Problemi speciali
Pioderma gangrenoso
Pyoderma gangrenosum as a possible sign of a rheumatic disease also in paediatric age
Francesca Angrisani1, Sabrina Acierno1, Achille Marino2, Cecilia Chighinzola2,3, Roberto Caporali3,4, Teresa Giani3, Rolando Cimaz2,3
1Scuola Università della Campania “Luigi Vanvitelli”, Napoli
2Reumatologia pediatrica, ASST Pini, Milano
3Università di Milano
4Reumatologia clinica, ASST Pini, Milano
Dicembre 2022 - pagg. 627 -631 | DOI: 10.53126/MEB41627
Abstract
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis, characterized by unknown aetiology and sterile neutrophilic infiltrate on histological examination, clinically showing recurrent skin ulcers and mostly affecting lower limbs areas. In 2/3 of the cases, PG is secondary to another disorder such as inflammatory bowel and lymphoproliferative diseases, infections, rheumatic diseases and auto-inflammatory syndromes. The paper describes the cases of three paediatric patients showing the association of PG with rheumatic conditions such as Behçet’s disease, chronic recurrent multifocal osteomyelitis (CRMO) and PAPA (Pyogenic Arthritis, Pyoderma gangrenosum and Acne) syndrome and proposes a diagnostic flow-chart to help paediatricians recognize PG and associated conditions.
Riassunto
Il pioderma gangrenoso (PG) è una rara dermatosi che si manifesta con ulcere cutanee ricorrenti; appartiene alle dermatosi neutrofiliche, caratterizzate dalla presenza di un infiltrato sterile neutrofilico all’esame istologico. Le aree maggiormente interessate sono gli arti inferiori. L’eziologia è ignota. Nei 2/3 dei casi il PG è secondario a un’altra patologia: malattia infiammatoria dell’intestino, patologie linfoproliferative, infezioni, malattie reumatologiche e sindromi autoinfiammatorie.
In questo articolo vengono descritti tre casi pediatrici nei quali il PG si accompagna a patologie reumatologiche quali morbo di Behçet, osteomielite multifocale cronica ricorrente e sindrome PAPA (artrite piogenica, pioderma gangrenoso e acne). Viene infine proposta una flow-chart per l’inquadramento diagnostico del PG con lo scopo di fornire uno strumento utile al pediatra per il riconoscimento di eventuali patologie correlate, il cui riscontro può condizionare la terapia e la prognosi di entrambe.
Parole chiave
Suggerite dall'AI
Classificazione MeSH
Contenuto riservato
Per leggere l'articolo completo è necessario effettuare il login.
Non sei ancora registrato? Registrati
Bibliografia
1. Newell LM, Malkinson FD. “Pyoderma (ecthyma) gangrenosum” by Brunsting, Goeckerman and O’Leary, October 1930. Commentary: Pyoderma gangrenosum. Arch Dermatol 1982;118(10):743-73. doi: 10.1001/ archderm.118.10.743.
2. Ashchyan HJ, Butler DC, Nelson CA, et al. The Association of Age With Clinical Presentation and Comorbidities of Pyoderma Gangrenosum. JAMA Dermatol 2018;154(4):409-413. doi: 10.1001/jamadermatol.2017.5978. Erratum in: JAMA Dermatol 2018;154(5):630.
3. Nelson CA, Stephen S, Ashchyan HJ, James WD, Micheletti RG, Rosenbach M. Neutrophilic dermatoses: Pathogenesis, Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease. J Am Acad Dermatol 2018;79(6):987-1006. doi: 10.1016/j.jaad. 2017.11.064.
4. Bucchia M, Barbarot S, Reumaux H, et al.; Groupe de recherche de la Société Française de Dermatologie Pédiatrique; Société Francophone pour la rhumatologie et les Maladies Inflammatoires en Pédiatrie (SOFREMIP). Age-specific characteristics of neutrophilic dermatoses and neutrophilic diseases in children. J Eur Acad Dermatol Venereol 2019;33(11):2179-2187. doi: 10.1111/jdv. 15730.
