. 2018; 154(4): 461–466.
Weenig RH, Davis MD, Dahl PR, et al. Skin ulcers misdiagnosed as pyoderma gangrenosum. N Engl J Med. 2002; 347(18): 1412–8.
Chow RKP, Ho VC. Treatment of pyoderma gangrenosum. J Am Acad Dermato. 1996; 34:1047–60.
Powell FC, Schroeter AL, Su WP et al. Pyoderma gangrenosum: a review of 86 patients. Q J Med. 1985; 55:173–86.
Massa MC, Doyle JA. Cutaneous cryptococcosis simulating pyoderma gangrenosum. J Am Acad Dermatol. 1981; 5: 32-36.
Binus AM, Qureshi AA, Li VW, et al. Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br J Dermatol. 2011;165(6):1244- 1250.
Von den Driesch P. Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol. 1997;137(6):1000-1005.
Bennett ML, Jackson JM, Jorizzo JL, et al. Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions. Medicine (Baltimore). 2000;79(1):37-46.
Al Ghazal P, Körber A, Klode J, Dissemond J. Investigation of new co-factors in 49 patients with pyoderma gangrenosum. J Dtsch Dermatol Ges. 2012 Apr;10(4):251-7.
Al Ghazal P, Herberger K, Schaller J, et al. Associated factors and comorbidities in patients with pyoderma gangrenosum in Germany: a retrospective multicentric analysis in 259 patients.Orphanet J Rare Dis. 2013;8:136.
Pereira N, Brites MM, Gonçalo M, et al. Pyoderma gangrenosum--a review of 24 cases observed over 10 years. Int J Dermatol. 2013 Aug;52(8):938-45.
Saracino A, Kelly R, Liew D, Chong A. Pyoderma gangrenosum requiring inpatient management: a report of 26 cases with follow up. Australas J Dermatol. 2011 Aug;52(3):218-21.
Hasselmann DO, Bens G, Tilgen W, Reichrath J. Pyoderma gangrenosum: clinical presentation and outcome in 18 cases and review of the literature. J Dtsch Dermatol Ges. 2007 Jul;5(7):560-4.
Vidal D, Puig L, Gilaberte M, Alomar A. Review of 26 cases of classical pyoderma gangrenosum: clinical and therapeutic features. J Dermatolog Treat. 2004 Jun;15(3):146-52.
Bennett ML, Jackson JM, Jorizzo JL, et al. Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions. Medicine (Baltimore). 2000 Jan;79(1):37-46.
Callen JP, Jackson JM. Pyoderma gangrenosum: an update. Rheum Dis Clin North Am. 2007; 33:787–802.
Jackson JM, Callen JP. Pyoderma gangrenosum: an expert commentary. Expert Rev. Dermatol. 2006; 1:391–400.
Varol A, Seifert O, Anderson CD. The skin pathergy test: innately useful? Arch Dermatol Res. 2010 Apr;302(3):155-68.
Partridge ACR, Bai JW, Rosen CF, et al. Effectiveness of systemic treatments for pyoderma gangrenosum: a systematic review of observational studies and clinical trials. Br J Dermatol. 2018 Aug;179(2):290-295.
Alavi A, French LE, Davis MD, et al. Pyoderma Gangrenosum: An Update on Pathophysiology, Diagnosis and Treatment. Am J Clin Dermatol. 2017 Jun;18(3):355-372.