Pediatrics International (2015) 57, 505–506
doi: 10.1111/ped.12648
Photos in Pediatrics
Pyoderma gangrenosum with systemic and pulmonary involvement in a toddler Despoina N Maritsi,1 Kyriaki Tavernaraki2 and George Vartzelis1 Second Department of Pediatrics, “P. & A. Kyriakou” Children’s Hospital, Medical School, Athens University, Athens, Greece and 2 Department of Medical Imaging and Interventional Radiology, ‘Sotiria’ General Hospital, Athens, Greece
1
Pyoderma gangrenosum (PG) is a chronic ulcerative skin condition of unknown origin that is exceedingly rare in children.1,2 This entity is associated with certain systemic illnesses such as inflammatory bowel disease, malignancies6 and autoinflammatory syndromes.5 Pulmonary involvement in association with PG is uncommon.7,8 We report the case of a toddler with PG and necrotic pulmonary lesions, which responded to treatment with corticosteroids and i.v. immunoglobulin (IVIG). A 14-month-old girl presented with pyrexia, raised inflammatory markers and an erythematous, painful, indurated lesion on the anterior tibia, which erupted, creating a wide ulcer with violaceous borders (Fig. 1). Blood, sputum and skin cultures including extended cultures for mycobacteria and fungi were negative.
Her condition deteriorated despite antibiotic treatment, and she had weight loss, anemia, fever and tachypnea. Chest radiography showed interstitial involvement (Fig. 2a); chest computed tomography (CT; Fig. 2b) showed consolidations with internal areas of cavitation, surrounding ground-glass changes and lymphadenopathy. Differential diagnosis included primary immunodeficiency, systemic vasculitides or malignancy. Humoral and cellular immune response and serum angiotensin-converting-enzyme were normal;
a
b
Fig. 1 Pyoderma gangrenosum on the left anterior tibial surface.
Correspondence: Despoina N. Maritsi, MD, MSc, PhD, MRCPCH, Second Department of Pediatrics, “P. & A. Kyriakou” Children’s Hospital, Medical Faculty, National and Kapodestrian University of Athens, Athens, Greece. Email: [email protected] Received 24 February 2014; revised 17 December 2014; accepted 13 February 2015. © 2015 Japan Pediatric Society
Fig. 2 (a) Chest X-ray. (b) Chest computed tomography.
506 D N Maritsi et al. perinuclear and cytoplasmic anti-neutrophilic cytoplasmic antibody were negative, upper and lower gastrointestinal endoscopy was normal; and bone marrow aspirate showed reactive changes in keeping with an inflammatory process. The patient tested negative for pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome (PTSPIP1 gene sequence analysis). Skin biopsy showed necrosis, dermal neutrophil infiltration and perivascular lymphocytic infiltrates, consistent with PG; there was no evidence of granulomatous inflammation or primary vasculitis. The patient received pulsed i.v. methylprednisolone followed by 6 week oral steroid taper and monthly IVIG for 6 months. Skin improvement was gradual, leaving a residual scar. Follow-up chest CT at 6 months showed no lesions. Despite advances in management, the long-term outcome of PG remains unpredictable.9 Treatment with steroids, cyclosporine and mycofenolate-mofetyl has been shown to be effective, while the use of anti-tumor necrosis factor-α inhibitors is reserved for refractory cases.10 In the present case IVIG was successful as a steroid-sparing agent;11,12 thus it may serve as safe and effective adjuvant therapy in the treatment of PG.
Acknowledgments The authors declare no conflicts of interest and this study received no specific funding.
References 1 Callen JP. Pyoderma gangrenosum. Lancet 1998; 351: 581–5.
© 2015 Japan Pediatric Society
2 Binus AM, Qureshi AA, Li VW, Winterfield LS. Pyoderma gangrenosum: A retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br. J. Dermatol. 2011; 165: 1244–50. 3 Ampuero J, Rojas-Feria M, Castro-Fernández M, Cano C, Romero-Gómez M. Predictive factors for erythema nodosum and pyoderma gangrenosum in inflammatory bowel disease. J. Gastroenterol. Hepatol. 2014; 29: 291–5. 4 Aloi M, Cucchiara S. Extradigestive manifestations of IBD in pediatrics. Eur. Rev. Med. Pharmacol. Sci. 2009; 13 (Suppl 1): 23–32. 5 Almeida de JA, Goldbach-Mansky R. Monogenic autoinflammatory diseases: Concept and clinical manifestations. Clin. Immunol. 2013; 147: 155–74. 6 Kurzrock R, Cohen PR. Mucocutaneous paraneoplastic manifestations of hematologic malignancies. Am. J. Med. 1995; 99: 207–16. 7 Chahine B, Chenivesse C, Tillie-Leblond I et al. Pulmonary manifestations of pyoderma gangrenosum. Presse Med. 2007; 36: 1395–8. 8 Liu ZH, Lu XL, Fu MH, Zhang GY, Liu WD. Pyoderma gangrenosum with pulmonary involvement? Eur. J. Dermatol. 2008; 18: 583–5. 9 Wollina U. Pyoderma gangrenosum: A review. Orphanet J. Rare Dis.2007; 2: 19. 10 Ahronowitz I, Harp J, Shinkai K. Etiology and management of pyoderma gangrenosum: A comprehensive review. Am. J. Clin. Dermatol. 2012; 13: 191–211. 11 Cummins DL, Anhalt GJ, Monahan T, Meyerle JH. Treatment of pyoderma gangrenosum with intravenous immunoglobulin. Br. J. Dermatol. 2007; 157: 1235–9. 12 Kreuter A, Reich-Schupke S, Stücker M, Altmeyer P, Gambichler T. Intravenous immunoglobulin for pyoderma gangrenosum. Br. J. Dermatol. 2008; 158: 856–7.
