{"id":486,"date":"2007-06-07T18:56:47","date_gmt":"2007-06-07T22:56:47","guid":{"rendered":"http:\/\/piel-l.org\/blog\/?p=486"},"modified":"2007-06-09T10:44:02","modified_gmt":"2007-06-09T14:44:02","slug":"enfermedad-de-hansen","status":"publish","type":"post","link":"https:\/\/piel-l.org\/blog\/486","title":{"rendered":"Enfermedad de Hansen"},"content":{"rendered":"<p>Leprosy as immune reconstitution disease has been reported in patients receiving antiretroviral therapy for HIV infection in countries where leprosy is endemic (BMJ 2007 Feb 3)<\/p>\n<p> Infliximab reported to be effective for recurrent erythema nodosum leprosum (N Engl J Med 2006 Aug 17)<br \/> Description: mycobacterial infection, leprosy is also called Hansen&#39;s disease (Hansen discovered M. leprae)<\/p>\n<p> <!--more--> <\/p>\n<p>Organs Involved: skin and peripheral nerves<\/p>\n<p>Causes: Mycobacterium leprae and deficient cell-mediated immunity<\/p>\n<p>Pathogenesis:<\/p>\n<ul>\n<li>M. leprae is psychrophilic, acid-fast      bacillus (AFB) with high lipid content, very slow growing<\/li>\n<li>transmission via prolonged contact with      respiratory secretions + skin lesions<\/li>\n<li>incubation period 2-6 years<\/li>\n<\/ul>\n<p>Likely risk factors:<\/p>\n<ul>\n<li>recent immigrants<\/li>\n<li>endemic focus of Hansen&#39;s disease exists      in Texas along Gulf       of Mexico coast (<a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?db=pubmed&amp;cmd=Retrieve&amp;dopt=Abstract&amp;list_uids=10466975\" target=\"_blank\">Am J Trop      Med Hyg. 1999 Mar;60(3):449<\/a>      <a href=\"http:\/\/www.ajtmh.org\/cgi\/reprint\/60\/3\/449\" target=\"_blank\">PDF<\/a> in JAMA 1999 Jun 23\/30;281(24):2270f)<\/li>\n<\/ul>\n<p>Possible risk factors:<\/p>\n<ul>\n<li>leprosy as immune reconstitution disease      has been reported in patients receiving antiretroviral therapy for HIV infection      in countries where leprosy is endemic (<a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?db=pubmed&amp;cmd=Retrieve&amp;dopt=Abstract&amp;list_uids=17272530\" target=\"_blank\">BMJ 2007      Feb 3;334(7587):217<\/a>)<\/li>\n<\/ul>\n<p>Complications:<\/p>\n<ul>\n<li>trauma due to sensory loss<\/li>\n<li>chronic neuropathic pain described in 16      patients with multibacillary leprosy (<a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?db=pubmed&amp;cmd=Retrieve&amp;dopt=Abstract&amp;list_uids=11009148\" target=\"_blank\">Lancet      2000 Sep 23;356(9235):1080<\/a> &nbsp;, commentary can be found in <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?db=pubmed&amp;cmd=Retrieve&amp;dopt=Abstract&amp;list_uids=11214161\" target=\"_blank\">Lancet      2001 Jan 27;357(9252):313<\/a> &nbsp;<\/li>\n<li>&nbsp;<\/li>\n<\/ul>\n<p>Associated conditions: erythema nodosum leprosum (ENL, type II Lepra reaction) develops in 10-50% patients treated for lepromatous leprosy; reactive syndrome with painful skin nodules, fever, malaise, wasting, vasculitis and peripheral neuritis; use of thalidomide for severe or recurrent ENL is associated with rapid relief of symptoms and signs and a decrease in elevated TNF-alpha (<a href=\"http:\/\/www.themedicalletter.com\/scripts\/toc.cgi?type=1996&amp;inst=null\" target=\"_blank\">The Medical Letter 1996<\/a> Feb 16;38(968 ):15)<\/p>\n<p>General Physical: possibly lymphadenopathy<\/p>\n<p>Skin:<\/p>\n<p>multiple