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A YOUNG MALE WITH CYSTIC FIBROSIS AND LINEAR SCLERODERMA
RCIAPCON 2005


Lakshmi N Moorthy1, Margaret Peterson2,
Karen Onel3, Maria Baratelli1, Thomas Lehman2


1: Robert Wood Johnson Medical School-University of Medicine and Dentistry or New Jersey , New Brunswick , NJ ; 2: Hospital for Special Surgery, New York , NY ; 3: La Rabida Hospital , Chicago , IL .

Objective:
To describe an interesting case of linear scleroderma and morphea in patient with cystic fibrosis.

Case report:
A 10 and ¾-year-old Caucasian male with cystic fibrosis diagnosed at birth developed a rash over his right leg covering his shin and his right foot at 9 and a half years of age. The rash was a coppery reddish-brown macular and blotchy non-pruritic rash. At that time, there was no area of skin thickening or roughness. Two months later, he developed the similar rash over his abdomen. Diagnosis of Schamberg's disease was made after the biopsy of the rash. He applied corticosteroid cream and in 10 months, the rash over his abdomen and legs completely resolved without any residua. The patient had taken levofloxacin prior to the appearance of the rash. Of note, his twin sister, who also has cystic fibrosis, was given levofloxacin and she developed a similar rash as well. About 4-6 weeks after the complete resolution of the rash, he developed a small lump on his abdomen which was exactly in the spot of his previous rash. The skin progressively roughened over the period of 6 weeks and the biopsy was consistent with morphea. He additionally developed skin thickening and some erythema over his right leg, which was also in the area of his previous rash, clinically consistent with linear scleroderma. His twin sister has not developed any morphea/linear scleroderma lesions.

Further exploration into pathogenesis of linear scleroderma is necessary to determine if cystic fibrosis is a predisposing factor.


Last Updated on 15-06-2006

How to cite this url
RCIAPCON 2005 - Conference Abstracts.Pediatric Oncall [serial online] 2006 [cited 15 June 2006(Supplement 6)];3. Available from:
http://www.pediatriconcall.com/fordoctor/Conference_abstracts/
cystic_fibrosis_linear_scleroderma.asp
 
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