doi: 10.1111/ped.12648
Photos in Pediatrics
Pyoderma gangrenosum with systemic and pulmonary involvement in a toddler Despoina N Maritsi,1 Kyriaki Tavernaraki2 and George Vartzelis1 Second Department of Pediatrics, “P. & A. Kyriakou” Children’s Hospital, Medical School, Athens University, Athens, Greece and 2 Department of Medical Imaging and Interventional Radiology, ‘Sotiria’ General Hospital, Athens, Greece
1
Pyoderma gangrenosum (PG) is a chronic ulcerative skin condition of unknown origin that is exceedingly rare in children.1,2 This entity is associated with certain systemic illnesses such as inflammatory bowel disease, malignancies6 and autoinflammatory syndromes.5 Pulmonary involvement in association with PG is uncommon.7,8 We report the case of a toddler with PG and necrotic pulmonary lesions, which responded to treatment with corticosteroids and i.v. immunoglobulin (IVIG). A 14-month-old girl presented with pyrexia, raised inflammatory markers and an erythematous, painful, indurated lesion on the anterior tibia, which erupted, creating a wide ulcer with violaceous borders (Fig. 1). Blood, sputum and skin cultures including extended cultures for mycobacteria and fungi were negative.
Her condition deteriorated despite antibiotic treatment, and she had weight loss, anemia, fever and tachypnea. Chest radiography showed interstitial involvement (Fig. 2a); chest computed tomography (CT; Fig. 2b) showed consolidations with internal areas of cavitation, surrounding ground-glass changes and lymphadenopathy. Differential diagnosis included primary immunodeficiency, systemic vasculitides or malignancy. Humoral and cellular immune response and serum angiotensin-converting-enzyme were normal;
a
b
Fig. 1 Pyoderma gangrenosum on the left anterior tibial surface.
Correspondence: Despoina N. Maritsi, MD, MSc, PhD, MRCPCH, Second Department of Pediatrics, “P. & A. Kyriakou” Children’s Hospital, Medical Faculty, National and Kapodestrian University of Athens, Athens, Greece. Email: [email protected] Received 24 February 2014; revised 17 December 2014; accepted 13 February 2015. © 2015 Japan Pediatric Society
Fig. 2 (a) Chest X-ray. (b) Chest computed tomography.
506 D N Maritsi et al. perinuclear and cytoplasmic anti-neutrophilic cytoplasmic antibody were negative, upper and lower gastrointestinal endoscopy was normal; and bone marrow aspirate showed reactive changes in keeping with an inflammatory process. The patient tested negative for pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome (PTSPIP1 gene sequence analysis). Skin biopsy showed necrosis, dermal neutrophil infiltration and perivascular lymphocytic infiltrates, consistent with PG; there was no evidence of granulomatous inflammation or primary vasculitis. The patient received pulsed i.v. methylprednisolone followed by 6 week oral steroid taper and monthly IVIG for 6 months. Skin improvement was gradual, leaving a residual scar. Follow-up chest CT at 6 months showed no lesions. Despite advances in management, the long-term outcome of PG remains unpredictable.9 Treatment with steroids, cyclosporine and mycofenolate-mofetyl has been shown to be effective, while the use of anti-tumor necrosis factor-α inhibitors is reserved for refractory cases.10 In the present case IVIG was successful as a steroid-sparing agent;11,12 thus it may serve as safe and effective adjuvant therapy in the treatment of PG.
Acknowledgments The authors declare no conflicts of interest and this study received no specific funding.
References 1 Callen JP. Pyoderma gangrenosum. Lancet 1998; 351: 581–5.
© 2015 Japan Pediatric Society
2 Binus AM, Qureshi AA, Li VW, Winterfield LS. Pyoderma gangrenosum: A retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br. J. Dermatol. 2011; 165: 1244–50. 3 Ampuero J, Rojas-Feria M, Castro-Fernández M, Cano C, Romero-Gómez M. Predictive factors for erythema nodosum and pyoderma gangrenosum in inflammatory bowel disease. J. Gastroenterol. Hepatol. 2014; 29: 291–5. 4 Aloi M, Cucchiara S. Extradigestive manifestations of IBD in pediatrics. Eur. Rev. Med. Pharmacol. Sci. 2009; 13 (Suppl 1): 23–32. 5 Almeida de JA, Goldbach-Mansky R. Monogenic autoinflammatory diseases: Concept and clinical manifestations. Clin. Immunol. 2013; 147: 155–74. 6 Kurzrock R, Cohen PR. Mucocutaneous paraneoplastic manifestations of hematologic malignancies. Am. J. Med. 1995; 99: 207–16. 7 Chahine B, Chenivesse C, Tillie-Leblond I et al. Pulmonary manifestations of pyoderma gangrenosum. Presse Med. 2007; 36: 1395–8. 8 Liu ZH, Lu XL, Fu MH, Zhang GY, Liu WD. Pyoderma gangrenosum with pulmonary involvement? Eur. J. Dermatol. 2008; 18: 583–5. 9 Wollina U. Pyoderma gangrenosum: A review. Orphanet J. Rare Dis.2007; 2: 19. 10 Ahronowitz I, Harp J, Shinkai K. Etiology and management of pyoderma gangrenosum: A comprehensive review. Am. J. Clin. Dermatol. 2012; 13: 191–211. 11 Cummins DL, Anhalt GJ, Monahan T, Meyerle JH. Treatment of pyoderma gangrenosum with intravenous immunoglobulin. Br. J. Dermatol. 2007; 157: 1235–9. 12 Kreuter A, Reich-Schupke S, Stücker M, Altmeyer P, Gambichler T. Intravenous immunoglobulin for pyoderma gangrenosum. Br. J. Dermatol. 2008; 158: 856–7.