nodular skin lesions<\/p>\n<ul>\n<li>differential diagnosis of annular lesions      includes<\/li>\n<ul>\n<li><a href=\"http:\/\/dynamed102.ebscohost.com\/Detail.aspx?style=1&amp;docid=\/dynamed\/0707784c9ddeabe88525639800649074\">tinea       corporis<\/a><\/li>\n<li><a href=\"http:\/\/dynamed102.ebscohost.com\/Detail.aspx?style=1&amp;docid=\/dynamed\/b4c9fffec8d5e04b852562890052e017\">pityriasis       rosea<\/a><\/li>\n<li>granuloma annulare<\/li>\n<li><a href=\"http:\/\/dynamed102.ebscohost.com\/Detail.aspx?style=1&amp;docid=\/dynamed\/f1d56fd17b5cbd38852565da0081c50d\">sarcoidosis<\/a><\/li>\n<li>Hansen&#39;s disease (<a href=\"http:\/\/dynamed102.ebscohost.com\/Detail.aspx?style=1&amp;docid=\/dynamed\/4a8168277b77a30286256baf007c3b6f\">leprosy<\/a>)<\/li>\n<li><a href=\"http:\/\/dynamed102.ebscohost.com\/Detail.aspx?style=1&amp;docid=\/dynamed\/7e5aa39b2ab9d4138525628f00175477\">urticaria<\/a><\/li>\n<li>subacute cutaneous <a href=\"http:\/\/dynamed102.ebscohost.com\/Detail.aspx?style=1&amp;docid=\/dynamed\/ae1790d64ff7648e86256be0007369de\">lupus       erythematosus<\/a><\/li>\n<li>erythema annulare centrifugum (case       presentation and discussion in <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?db=pubmed&amp;cmd=Retrieve&amp;dopt=Abstract&amp;list_uids=15689287\" target=\"_blank\">J Fam       Pract 2005 Feb;54(2):131<\/a> &nbsp;picture in <a href=\"http:\/\/www.aafp.org\/afp\/20050815\/photo.html\" target=\"_blank\">Am Fam Physician 2005 Aug       15;72(4):671<\/a>)<\/li>\n<li>erythema chronicum migrans (<a href=\"http:\/\/dynamed102.ebscohost.com\/Detail.aspx?style=1&amp;docid=\/dynamed\/38e325f21f134dd5852562c6000f8eaa\">Lyme disease<\/a>)<\/li>\n<li><a href=\"http:\/\/dynamed102.ebscohost.com\/Detail.aspx?style=1&amp;docid=\/dynamed\/4274bd82a2c403de852562890052e008\">erythema       multiforme<\/a><\/li>\n<li><a href=\"http:\/\/dynamed102.ebscohost.com\/Detail.aspx?style=1&amp;docid=\/dynamed\/ee6832d000195697852562890052e025\">psoriasis<\/a><\/li>\n<li><a href=\"http:\/\/dynamed102.ebscohost.com\/Detail.aspx?style=1&amp;docid=\/dynamed\/2d18a8fe6108f2f8852562890052e011\">nummular       eczema<\/a><\/li>\n<li>Reference &#8211; <a href=\"http:\/\/www.aafp.org\/afp\/20010715\/289.html\" target=\"_blank\">Am Fam Physician 2001 Jul       15;64(2):289<\/a><\/li>\n<\/ul>\n<\/ul>\n<p>Testing to consider:<\/p>\n<p>skin biopsy of lesions or nasal scrapings<\/p>\n<ul>\n<li>acid-fast bacilli (AFB) (Z-N\/Fite) smear      and histology<\/li>\n<li>Mycobacterium leprae      polymerase chain reaction (PCR)<\/li>\n<\/ul>\n<p>Blood tests: false positive for syphilis<\/p>\n<p>Pathology tests:<\/p>\n<ul>\n<li>many AFB, foamy histiocytes<\/li>\n<li>picture can be found in <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?db=pubmed&amp;cmd=Retrieve&amp;dopt=Abstract&amp;list_uids=11320388\" target=\"_blank\">N Engl J      Med 2001 Apr 26;344(17):1293<\/a><\/li>\n<\/ul>\n<p>Other diagnostic testing: negative lepromin skin test<\/p>\n<p>Prognosis: <strong>multibacillary leprosy and nerve-function loss predict nerve-function impairment after 6 months of chemotherapy<\/strong><\/p>\n<ul>\n<ul>\n<li>prospective study of 2510 patients with       new diagnosis of leprosy followed for 2 years<\/li>\n<li>166 developed       nerve-function impairment<\/li>\n<li>risk of nerve-function impairment was<\/li>\n<ul>\n<li>65% (95% confidence