Tags

Type your tag names separated by a space and hit enter

Scarring folliculitis in the ectrodactyly-ectodermal dysplasia-clefting syndrome. Histologic, scanning electron-microscopic and biophysical studies of hair.
Dermatology. 1997; 194(2):191-4.D

Abstract

Several clinical syndromes are characterized by ectodermal dysplasia (ED) in association with clefting of the lip and/or palate. In these syndromes, alopecia is primarily due to abnormalities of the hair shaft associated with increased hair fragility. Scalp dermatitis is yet another peculiar finding, primarily seen in the ankyloblepharon-ED-clefting (AEC) syndrome. We report on a 16-year-old patient with ectrodactyly-ED-clefting (EEC) syndrome, who exhibited a scarring alopecia due to deep folliculitis. On scanning electron microscopy, irregular torsion and longitudinal grooving of the hair shaft (pili torti et canaliculi) were observed. Quantitative determinations of the elastic and viscous parameters of hair demonstrated a normal viscosity but a significantly reduced hair elasticity, indicating either an abnormal composition or a disordered arrangement of microfibrils within the apparently normal keratin matrix. In contrast to the erosive scalp dermatitis of early onset in the AEC syndrome, alopecia in this case of EEC syndrome demonstrated follicular scarring with onset during puberty. We question a possible role of the anatomical hair abnormality in the pathogenesis of chronic deep folliculitis in this and clinically related syndromes.

Authors+Show Affiliations

Department of Dermatology, University Hospital of Zurich, Switzerland.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

9094476

Citation

Trüeb, R M., et al. "Scarring Folliculitis in the Ectrodactyly-ectodermal Dysplasia-clefting Syndrome. Histologic, Scanning Electron-microscopic and Biophysical Studies of Hair." Dermatology (Basel, Switzerland), vol. 194, no. 2, 1997, pp. 191-4.
Trüeb RM, Tsambaos D, Spycher MA, et al. Scarring folliculitis in the ectrodactyly-ectodermal dysplasia-clefting syndrome. Histologic, scanning electron-microscopic and biophysical studies of hair. Dermatology. 1997;194(2):191-4.
Trüeb, R. M., Tsambaos, D., Spycher, M. A., Müller, J., & Burg, G. (1997). Scarring folliculitis in the ectrodactyly-ectodermal dysplasia-clefting syndrome. Histologic, scanning electron-microscopic and biophysical studies of hair. Dermatology (Basel, Switzerland), 194(2), 191-4.
Trüeb RM, et al. Scarring Folliculitis in the Ectrodactyly-ectodermal Dysplasia-clefting Syndrome. Histologic, Scanning Electron-microscopic and Biophysical Studies of Hair. Dermatology. 1997;194(2):191-4. PubMed PMID: 9094476.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Scarring folliculitis in the ectrodactyly-ectodermal dysplasia-clefting syndrome. Histologic, scanning electron-microscopic and biophysical studies of hair. AU - Trüeb,R M, AU - Tsambaos,D, AU - Spycher,M A, AU - Müller,J, AU - Burg,G, PY - 1997/1/1/pubmed PY - 1997/1/1/medline PY - 1997/1/1/entrez SP - 191 EP - 4 JF - Dermatology (Basel, Switzerland) JO - Dermatology VL - 194 IS - 2 N2 - Several clinical syndromes are characterized by ectodermal dysplasia (ED) in association with clefting of the lip and/or palate. In these syndromes, alopecia is primarily due to abnormalities of the hair shaft associated with increased hair fragility. Scalp dermatitis is yet another peculiar finding, primarily seen in the ankyloblepharon-ED-clefting (AEC) syndrome. We report on a 16-year-old patient with ectrodactyly-ED-clefting (EEC) syndrome, who exhibited a scarring alopecia due to deep folliculitis. On scanning electron microscopy, irregular torsion and longitudinal grooving of the hair shaft (pili torti et canaliculi) were observed. Quantitative determinations of the elastic and viscous parameters of hair demonstrated a normal viscosity but a significantly reduced hair elasticity, indicating either an abnormal composition or a disordered arrangement of microfibrils within the apparently normal keratin matrix. In contrast to the erosive scalp dermatitis of early onset in the AEC syndrome, alopecia in this case of EEC syndrome demonstrated follicular scarring with onset during puberty. We question a possible role of the anatomical hair abnormality in the pathogenesis of chronic deep folliculitis in this and clinically related syndromes. SN - 1018-8665 UR - https://wwww.unboundmedicine.com/medline/citation/9094476/Scarring_folliculitis_in_the_ectrodactyly_ectodermal_dysplasia_clefting_syndrome__Histologic_scanning_electron_microscopic_and_biophysical_studies_of_hair_ L2 - https://www.karger.com?DOI=10.1159/000246097 DB - PRIME DP - Unbound Medicine ER -