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Picture of the Month
Avinash K. Shetty, MD;
David Ode, MD;
Wesley Galen, MD;
Raj P. Warrier, MD
From the Departments of Pediatrics (Drs Shetty, Ode, and Warrier) and Dermatology (Dr Galen), Louisiana State Medical Center and Children's Hospital, New Orleans.
Arch Pediatr Adolesc Med. 1999;153:1197-1198.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. |
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A 5-YEAR-OLD BOY, born in the United States to Pakistani parents, had a history of skin problems and the passage of pink-red urine since birth. He developed severe phototoxic reactions, including vesicles and bullae with resultant scarring, in areas of the skin exposed to the sun. His face was most severely affected (Figure 1). The dorsum of both hands demonstrated hyperpigmentation with irregular areas of hypopigmentation and skin atrophy (Figure 2). In addition to the cutaneous changes, on physical examination he was found to have hepatosplenomegaly and red-stained teeth.
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Figure 1.
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Figure 2.
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Denouement and Discussion: Congenital Erythropoietic Porphyria (Günther Disease)
Figure 1. The skin of the face, particularly the nose and perioral areas, shows severe phototoxic damage with bullae, vesicles, hemorrhagic crusts, and scarring.
Figure 2. The dorsa of the hands are hyperpigmented with areas of hypopigmentation . . . [Full Text of this Article]
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