interval 56-73%) for        patients with multibacillary leprosy and nerve-function loss at baseline<\/li>\n<li>1.3% (95% CI 0.8-1.8%) for patients with        paucibacillary leprosy and no nerve-function loss at baseline<\/li>\n<li>16% (95% CI 12-20%) for patients with 1        of these 2 risk factors<\/li>\n<\/ul>\n<li>Reference &#8211; <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?db=pubmed&amp;cmd=Retrieve&amp;dopt=Abstract&amp;list_uids=10821364\" target=\"_blank\">Lancet       2000 May 6;355(9215):1603<\/a> , commentary can be found in <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?db=pubmed&amp;cmd=Retrieve&amp;dopt=Abstract&amp;list_uids=11095280\" target=\"_blank\">Lancet       2000 Nov 18;356(9243):1767<\/a>&nbsp;<\/li>\n<\/ul>\n<\/ul>\n<p>Medications:<\/p>\n<ul>\n<li>recommended treatment of      Hansen&#39;s disease<\/li>\n<ul>\n<li>treatment of       paucibacillary disease<\/li>\n<ul>\n<li>rifampin (Rifadin) 600 mg monthly and        dapsone 100 mg daily for 6 months (recommended by World Health        Organization [WHO])<\/li>\n<li>rifampin 600 mg and daily and dapsone        100 mg daily for 12 months (has been recommended in United States)<\/li>\n<\/ul>\n<li>treatment of       multibacillary disease<\/li>\n<ul>\n<li>rifampin 600 mg monthly, clofazimine        (Lamprene) 300 mg monthly, dapsone 100 mg daily and clofazimine 50 mg        daily for 12 months (recommended by WHO)<\/li>\n<li>rifampin 600 mg, dapsone 100 mg and        clofazimine 50 mg daily for 2 years (has been recommended in Unites        States, or use WHO regimen)<\/li>\n<\/ul>\n<li>single doses of rimpanin, ofloxacin       (Floxin) and minocycline (Minocin) may be adequate for single skin       lesions in paucibacillary leprosy<\/li>\n<li>Reference &#8211; <a href=\"http:\/\/www.aafp.org\/afp\/20020401\/letters.html\" target=\"_blank\">Am       Fam Physician 2002 Apr 1;66(7):1280<\/a><\/li>\n<\/ul>\n<li>antibiotic selection<\/li>\n<ul>\n<li>drug of choice &#8211; dapsone plus rifampin       with or without clofazimine<\/li>\n<li>alternatives &#8211;       minocycline, ofloxacin, clarithromycin<\/li>\n<li>Reference &#8211; <a href=\"http:\/\/www.themedicalletter.com\/scripts\/toc.cgi?type=tg04&amp;inst=null\" target=\"_blank\">Treatment       Guidelines from The Medical Letter 2004<\/a> Mar;2(9):13<\/li>\n<\/ul>\n<li>adverse effects of dapsone<\/li>\n<ul>\n<li>frequent side effects &#8211; rash, transient       headache, GI irritation, anorexia, infectious mononucleosis-like syndrome<\/li>\n<li>occasional side effects &#8211; cyanosis due to       methemoglobinemia and sulfhemoglobinemia, other blood dyscrasias       including hemolytic anemia, nephrotic syndrome, liver damage, peripheral       neuropathy, hypersensitivity reactions, increased risk of lepra       reactions, insomnia, irritability, uncoordinated speech, agitation, acute       psychosis<\/li>\n<li>rare side effects &#8211; renal papillary       necrosis, severe hypoalbuminemia, epidermal necrolysis, optic atrophy,       agranulocytosis, neonatal hyperbilirubinemia after use in pregnancy<\/li>\n<li>case report of dapsone hypersensitivity       syndrome can be found in <a href=\"http:\/\/www.occup-med.com\/content\/1\/1\/9\" target=\"_blank\">Journal of Occupational       Medicine and Toxicology 2006 Jun 6;1:9 <\/a><\/li>\n<\/ul>\n<li>thalidomide<\/li>\n<ul>\n<li>thalidomide has shown efficacy in       thousands of leprosy patients with painful sores, FDA approval expected       with strictest controls in history such as signed informed consent,       provision of pregnancy testing + birth control information, + possibly       even picture of birth-deformed child on packaging (Cortlandt Forum 1997       Nov;10(11);79)<\/li>\n<\/ul>\n<\/ul>\n<p>thalidomide (Thalomid) FDA approved for erythema nodosum leprosum, immune reaction to dead bacteria after treatment of leprosy, call 732-271-1001 for details (Monthly Prescribing<\/p>\n<ul>\n<ul>\n<li>Reference 1998 Sep;A-23)<\/li>\n<li>thalidomide (Thalomid) 50 mg costs       pharmacist $630 for 84 capsules, special registration of physician +       pharmacists required for prescription; adverse effects include       teratogenicity even with small doses, peripheral neuropathy (may be       irreversible), sedation, constipation, orthostatic hypotension, dry       mouth, dry skin, rash, edema, hypothyroidism, neutropenia (<a href=\"http:\/\/www.themedicalletter.com\/scripts\/toc.cgi?type=1998&amp;inst=null\" target=\"_blank\">The       Medical Letter 1998<\/a> Oct 23;40(1038):103)<\/li>\n<\/ul>\n<li><strong>low dose prednisolone during first 4      months of multidrug treatment for leprosy reduces short-term incidence of      new reactions and nerve function impairment (<a href=\"http:\/\/www.epnet.com\/dynamed\/levels.php\" target=\"_blank\">l<\/a><\/strong><strong>)<\/strong><\/li>\n<ul>\n<li>636 patients aged 15-50 years with newly       diagnosed multibacillary leprosy treated with multidrug treatment for 1       year and randomized at onset to prednisolone (20 mg\/day for 3 months then       tapered over 1 month) vs. placebo<\/li>\n<li>90% follow-up rates and intent to treat       analysis<\/li>\n<li>comparing prednisolone       vs. placebo<\/li>\n<ul>\n<li>4% vs. 15% had skin or nerve reaction at        4 months (NNT 9)<\/li>\n<li>effect not sustained at 1 year when 22%        vs. 17% had skin or nerve reaction (not significant)<\/li>\n<\/ul>\n<li>prednisolone only effective in patients       without pre-existing nerve function impairment<\/li>\n<li>Reference &#8211; <a href=\"http:\/\/bmj.bmjjournals.com\/cgi\/content\/full\/328\/7454\/1459\" target=\"_blank\">BMJ 2004       Jun 19;328(7454):1459<\/a>, editorial can be found in <a href=\"http:\/\/bmj.bmjjournals.com\/cgi\/content\/full\/328\/7454\/1447\" target=\"_blank\">BMJ 2004       Jun 19;328(7454):1447<\/a><\/li>\n<li>non-significant increased risk at 1 year       suggests that prednisolone may delay instead of prevent reactions       (DynaMed commentary)<\/li>\n<\/ul>\n<li>infliximab reported to be effective for      recurrent erythema nodosum leprosum in case report (<a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?db=pubmed&amp;cmd=Retrieve&amp;dopt=Abstract&amp;list_uids=16914716\" target=\"_blank\">N Engl J      Med 2006 Aug 17;355(7):739<\/a>)<\/li>\n<\/ul>\n<p>Prevention: dapsone for close family contacts<\/p>\n<p><a href=\"javascript:changeView(%22References_including_Reviews_and_Guidelines%22);\">References including Reviews and Guidelines<\/a><\/p>\n<p>Reviews:<\/p>\n<ul>\n<li>discussion of leprosy can be found in      Lancet 1999 Feb 20;353(9153):655<\/li>\n<li>review of leprosy can be found in <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?cmd=retrieve&amp;db=pubmed&amp;list_uids=14707226&amp;dopt=Abstract\" target=\"_blank\">CMAJ 2004      Jan 6;170(1):71<\/a>      &nbsp;<\/li>\n<li>review of leprosy can be found in <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?cmd=Retrieve&amp;db=pubmed&amp;dopt=Abstract&amp;list_uids=15081655\" target=\"_blank\">Lancet      2004 Apr 10;363(9416):1209<\/a> &nbsp;commentary can be found in Lancet 2004 Jun 5;363(9424):1911<\/li>\n<li>cohort of 184 leprosy patients in Canada      described in <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?cmd=retrieve&amp;db=pubmed&amp;list_uids=14707220&amp;dopt=Abstract\" target=\"_blank\">CMAJ 2004      Jan 6;170(1):55<\/a>      &nbsp;case presentation and discussion can be found in Postgrad Med 2000      May 1;107(5):87<\/li>\n<li>case presentation can be found in <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?cmd=Retrieve&amp;db=pubmed&amp;dopt=Abstract&amp;list_uids=16650656\" target=\"_blank\">Lancet      2006 Apr 29;367(9520):1458<\/a> &nbsp;case presentation can be found in <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?db=pubmed&amp;cmd=Retrieve&amp;dopt=Abstract&amp;list_uids=14711916\" target=\"_blank\">N Engl J      Med. 2004 Jan 8;350(2):166<\/a><\/li>\n<li>review of antimycobacterial therapy can be      found in <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?cmd=Retrieve&amp;db=PubMed&amp;list_uids=10918872&amp;dopt=Abstract\" target=\"_blank\">Mayo Clin      Proc 1999 Oct;74(10):1038<\/a> (summary can be found in <a href=\"http:\/\/www.aafp.org\/afp\/20000201\/tips\/25.html\" target=\"_blank\">Am Fam Physician 2000 Feb      1;61(3):861<\/a>)<\/li>\n<\/ul>\n<p>Authors:<\/p>\n<p> Brian S. Alper MD, MSPH (Editor-in-Chief, DynaMed, Ipswich, Massachusetts,  USA)<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Leprosy as immune reconstitution disease has been reported in patients receiving antiretroviral therapy for HIV infection in countries where leprosy is endemic (BMJ 2007 Feb 3) Infliximab reported to be effective for recurrent erythema nodosum leprosum (N Engl J Med 2006 Aug 17) Description: mycobacterial infection, leprosy is also called Hansen&#39;s disease (Hansen discovered M. &hellip;<\/p>\n","protected":false},"author":16,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[8],"tags":[],"class_list":["post-486","post","type-post","status-publish","format-standard","","category-articulos-cientificos"],"_links":{"self":[{"href":"https:\/\/piel-l.org\/blog\/wp-json\/wp\/v2\/posts\/486","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/piel-l.org\/blog\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/piel-l.org\/blog\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/piel-l.org\/blog\/wp-json\/wp\/v2\/users\/16"}],"replies":[{"embeddable":true,"href":"https:\/\/piel-l.org\/blog\/wp-json\/wp\/v2\/comments?post=486"}],"version-history":[{"count":0,"href":"https:\/\/piel-l.org\/blog\/wp-json\/wp\/v2\/posts\/486\/revisions"}],"wp:attachment":[{"href":"https:\/\/piel-l.org\/blog\/wp-json\/wp\/v2\/media?parent=486"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/piel-l.org\/blog\/wp-json\/wp\/v2\/categories?post=486"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/piel-l.org\/blog\/wp-json\/wp\/v2\/tags?post=486"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}