5. Xia FD, Liu K, Lockwood S,et al. Risk of developing pyoderma gangrenosum after procedures in patients with a known history of pyoderma gangrenosum-A retrospective analysis. J Am Acad Dermatol 2018;78(2): 310-314.e1. doi: 10.1016/j.jaad.2017.09.040.
6. Maverakis E, Marzano AV, Le ST, et al. Pyoderma gangrenosum. Nat Rev Dis Primers 2020;6(1):81. doi: 10.1038/s41572-020-0213-x.
7. Katsuo K, Honda T, Kaku Y, et al. Pyoderma gangrenosum associated with chronic recurrent multifocal osteomyelitis as a possible paradoxical reaction to anti-tumor necrosis factor-α therapy. J Dermatol 2020;47(8):e283-e284. doi: 10.1111/1346-8138.15417.
8. 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. doi: 10.1001/jamadermatol.2017. 5980.
9. Jockenhöfer F, Wollina U, Salva KA, et al. The PARACELSUS score: a novel diagnostic tool for pyoderma gangrenosum. Br J Dermatol 2018;180(3):615‐20. doi: 10.1111/bjd. 16401.
10. Hali F, Khadir K, Chiheb S, Bouayad K, Mikou N, Benchikhi H. Pyoderma gangrenosum et maladie de Behçet : étude de 2 observations pédiatriques [Pyoderma gangrenosum and Behçet’s disease: a study of two pediatric cases]. Arch Pediatr 2011;18(12):1320-3. French. doi: 10.1016/j.arcped.2011.09.007.
11. Akay N, Boyvat A, Heper AO, et al. Behçet’s disease-like presentation of bullous pyoderma gangrenosum associated with Crohn’s disease. Clin Exp Dermatol 2006;31(3):384-6. doi: 10.1111/j.1365-2230. 2006.02093.x.
12. Vettiyil G, Punnen A, Kumar S. An Unusual Association of Chronic Recurrent Multifocal Osteomyelitis, Pyoderma Gangrenosum, and Takayasu Arteritis. J Rheumatol 2017;44(1):127-8. doi: 10.3899/jrheum. 160491.
13. Nurre LD, Rabalais GP, Callen JP. Neutrophilic dermatosis-associated sterile chronic multifocal osteomyelitis in pediatric patients: case report and review. Pediatr Dermatol 1999;16(3):214-6. doi: 10.1046/j.1525-1470.1999.00058.x.
14. Maejima H, Watarai A, Tanabe K, Takasu H, Katsuoka K, Sada M. Osteomyelitis in association with pyoderma gangrenosum and ulcerative colitis. Cutis 2014;93(4):E14-6.
15. Ferrara G, Sandrin S, Pastore S, Gortani G, Taddio A, Ventura A. L’osteomielite cronica multifocale ricorrente: se la conosci la riconosci. Medico e Bambino 2014;33(7):433-437.
16. Chen J, Yao X. A Contemporary Review of Behcet’s Syndrome. Clin Rev Allergy Immunol 2021;61(3):363-76. doi: 10.1007/ s12016-021-08864-3.
17. Moreira A, Torres B, Peruzzo J, Mota A, Eyerich K, Ring J. Skin symptoms as diagnostic clue for autoinflammatory diseases. An Bras Dermatol 2017;92(1):72-80. doi: 10. 1590/abd1806-4841.20175208.
18. Cugno M, Borghi A, Marzano AV. PAPA, PASH and PAPASH Syndromes: Pathophysiology, Presentation and Treatment. Am J Clin Dermatol 2017;18(4):555-62. doi: 10.1007/s40257-017-0265-1.
19. Kridin K, Cohen AD, Amber KT. Underlying Systemic Diseases in Pyoderma Gangrenosum: A Systematic Review and Meta-Analysis. Am J Clin Dermatol 2018;19(4): 479-87. doi: 10.1007/s40257-018-0356-7.
20. 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;56(5):486-495. doi: 10.1111/ijd.13584.
Corrispondenza: teresa.giani@gmail.com