[Hutchinson-Gilford syndrome].
A case report on a 6-year-old boy suffering from the extremely rare Hutchinson-Gilford syndrome (progeria) is presented. The results of histopathological and immunohistological examination of the scar-like skin lesions are reported. Subcutaneous amorphous nodules were eosinophilic, PAS- und elastica-negative und remained unstained with antibodies against collagen type IV, vimentin, and collagenase. The dense perivascular infiltration consisted of CD4+, CD8-, alpha-1-antichymotrypsin-, MAC 387-, and some vimentin-positive cells. Perinodular blood vessels were more abundant and had a thickened wall. Collagen bundles were swollen. The epidermis appeared atrophic with focal basal cell degeneration.
Wollina
U
U
Klinik und Poliklinik für Hautkrankheiten, Friedrich-Schiller-Universität, Jena.
Reuter
A
A
Schaarschmidt
H
H
Müller
E
E
Maak
B
B
Schmidt
U
U
D000273:Adipose Tissue / Q000473:pathology; D002648:Child; D003094:Collagen / Q000378:metabolism; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D011371:Progeria / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D012867:Skin / Q000473:pathology
D003094:Collagen
Hutchinson-Gilford-Syndrom.