https://idwiki.org/index.php?action=history&feed=atom&title=Porphyria_cutanea_tarda 2026-05-13T00:16:36Z Revision history for this page on the wiki MediaWiki 1.43.8 https://idwiki.org/index.php?title=Porphyria_cutanea_tarda&diff=5096&oldid=prev 2020-07-20T14:19:35Z

<p>Text replacement - &quot;Clinical Presentation&quot; to &quot;Clinical Manifestations&quot;</p> <table style="background-color: #fff; color: #202122;" data-mw="interface"> <col class="diff-marker" /> <col class="diff-content" /> <col class="diff-marker" /> <col class="diff-content" /> <tr class="diff-title" lang="en"> <td colspan="2" style="background-color: #fff; color: #202122; text-align: center;">← Older revision</td> <td colspan="2" style="background-color: #fff; color: #202122; text-align: center;">Revision as of 14:19, 20 July 2020</td> </tr><tr> <td colspan="2" class="diff-lineno">Line 8:</td> <td colspan="2" class="diff-lineno">Line 8:</td> </tr> <tr> <td class="diff-marker"></td> <td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div>* A defect in uroporphyrinogen decarboxylase, which normally converts uroporphyrinogen I and III to coproporphyrinogen I and III</div></td> <td class="diff-marker"></td> <td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div>* A defect in uroporphyrinogen decarboxylase, which normally converts uroporphyrinogen I and III to coproporphyrinogen I and III</div></td> </tr> <tr> <td class="diff-marker"></td> <td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><br /></td> <td class="diff-marker"></td> <td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><br /></td> </tr> <tr> <td class="diff-marker" data-marker="−"></td> <td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #ffe49c; vertical-align: top; white-space: pre-wrap;"><div>== Clinical <del style="font-weight: bold; text-decoration: none;">Presentation</del> ==</div></td> <td class="diff-marker" data-marker="+"></td> <td style="color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #a3d3ff; vertical-align: top; white-space: pre-wrap;"><div>== Clinical <ins style="font-weight: bold; text-decoration: none;">Manifestations</ins> ==</div></td> </tr> <tr> <td class="diff-marker"></td> <td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><br /></td> <td class="diff-marker"></td> <td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><br /></td> </tr> <tr> <td class="diff-marker"></td> <td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div>* Blistering skin lesions on the back of the hands</div></td> <td class="diff-marker"></td> <td style="background-color: #f8f9fa; color: #202122; font-size: 88%; border-style: solid; border-width: 1px 1px 1px 4px; border-radius: 0.33em; border-color: #eaecf0; vertical-align: top; white-space: pre-wrap;"><div>* Blistering skin lesions on the back of the hands</div></td> </tr> <!-- diff cache key site7_mediawiki:diff:1.41:old-4413:rev-5096:wikidiff2=table:1.13.0:bc2a06be --> </table>

Aidan https://idwiki.org/index.php?title=Porphyria_cutanea_tarda&diff=4413&oldid=prev 2020-07-04T01:17:51Z

<p>Imported from text file</p> <p><b>New page</b></p><div>== Definition ==<br /> <br /> * A defect in the heme synthesis pathway that causes cutaneous manifestations in adults<br /> * Either type 1 (sporadic) or type 2 (familial)<br /> <br /> == Pathophysiology ==<br /> <br /> * A defect in uroporphyrinogen decarboxylase, which normally converts uroporphyrinogen I and III to coproporphyrinogen I and III<br /> <br /> == Clinical Presentation ==<br /> <br /> * Blistering skin lesions on the back of the hands<br /> ** They rupture and crust over, leaving atrophy and scarring<br /> ** Scarring and calcification can look like sclerosis<br /> * Lesions may appear on forearms, face, legs, and feet<br /> * May have hyperpigmentation and hypertrichosis of the face<br /> * No neurological manifestations<br /> * Often have liver disease, and are higher risk for hepatocellular carcinoma<br /> <br /> == Investigations ==<br /> <br /> * Porphyrins in liver, plasma, urine, or stool are elevated<br /> ** Urinary porphyrins include uroporphyrins and heptacarboxylate porphyrin<br /> * Urinary ALA level may be elevated<br /> * PBG level is normal<br /> <br /> == Management ==<br /> <br /> * Avoid alcohol<br /> * Stop estrogen supplements, iron supplements, and any other drugs that may be exacerbating<br /> * Repeated phlebotomy to reduce hepatic iron stores<br /> ** Remove 1 unit every 1 to 2 weeks<br /> ** Remission may require more than six phlebotomies<br /> ** Follow the serum porphyrin levels every 6 to 12 months; they should decrease<br /> * Hydroxychloroquine is second-line<br /> * Liver imaging to screen for hepatocellular carcinoma<br /> <br /> [[Category:Hematology]]<br /> [[Category:Dermatology]]</div>

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