INSIBIO   05451
INSTITUTO SUPERIOR DE INVESTIGACIONES BIOLOGICAS
Unidad Ejecutora - UE
artículos
Título:
Focal dermal hypoplasia: ultrastructural abnormalities of the connective tissue
Autor/es:
BOENTE MC; ASIAL RA; WINIK BC
Revista:
JOURNAL OF CUTANEOUS PATHOLOGY
Referencias:
Año: 2006
ISSN:
0303-6987
Resumen:
We followed over 10 years three girls with focal dermal
hypoplasia syndrome. The histopathological changes demonstrated at
the optical level an hypoplastic dermis with thin and scarce collagen
bundles and a marked diminution of elastic fibers. Mature adipose
tissue was found scattered within the papillary and reticular dermis. No
alterations in the basal membrane were observed by immunocyto-chemical
or ultrastructural techniques. Ultrastructurally, in the skin-affected
areas, loosely arranged collagen bundles composed of few
fibrils were seen scattered in the extracellular matrix. Scarce elastic
fibers of normal morphology were also observed. Fibroblasts were
smaller, oval-shaped, and diminished in number with a poorly devel-oped
cytoplasm. In these fibroblasts, the most conspicuous feature was
a remarkable and irregular thickening of the nuclear fibrous lamina.
Taking into account that a common link between all laminopaties may
be a failure of stem cells to regenerate mesenchymal tissue, this failure
would induce the dermal hypoplasia observed in our patients present-ing
Goltz syndrome.
Boente MdC, Asial RA, Winik BC. Focal dermal hypoplasia: ultra-structural
abnormalities of the connective tissue.
J Cutan Pathol 2006; 00: 17. # Blackwell Munksgaard 2006.
hypoplasia syndrome. The histopathological changes demonstrated at
the optical level an hypoplastic dermis with thin and scarce collagen
bundles and a marked diminution of elastic fibers. Mature adipose
tissue was found scattered within the papillary and reticular dermis. No
alterations in the basal membrane were observed by immunocyto-chemical
or ultrastructural techniques. Ultrastructurally, in the skin-affected
areas, loosely arranged collagen bundles composed of few
fibrils were seen scattered in the extracellular matrix. Scarce elastic
fibers of normal morphology were also observed. Fibroblasts were
smaller, oval-shaped, and diminished in number with a poorly devel-oped
cytoplasm. In these fibroblasts, the most conspicuous feature was
a remarkable and irregular thickening of the nuclear fibrous lamina.
Taking into account that a common link between all laminopaties may
be a failure of stem cells to regenerate mesenchymal tissue, this failure
would induce the dermal hypoplasia observed in our patients present-ing
Goltz syndrome.
Boente MdC, Asial RA, Winik BC. Focal dermal hypoplasia: ultra-structural
abnormalities of the connective tissue.
J Cutan Pathol 2006; 00: 17. # Blackwell Munksgaard 2006.
hypoplasia syndrome. The histopathological changes demonstrated at
the optical level an hypoplastic dermis with thin and scarce collagen
bundles and a marked diminution of elastic fibers. Mature adipose
tissue was found scattered within the papillary and reticular dermis. No
alterations in the basal membrane were observed by immunocyto-chemical
or ultrastructural techniques. Ultrastructurally, in the skin-affected
areas, loosely arranged collagen bundles composed of few
fibrils were seen scattered in the extracellular matrix. Scarce elastic
fibers of normal morphology were also observed. Fibroblasts were
smaller, oval-shaped, and diminished in number with a poorly devel-oped
cytoplasm. In these fibroblasts, the most conspicuous feature was
a remarkable and irregular thickening of the nuclear fibrous lamina.
Taking into account that a common link between all laminopaties may
be a failure of stem cells to regenerate mesenchymal tissue, this failure
would induce the dermal hypoplasia observed in our patients present-ing
Goltz syndrome.
Boente MdC, Asial RA, Winik BC. Focal dermal hypoplasia: ultra-structural
abnormalities of the connective tissue.
J Cutan Pathol 2006; 00: 17. # Blackwell Munksgaard 2006.
hypoplasia syndrome. The histopathological changes demonstrated at
the optical level an hypoplastic dermis with thin and scarce collagen
bundles and a marked diminution of elastic fibers. Mature adipose
tissue was found scattered within the papillary and reticular dermis. No
alterations in the basal membrane were observed by immunocyto-chemical
or ultrastructural techniques. Ultrastructurally, in the skin-affected
areas, loosely arranged collagen bundles composed of few
fibrils were seen scattered in the extracellular matrix. Scarce elastic
fibers of normal morphology were also observed. Fibroblasts were
smaller, oval-shaped, and diminished in number with a poorly devel-oped
cytoplasm. In these fibroblasts, the most conspicuous feature was
a remarkable and irregular thickening of the nuclear fibrous lamina.
Taking into account that a common link between all laminopaties may
be a failure of stem cells to regenerate mesenchymal tissue, this failure
would induce the dermal hypoplasia observed in our patients present-ing
Goltz syndrome.
