Publication
Acute Disseminated Panniculitis Associated with Alpha-1 Antitrypsin Deficiency
| dc.contributor.author | Murinello, A | |
| dc.contributor.author | Sá Damásio, H | |
| dc.contributor.author | Guedes, P | |
| dc.contributor.author | Figueiredo, AM | |
| dc.contributor.author | Santos, A | |
| dc.contributor.author | Serrano, A | |
| dc.date.accessioned | 2023-02-28T15:55:56Z | |
| dc.date.available | 2023-02-28T15:55:56Z | |
| dc.date.issued | 2022 | |
| dc.description.abstract | Alpha-1 antitrypsin deficiency, although one of the most common genetic diseases, is a very rare and often undiagnosed cause of panniculitis. The authors present a case characterized by an acute involvement of several areas in the thorax, abdomen, and limbs, occurring after repetitive trauma of the perineal area caused by a long period of cycling. After performing the differential diagnosis and establishing etiology, the patient was started on augmentation therapy with plasma-derived synthetic human alpha-1 proteinase inhibitor and the disease has been under control since then. We recommend lifelong treatment with this medication. At the end of a 10-year follow-up, there has been no evidence of pulmonary emphysema or liver disease. The authors perform a concise review of the genetic and pathogenic mechanisms behind this disease, with a special focus on panniculitis and its treatment. | pt_PT |
| dc.description.version | info:eu-repo/semantics/publishedVersion | pt_PT |
| dc.identifier.citation | Case Rep Dermatol . 2022 Oct 6;14(3):283-290. | pt_PT |
| dc.identifier.doi | 10.1159/000526156 | pt_PT |
| dc.identifier.uri | http://hdl.handle.net/10400.17/4423 | |
| dc.language.iso | eng | pt_PT |
| dc.publisher | Karger | pt_PT |
| dc.subject | HCC MED | pt_PT |
| dc.subject | Acute Panniculitis | pt_PT |
| dc.subject | Alpha-1 Antitrypsin Deficiency | pt_PT |
| dc.subject | Pulmonary Emphysema | pt_PT |
| dc.title | Acute Disseminated Panniculitis Associated with Alpha-1 Antitrypsin Deficiency | pt_PT |
| dc.type | journal article | |
| dspace.entity.type | Publication | |
| oaire.citation.endPage | 290 | pt_PT |
| oaire.citation.issue | 3 | pt_PT |
| oaire.citation.startPage | 283 | pt_PT |
| oaire.citation.title | Case Reports in Dermatology | pt_PT |
| oaire.citation.volume | 14 | pt_PT |
| rcaap.rights | openAccess | pt_PT |
| rcaap.type | article | pt_PT |