Increased procollagen alpha1(I) mRNA expression by dermal fibroblasts in melorheostosis.
We report a patient with melorheostosis in whom increased procollagen alpha1(I) mRNA expression and alpha1(I), alpha2(I) and alpha1(III) collagen secretion were observed in dermal fibroblasts obtained from a skin biopsy overlying the involved bone. The patient was a 53-year-old man with melorheostosis lesions over the left knee joint. Multiple pigmented macules were present on the medial aspect of the lower left leg. Hyperpigmentation of the basal keratinocytes, thick-walled vessels in the reticular dermis, and proliferation of normal-appearing collagen around the hair follicles were observed histologically.
Endo
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Department of Clinical Biology of Extracellular Matrix, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan. chw92121@syd.odn.ne.jp
Katsumi
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A
Kuroda
K
K
Utani
A
A
Moriya
H
H
Shinkai
H
H
D005347:Fibroblasts / Q000378:metabolism; D015870:Gene Expression; D006801:Humans; D017495:Hyperpigmentation / Q000378:metabolism* / Q000473:pathology; D007868:Leg Dermatoses / Q000378:metabolism* / Q000473:pathology; D008297:Male; D008557:Melorheostosis / Q000378:metabolism*; D008875:Middle Aged; D011347:Procollagen / Q000096:biosynthesis* / Q000235:genetics; D012333:RNA, Messenger / Q000235:genetics; D012867:Skin / Q000378:metabolism* / Q000473:pathology
D011347:Procollagen; D012333:RNA, Messenger