Alpha‐1‐antitrypsin deficiency‐related panniculitis: two cases with diverse clinical courses
Summary Alpha‐1‐antitrypsin deficiency (AATD)‐related panniculitis is an extremely rare and underdiagnosed entity, and there is a paucity of data on its treatment. We report two cases of AATD‐related panniculitis. The first was a 24‐year‐old woman with known AATD who presented with painful leg ulcer...
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| Veröffentlicht in: | Clinical and experimental dermatology 2017-07, Vol.42 (5), p.520-522 |
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| container_title | Clinical and experimental dermatology |
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| creator | Storan, E. R. O' Gorman, S. M. Hawkins, P. Aalto, L. Murphy, A. Markham, T. |
| description | Summary
Alpha‐1‐antitrypsin deficiency (AATD)‐related panniculitis is an extremely rare and underdiagnosed entity, and there is a paucity of data on its treatment. We report two cases of AATD‐related panniculitis. The first was a 24‐year‐old woman with known AATD who presented with painful leg ulcers refractory to treatment with corticosteroids and colchicine. She had a good response to α1‐antitrypsin infusions but required dose adjustment due to flares in disease activity. The second case was a 38‐year‐old woman who presented with painful nodules on the legs refractory to corticosteroid therapy. Laboratory investigations revealed severe AATD. She had an excellent response to colchicine therapy. In both these cases of AATD, panniculitis was the first clinical manifestation of the disease. AATD‐related panniculitis may have none of the typical clinical clues for AATD, such as a family history, cirrhosis or emphysema. Early identification may help prevent these complications from developing. |
| doi_str_mv | 10.1111/ced.13102 |
| format | Article |
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Alpha‐1‐antitrypsin deficiency (AATD)‐related panniculitis is an extremely rare and underdiagnosed entity, and there is a paucity of data on its treatment. We report two cases of AATD‐related panniculitis. The first was a 24‐year‐old woman with known AATD who presented with painful leg ulcers refractory to treatment with corticosteroids and colchicine. She had a good response to α1‐antitrypsin infusions but required dose adjustment due to flares in disease activity. The second case was a 38‐year‐old woman who presented with painful nodules on the legs refractory to corticosteroid therapy. Laboratory investigations revealed severe AATD. She had an excellent response to colchicine therapy. In both these cases of AATD, panniculitis was the first clinical manifestation of the disease. AATD‐related panniculitis may have none of the typical clinical clues for AATD, such as a family history, cirrhosis or emphysema. Early identification may help prevent these complications from developing.</description><identifier>ISSN: 0307-6938</identifier><identifier>EISSN: 1365-2230</identifier><identifier>DOI: 10.1111/ced.13102</identifier><identifier>PMID: 28512995</identifier><language>eng</language><publisher>England: Oxford University Press</publisher><subject>Adult ; alpha 1-Antitrypsin - therapeutic use ; alpha 1-Antitrypsin Deficiency - complications ; Cirrhosis ; Colchicine ; Colchicine - therapeutic use ; Corticosteroids ; Dapsone - therapeutic use ; Emphysema ; Female ; Humans ; Infusions, Intravenous ; Leg ; Liver cirrhosis ; Nodules ; Panniculitis - drug therapy ; Panniculitis - etiology ; Panniculitis - pathology ; Tubulin Modulators - therapeutic use ; Ulcers ; Young Adult</subject><ispartof>Clinical and experimental dermatology, 2017-07, Vol.42 (5), p.520-522</ispartof><rights>2017 British Association of Dermatologists</rights><rights>2017 British Association of Dermatologists.</rights><rights>Copyright © 2017 British Association of Dermatologists</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c3132-1c7cca916000a1d989038bd879c00161a89e5a44f290379e117ee61c78ad1c153</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/28512995$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Storan, E. R.</creatorcontrib><creatorcontrib>O' Gorman, S. M.</creatorcontrib><creatorcontrib>Hawkins, P.</creatorcontrib><creatorcontrib>Aalto, L.</creatorcontrib><creatorcontrib>Murphy, A.</creatorcontrib><creatorcontrib>Markham, T.</creatorcontrib><title>Alpha‐1‐antitrypsin deficiency‐related panniculitis: two cases with diverse clinical courses</title><title>Clinical and experimental dermatology</title><addtitle>Clin Exp Dermatol</addtitle><description>Summary
Alpha‐1‐antitrypsin deficiency (AATD)‐related panniculitis is an extremely rare and underdiagnosed entity, and there is a paucity of data on its treatment. We report two cases of AATD‐related panniculitis. The first was a 24‐year‐old woman with known AATD who presented with painful leg ulcers refractory to treatment with corticosteroids and colchicine. She had a good response to α1‐antitrypsin infusions but required dose adjustment due to flares in disease activity. The second case was a 38‐year‐old woman who presented with painful nodules on the legs refractory to corticosteroid therapy. Laboratory investigations revealed severe AATD. She had an excellent response to colchicine therapy. In both these cases of AATD, panniculitis was the first clinical manifestation of the disease. AATD‐related panniculitis may have none of the typical clinical clues for AATD, such as a family history, cirrhosis or emphysema. Early identification may help prevent these complications from developing.