SCARRING FOLLICULITIS IN THE ECTRODACTYLY-ECTODERMAL DYSPLASIA-CLEFTING SYNDROME - HISTOLOGIC, SCANNING ELECTRON-MICROSCOPIC AND BIOPHYSICAL STUDIES OF HAIR

Several clinical syndromes are characterized by ectodermal dysplasia ( ED) in association with clefting of the lip and/or palate. In these sy ndromes, alopecia is primarily due to abnormalities of the hair shaft associated with increased hair fragility. Scalp dermatitis is yet anot her peculiar finding, primarily seen in the ankyloblepharon-ED-cleftin g (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 (pill torti et canaliculi) were observed. Quantitative determinations of the elastic and viscous parameters of hair demonstrated a normal viscosity but a significantl y reduced hair elasticity, indicating either an abnormal composition o r a disordered arrangement of microfibrils within the apparently norma l keratin matrix. In contrast to the erosive scalp dermatitis of early onset in the AEC syndrome, alopecia in this case of EEC syndrome demo nstrated follicular scarring with onset during puberty. We question a possible role of the anatomical hair abnormality in the pathogenesis o f chronic deep folliculitis in this and clinically related syndromes.