Boente MdC, Asial RA, Winik BC. Focal dermal hypoplasia: ultra-structural
abnormalities of the connective tissue.
J Cutan Pathol 2006; 00: 17. # Blackwell Munksgaard 2006.
hypoplasia syndrome. The histopathological changes demonstrated at
the optical level an hypoplastic dermis with thin and scarce collagen
bundles and a marked diminution of elastic fibers. Mature adipose
tissue was found scattered within the papillary and reticular dermis. No
alterations in the basal membrane were observed by immunocyto-chemical
or ultrastructural techniques. Ultrastructurally, in the skin-affected
areas, loosely arranged collagen bundles composed of few
fibrils were seen scattered in the extracellular matrix. Scarce elastic
fibers of normal morphology were also observed. Fibroblasts were
smaller, oval-shaped, and diminished in number with a poorly devel-oped
cytoplasm. In these fibroblasts, the most conspicuous feature was
a remarkable and irregular thickening of the nuclear fibrous lamina.
Taking into account that a common link between all laminopaties may
be a failure of stem cells to regenerate mesenchymal tissue, this failure
would induce the dermal hypoplasia observed in our patients present-ing
Goltz syndrome.
Boente MdC, Asial RA, Winik BC. Focal dermal hypoplasia: ultra-structural
abnormalities of the connective tissue.
J Cutan Pathol 2006; 00: 17. # Blackwell Munksgaard 2006.
hypoplasia syndrome. The histopathological changes demonstrated at
the optical level an hypoplastic dermis with thin and scarce collagen
bundles and a marked diminution of elastic fibers. Mature adipose
tissue was found scattered within the papillary and reticular dermis. No
alterations in the basal membrane were observed by immunocyto-chemical
or ultrastructural techniques. Ultrastructurally, in the skin-affected
areas, loosely arranged collagen bundles composed of few
fibrils were seen scattered in the extracellular matrix. Scarce elastic
fibers of normal morphology were also observed. Fibroblasts were
smaller, oval-shaped, and diminished in number with a poorly devel-oped
cytoplasm. In these fibroblasts, the most conspicuous feature was
a remarkable and irregular thickening of the nuclear fibrous lamina.
Taking into account that a common link between all laminopaties may
be a failure of stem cells to regenerate mesenchymal tissue, this failure
would induce the dermal hypoplasia observed in our patients present-ing
Goltz syndrome.
Boente MdC, Asial RA, Winik BC. Focal dermal hypoplasia: ultra-structural
abnormalities of the connective tissue.
J Cutan Pathol 2006; 00: 17. # Blackwell Munksgaard 2006.
hypoplasia syndrome. The histopathological changes demonstrated at
the optical level an hypoplastic dermis with thin and scarce collagen
bundles and a marked diminution of elastic fibers. Mature adipose
tissue was found scattered within the papillary and reticular dermis. No
alterations in the basal membrane were observed by immunocyto-chemical
or ultrastructural techniques. Ultrastructurally, in the skin-affected
areas, loosely arranged collagen bundles composed of few
fibrils were seen scattered in the extracellular matrix. Scarce elastic
fibers of normal morphology were also observed. Fibroblasts were
smaller, oval-shaped, and diminished in number with a poorly devel-oped
cytoplasm. In these fibroblasts, the most conspicuous feature was
a remarkable and irregular thickening of the nuclear fibrous lamina.
Taking into account that a common link between all laminopaties may
be a failure of stem cells to regenerate mesenchymal tissue, this failure
would induce the dermal hypoplasia observed in our patients present-ing
Goltz syndrome.
Boente MdC, Asial RA, Winik BC. Focal dermal hypoplasia: ultra-structural
abnormalities of the connective tissue.
J Cutan Pathol 2006; 00: 17. # Blackwell Munksgaard 2006.
hypoplasia syndrome. The histopathological changes demonstrated at
the optical level an hypoplastic dermis with thin and scarce collagen
bundles and a marked diminution of elastic fibers. Mature adipose
tissue was found scattered within the papillary and reticular dermis. No
alterations in the basal membrane were observed by immunocyto-chemical
or ultrastructural techniques. Ultrastructurally, in the skin-affected
areas, loosely arranged collagen bundles composed of few
fibrils were seen scattered in the extracellular matrix. Scarce elastic
fibers of normal morphology were also observed. Fibroblasts were
smaller, oval-shaped, and diminished in number with a poorly devel-oped
cytoplasm. In these fibroblasts, the most conspicuous feature was
a remarkable and irregular thickening of the nuclear fibrous lamina.
Taking into account that a common link between all laminopaties may
be a failure of stem cells to regenerate mesenchymal tissue, this failure
would induce the dermal hypoplasia observed in our patients present-ing
Goltz syndrome.
Boente MdC, Asial RA, Winik BC. Focal dermal hypoplasia: ultra-structural
abnormalities of the connective tissue.
J Cutan Pathol 2006; 00: 17. # Blackwell Munksgaard 2006.