elastin and Keratosis

This article reviews the diseases that may show epidermal perforation as a histologic feature. Many of these represent examples of transepithelial elimination (TEE), a mechanism by which the skin rids itself of abnormal substances. After a review of disorders in which perforation is an occasional finding, four diseases that have been considered essential perforating disorders are discussed: elastosis perforans serpiginosa (EPS), reactive perforating collagenosis (RPC), perforating folliculitis (PF), and Kyrle 's disease (KD). A review of the literature, including recent reports of perforating diseases associated with chronic renal failure, suggests that there may be considerable clinical and histologic overlap among PF, KD, and the adult form of "perforating collagenosis." A working classification for the perforating disorders is suggested.

Topics: Collagen Diseases; Elastic Tissue; Elastin; Folliculitis; Granuloma; Humans; Keratosis; Kidney Failure, Chronic; Microscopy, Electron; Pseudoxanthoma Elasticum; Skin; Skin Diseases

Photodamage is characterized by degradation of collagen and accumulation of abnormal elastin in the superficial dermis and several matrix metalloproteinases have previously been implicated in this process. Using immunohistochemistry and in situ hybridization, we have studied the localization of two elastolytic matrix metalloproteinases, matrilysin (matrix metalloproteinase-7) and human macrophage metalloelastase (matrix metalloproteinase-12) in solar damage. Human macrophage metalloelastase protein was detected in the superficial dermis in areas of elastotic material. Matrix metalloproteinase-7 was seen in the mid-dermis in regions with less damaged elastic fibers and morphologically better preserved collagen as well as in a band-like pattern below basal keratinocytes in eight of 18 solar elastosis. In samples taken from healthy volunteers 3 d after repeated ultraviolet A or ultraviolet B photoprovocation, occasional immunopositive cells for human macrophage metalloelastase (stromal) or matrix metalloproteinase-7 (sweat gland epithelium) were detected. In samples taken 1 d after ultraviolet B exposure, however, basal keratinocytes were matrix metalloproteinase-7 immunopositive, explaining the linear immunostaining below basal keratinocytes noted particularly in ultraviolet B treated 3 d specimens. Upregulation of metalloelastase was also demonstrated in the skin of hairless mice after repeated ultraviolet exposure. In normal skin, no staining for human macrophage metalloelastase or matrix metalloproteinase-7 was observed in association with elastin. The amount of immunoreactivity for the substrates of matrix metalloproteinase-7, versican, and tenascin, was clearly increased in solar elastosis and photoprovocated skin; versican but not tenascin was detected in the same areas as matrix metalloproteinase-7. Our results suggest that both matrix metalloproteinase-7 and -12 may contribute to remodeling of elastotic areas in sun-damaged skin.

Topics: Adult; Aged; Animals; Chondroitin Sulfate Proteoglycans; Elastin; Gene Expression Regulation, Enzymologic; Humans; Keratosis; Lectins, C-Type; Matrix Metalloproteinase 12; Matrix Metalloproteinase 7; Metalloendopeptidases; Mice; Mice, Hairless; RNA, Messenger; Skin; Sunlight; Tenascin; Transforming Growth Factor beta; Ultraviolet Rays; Versicans

Elastosis perforans serpiginosa (EPS) is an uncommon skin disease characterized by transepidermal elimination of abnormal elastic fibers. The disease is frequently associated with congenital connective tissue disorders or Down's syndrome. The pathogenesis of EPS is still unclear. There are a few reports in the literature about a familial occurrence of EPS in which different modes of inheritance are suggested. To support the hypothesis of a congenital origin of the disease, we have studied another family with EPS.. In this study, we describe a family in which two sisters and a brother were affected by EPS. The father and three paternal uncles were most probably affected by the same disease. There were no signs of other congenital connective tissue disease in the family members.. An autosomal dominant mode of inheritance with variable expression of EPS is suggested.

Topics: Adult; Aged; Atrophy; Cicatrix; Connective Tissue Diseases; Elastic Tissue; Elastin; Female; Humans; Keratins; Keratosis; Male; Middle Aged; Neck; Skin Diseases

Topics: Cations, Divalent; Cells, Cultured; Cutis Laxa; Elastin; Fibroblasts; Humans; Keratosis; Oligopeptides; Pancreatic Elastase; Pseudoxanthoma Elasticum; Skin

Topics: Basement Membrane; Biopsy; Cheek; Collagen; Connective Tissue; Denture, Complete; Elastic Tissue; Elastin; Follow-Up Studies; Humans; Keratosis; Mast Cells; Mouth Mucosa; Mouth, Edentulous; Staining and Labeling; Time Factors; Transplantation, Autologous; Vestibuloplasty

Topics: Aging; Amino Acids; Carcinoma, Squamous Cell; Collagen; Elastin; Geriatrics; Histology; Humans; Keratosis; Keratosis, Actinic; Radiation Effects; Skin; Skin Aging; Skin Diseases; Skin Neoplasms; Sunlight; Ultraviolet Rays