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==Overview==
==Overview==
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==History==
==History==

Revision as of 16:51, 27 August 2012

Porphyria Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

History

Symptoms

Acute porphyria

The cutaneous sensitivity to light is due to excitation of accumulated porphyrins in the skin by UV light. This leads to progressive damage, scarring and deformation.

The “ Acute porphyrias ”: AIP, VP, CP and ALA-dehydratase deficiency are characterized by acute attacks . The acute attack is characterized by:

The hepatic porphyrias primarily affect the nervous system, resulting in abdominal pain, vomiting, acute neuropathy, seizures and mental disturbances, including hallucinations, depression, anxiety and paranoia. Pain can be severe and can, in some cases, be both acute and chronic in nature. Constipation is frequently present, as the nervous system of the gut is affected, but diarrhea can also occur.

Given the many presentations and the relatively uncommon occurrence of porphyria the patient may initially be suspected to have other, unrelated conditions. For instance, the polyneuropathy of acute porphyria may be mistaken for Guillain-Barré syndrome, and porphyria testing is commonly recommended in those scenarios.[1] Lupus erythematosus features photosensitivity, pain attacks and shares various other symptoms with porphyria.[2]

Cutaneous porphyria

The erythropoietic porphyrias primarily affect the skin, causing photosensitivity (photodermatitis), blisters, necrosis of the skin and gums, itching, and swelling, and increased hair growth on areas such as the forehead. Often there is no abdominal pain which distinguishes it from other porphyrias.

In some forms of porphyria, accumulated heme precursors excreted in the urine may cause various changes in color, after exposure to sunlight, to a dark reddish or dark brown color. Even a purple hue or pink urine may be seen. Heme precursors may also accumulate in the teeth and fingernails, giving them a reddish appearance.

References

  1. Albers JW, Fink JK. Porphyric neuropathy. Muscle Nerve 2004;30:410-22. PMID 15372536.
  2. Roelandts R. The diagnosis of photosensitivity. Arch Dermatol 2000;136:1152-7. PMID 10987875.

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