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ERYTHROPOIETIC PROTOPORPHYRIA (1997 Uniformed Services Dermatology Seminar, Case #29) Mary K. Mather, MAJ, MC, USA

Cirivello MJC - MedPix

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: ERYTHROPOIETIC PROTOPORPHYRIA (1997 Uniformed Services Dermatology Seminar, Case #29) Mary K. Mather, MAJ, MC, USA

History: 6 year old male whose mother states that at approximately 12 months of age, the patient would "scream" whenever taken into the sun. She also commented that she noticed that his dorsal hands looked "leathery" and the bridge of his nose and cheeks were red and irritated in appearance. Upon moving to Georgia from Ohio at age 18 months, patient developed a marked sunburn with short exposures to the sun. Patient was evaluated by Dermatology and Pediatrics, and found to have markedly elevated erythrocyte protoporphyrin levels of 224 mcg/dl (normal <35 mcg/dl). There is 4+ red blood cell florescence. No known history of photosensitivity in parents or younger sister.

Exam: PHYSICAL EXAM: Healthy appearing male. The skin of the dorsal hands is thickened and wrinkled over the knuckles, giving them an "aged" appearance. There are no blisters or milia. The skin of the nose shows scattered tiny reddish scars. LABORATORY: erythrocyte protoporphyrin 224 mcg/dl (NL <35 mcg/dl) HISTOPATHOLOGY: Punch biopsy of acral skin. There is a thick eosinophilic mantle surrounding the blood vessels in the papillary dermis. PAS stain highlights the reduplication of the basement membrane surrounding the blood vessels.

No MeSH data available.


ERYTHROPOIETIC PROTOPORPHYRIA
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MPX2393_synpic16726: ERYTHROPOIETIC PROTOPORPHYRIA


ERYTHROPOIETIC PROTOPORPHYRIA (1997 Uniformed Services Dermatology Seminar, Case #29) Mary K. Mather, MAJ, MC, USA

Cirivello MJC - MedPix

ERYTHROPOIETIC PROTOPORPHYRIA
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=MPX2393&req=5

MPX2393_synpic16726: ERYTHROPOIETIC PROTOPORPHYRIA

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: ERYTHROPOIETIC PROTOPORPHYRIA (1997 Uniformed Services Dermatology Seminar, Case #29) Mary K. Mather, MAJ, MC, USA

History: 6 year old male whose mother states that at approximately 12 months of age, the patient would "scream" whenever taken into the sun. She also commented that she noticed that his dorsal hands looked "leathery" and the bridge of his nose and cheeks were red and irritated in appearance. Upon moving to Georgia from Ohio at age 18 months, patient developed a marked sunburn with short exposures to the sun. Patient was evaluated by Dermatology and Pediatrics, and found to have markedly elevated erythrocyte protoporphyrin levels of 224 mcg/dl (normal <35 mcg/dl). There is 4+ red blood cell florescence. No known history of photosensitivity in parents or younger sister.

Exam: PHYSICAL EXAM: Healthy appearing male. The skin of the dorsal hands is thickened and wrinkled over the knuckles, giving them an "aged" appearance. There are no blisters or milia. The skin of the nose shows scattered tiny reddish scars. LABORATORY: erythrocyte protoporphyrin 224 mcg/dl (NL <35 mcg/dl) HISTOPATHOLOGY: Punch biopsy of acral skin. There is a thick eosinophilic mantle surrounding the blood vessels in the papillary dermis. PAS stain highlights the reduplication of the basement membrane surrounding the blood vessels.

No MeSH data available.