</description><subject>Adult</subject><subject>alpha 1-Antitrypsin - therapeutic use</subject><subject>alpha 1-Antitrypsin Deficiency - complications</subject><subject>Cirrhosis</subject><subject>Colchicine</subject><subject>Colchicine - therapeutic use</subject><subject>Corticosteroids</subject><subject>Dapsone - therapeutic use</subject><subject>Emphysema</subject><subject>Female</subject><subject>Humans</subject><subject>Infusions, Intravenous</subject><subject>Leg</subject><subject>Liver cirrhosis</subject><subject>Nodules</subject><subject>Panniculitis - drug therapy</subject><subject>Panniculitis - etiology</subject><subject>Panniculitis - pathology</subject><subject>Tubulin Modulators - therapeutic use</subject><subject>Ulcers</subject><subject>Young Adult</subject><issn>0307-6938</issn><issn>1365-2230</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2017</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kM1KAzEURoMotlYXvoAMuNHF2NxJZzJxJ_UXCm50HdLMLU1JZ8bJjKU7H8Fn9Em82nYjGAghuScfH4exU-BXQGtosbgCATzZY30QWRonieD7rM8Fl3GmRN5jRyEsOCdIpoesl-QpJEqlfTa98fXcfH18Am1Ttq5t1nVwZVTgzFmHpV3ToEFvWiyi2pSls513rQvXUbuqImsChmjl2nlUuHdsAkbWO4KMj2zV0T0cs4OZ8QFPtueAvd7fvYwf48nzw9P4ZhJbASKJwUprjYKMc26gULniIp8WuVSWimdgcoWpGY1mCQ2kQgCJmNGv3BRgIRUDdrHJrZvqrcPQ6qULFr03JVZd0JArJRVX6YjQ8z_ogrqW1E6DIo9KyjQh6nJD2aYKocGZrhu3NM1aA9c_4jWJ17_iiT3bJnbTJb3uyJ1pAoYbYOU8rv9P0uO7203kN3MpjsY</recordid><startdate>201707</startdate><enddate>201707</enddate><creator>Storan, E. R.</creator><creator>O' Gorman, S. M.</creator><creator>Hawkins, P.</creator><creator>Aalto, L.</creator><creator>Murphy, A.</creator><creator>Markham, T.</creator><general>Oxford University Press</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>K9.</scope><scope>7X8</scope></search><sort><creationdate>201707</creationdate><title>Alpha‐1‐antitrypsin deficiency‐related panniculitis: two cases with diverse clinical courses</title><author>Storan, E. R. ; O' Gorman, S. M. ; Hawkins, P. ; Aalto, L. ; Murphy, A. ; Markham, T.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3132-1c7cca916000a1d989038bd879c00161a89e5a44f290379e117ee61c78ad1c153</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2017</creationdate><topic>Adult</topic><topic>alpha 1-Antitrypsin - therapeutic use</topic><topic>alpha 1-Antitrypsin Deficiency - complications</topic><topic>Cirrhosis</topic><topic>Colchicine</topic><topic>Colchicine - therapeutic use</topic><topic>Corticosteroids</topic><topic>Dapsone - therapeutic use</topic><topic>Emphysema</topic><topic>Female</topic><topic>Humans</topic><topic>Infusions, Intravenous</topic><topic>Leg</topic><topic>Liver cirrhosis</topic><topic>Nodules</topic><topic>Panniculitis - drug therapy</topic><topic>Panniculitis - etiology</topic><topic>Panniculitis - pathology</topic><topic>Tubulin Modulators - therapeutic use</topic><topic>Ulcers</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Storan, E. R.</creatorcontrib><creatorcontrib>O' Gorman, S. M.</creatorcontrib><creatorcontrib>Hawkins, P.</creatorcontrib><creatorcontrib>Aalto, L.</creatorcontrib><creatorcontrib>Murphy, A.</creatorcontrib><creatorcontrib>Markham, T.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>MEDLINE - Academic</collection><jtitle>Clinical and experimental dermatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Storan, E. R.</au><au>O' Gorman, S. M.</au><au>Hawkins, P.</au><au>Aalto, L.</au><au>Murphy, A.</au><au>Markham, T.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Alpha‐1‐antitrypsin deficiency‐related panniculitis: two cases with diverse clinical courses</atitle><jtitle>Clinical and experimental dermatology</jtitle><addtitle>Clin Exp Dermatol</addtitle><date>2017-07</date><risdate>2017</risdate><volume>42</volume><issue>5</issue><spage>520</spage><epage>522</epage><pages>520-522</pages><issn>0307-6938</issn><eissn>1365-2230</eissn><abstract>Summary
Alpha‐1‐antitrypsin deficiency (AATD)‐related panniculitis is an extremely rare and underdiagnosed entity, and there is a paucity of data on its treatment. We report two cases of AATD‐related panniculitis. The first was a 24‐year‐old woman with known AATD who presented with painful leg ulcers refractory to treatment with corticosteroids and colchicine. She had a good response to α1‐antitrypsin infusions but required dose adjustment due to flares in disease activity. The second case was a 38‐year‐old woman who presented with painful nodules on the legs refractory to corticosteroid therapy. Laboratory investigations revealed severe AATD. She had an excellent response to colchicine therapy. In both these cases of AATD, panniculitis was the first clinical manifestation of the disease. AATD‐related panniculitis may have none of the typical clinical clues for AATD, such as a family history, cirrhosis or emphysema. Early identification may help prevent these complications from developing.</abstract><cop>England</cop><pub>Oxford University Press</pub><pmid>28512995</pmid><doi>10.1111/ced.13102</doi><tpages>3</tpages></addata></record> |
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| source | Oxford University Press Journals All Titles (1996-Current); MEDLINE; Alma/SFX Local Collection |
| subjects | Adult alpha 1-Antitrypsin - therapeutic use alpha 1-Antitrypsin Deficiency - complications Cirrhosis Colchicine Colchicine - therapeutic use Corticosteroids Dapsone - therapeutic use Emphysema Female Humans Infusions, Intravenous Leg Liver cirrhosis Nodules Panniculitis - drug therapy Panniculitis - etiology Panniculitis - pathology Tubulin Modulators - therapeutic use Ulcers Young Adult |
| title | Alpha‐1‐antitrypsin deficiency‐related panniculitis: two cases with diverse clinical courses |
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