{"id":7631,"date":"2013-03-04T11:41:31","date_gmt":"2013-03-04T16:41:31","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=7631"},"modified":"2013-03-08T16:48:33","modified_gmt":"2013-03-08T21:48:33","slug":"pemphigus-vulgaris","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2013\/03\/pemphigus-vulgaris\/","title":{"rendered":"Pemphigus Vulgaris"},"content":{"rendered":"

Pathophysiology of pemphigus vulgaris<\/h4>\n

1)<\/strong> pemphigus vulgaris is an autoimmune bullous skin disease resulting from formation of a pemphigus antibody against desmoglein 1 an 3 molecules (component of desmosomes located between keratinocytes)
\n2)<\/strong> type 2 hypersensitivity reaction<\/p>\n

<\/p>\n

Signs and Symptoms<\/h4>\n

1)<\/strong> flaccid blisters in mouth, face, scalp, neck, axilla, and torso
\n2)<\/strong> itching and pain at site of lesions
\n3)<\/strong> skin becomes denuded
\n4)<\/strong> posthealing hyperpigmentation
\n5)<\/strong> blisters can ulcerate and become secondarily infected
\n6)<\/strong> severe cachexia
\n7)<\/strong> positive Nikolsky’s sign<\/p>\n

\"pemphigus<\/a>

Flaccid blisters seen in pemphigus vulgaris<\/p><\/div>\n

Characteristic Test Findings<\/h4>\n

IgG autoantibodies against epithelial cell desmogleins (1 and 3)<\/p>\n

Histology and Gross Pathology<\/h4>\n

1)<\/strong> epidermal cells lose cohesion (acantholysis) with formation of intraepidermal blisters
\n2)<\/strong> intact basal cells form blister base<\/p>\n

Associated Conditions<\/h4>\n

1)<\/strong> increased incidence in HLA-DR4 and HLA-DRw6
\n2)<\/strong> lesions can also occur in esophagus, pharynx, larynx, conjunctiva, rectum, and vagina<\/p>\n

Inheritance\/Epidemiology<\/h4>\n

occurs mostly in elderly patients<\/p>\n

Treatment<\/h4>\n

1)<\/strong> glucocorticoids (usually prednisone)
\n2)<\/strong> azathioprine or
cyclophosphamide<\/a><\/p>\n

Tips for USMLE<\/h4>\n

1)<\/strong> Nikolsky’s sign is separation of epidermis when skin is manually pressed
\n2)<\/strong> if there are no mouth lesions, it is not pemphigus vulgaris
\n3)<\/strong> if question mentions denuded skin and flaccid blisters, think pemphigus vulgaris
\n4)<\/strong> if question mentions intraepidermal blisters, think pemphigus vulgaris<\/p>\n","protected":false},"excerpt":{"rendered":"

Pathophysiology of pemphigus vulgaris 1) pemphigus vulgaris is an autoimmune bullous skin disease resulting from formation of a pemphigus antibody against desmoglein 1 an 3 molecules (component of desmosomes located between keratinocytes) 2) type 2 hypersensitivity reaction<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"_genesis_hide_title":false,"_genesis_hide_breadcrumbs":false,"_genesis_hide_singular_image":false,"_genesis_hide_footer_widgets":false,"_genesis_custom_body_class":"","_genesis_custom_post_class":"","_genesis_layout":"","footnotes":""},"categories":[5151,11],"tags":[5424,5436,5438,5435,5437],"yoast_head":"\nPemphigus Vulgaris - InsideSurgery Medical Information Blog<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/insidesurgery.com\/2013\/03\/pemphigus-vulgaris\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Pemphigus Vulgaris - 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