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Nov 2012

Progressive Sclerotic Skin Lesions in a 49-Year-Old Man—Diagnosis

Author Affiliations
 

SECTION EDITOR: MARY S. STONE, MD; ASSISTANT SECTION EDITORS: SOON BAHRAMI, MD; CARRIE ANN R. CUSACK, MD; MOLLY A. HINSHAW, MD; ARNI K. KRISTJANSSON, MD; LORI D. PROK, MD;

Arch Dermatol. 2012;148(11):1318. doi:10.1001/archderm.148.11.1318-b

The punch biopsy result showed sclerosis with dense collagen deposition in the papillary and reticular dermis, extending into the subcutis and impinging on skin appendages. There was a scant perivascular and interstitial lymphocytic infiltrate. Laboratory studies revealed a normal complete blood cell count and renal function. His transaminase level was elevated, and hepatitis C viral load was more than 300 000 IU/mL. The ferritin level was twice the upper limit of normal. His serum porphyrin level was within normal limits, and analyses of his 24-hour urine porphyrins revealed a normal porphobilinogen level but were notable for uroporphyrin (3679 nmol/24 h), heptacarboxyl porphyrin (1497 nmol/24 h), coproporphyrin (713 nmol/24 h), hexacarboxyl porphyrin (176 nmol/24 h), and pentacarboxyl porphyrin (446 nmol/24 h) levels, which were highly elevated in a pattern consistent with PCT. Phlebotomy was initiated to withdraw 1 unit of blood every 2 weeks, to a target hematocrit level of 34%. He was advised to practice photoprotection, and a follow-up visit was arranged with the hepatology service for treatment of HCV. When the patient was seen at follow-up after 13 phlebotomy sessions, clinically evident softening of his indurated plaques was noted.

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