Abstract
Patients with porphyria may have cutaneous or neurologic symptoms or both. Cutaneous signs include vesiculobullous skin eruptions, crusted ulcers, scarring, and hyperpigmentation in sun-exposed areas. Fragile skin also develops. Patients with neuropathic porphyrias often have episodic attacks of abdominal pain with variable or no symptoms between attacks. Some may have chronic abdominal pain with a few acute attacks or long periods of remission punctuated by acute attacks. The acute attacks usually last for several days. Most attacks start after pubescence. Hypertension, hyponatremia from vomiting, and seizures can occur.
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Reference
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Suggested Reading
Kappas A, Sassa S, Galbraith RA, Nordmann Y. The porphyrias, in The Metabolic Basis ofInherited Disease, 6th ed. (Scriver CR, Beaudet AL, Sly WS, Valle D, eds). McGraw-Hill, New York, 1989, pp. 1305–1365.
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Solberg, L.A. (2001). An Approach to Porphyria. In: Tefferi, A. (eds) Primary Hematology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-228-9_28
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DOI: https://doi.org/10.1007/978-1-59259-228-9_28
Publisher Name: Humana Press, Totowa, NJ
Print ISBN: 978-1-61737-122-6
Online ISBN: 978-1-59259-228-9
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