CC-01   CYCLIC HYPOTRICHOSIS

J. Ferrando, R. Grimalt. Department of Dermatology. Hospital Clinic. University of Barcelona. Spain.

Atrichia and congenital hypotrichosis are a heterogeneous group of hair diseases not well understood and only some familiar clinical disorders have been well defined (hereditary simple hypotrichosis). In this group of disorders: Marie-Unna's hereditary trichodysplasia, genohypotrichosis of Villafranca de Duero of Toribio & Quiñones and the types described by Jeanselme & Rime, Benthey-Phillips & Grace, and Kenue & Al-Dhafri seems to be well recognized. We had the opportunity to follow the clinical evolution of three unrelated children that shows a characteristic hypotrichosis of the scalp with a peculiar cyclic behaviour. Patients: Two girls and a boy aged between 2 to 3 years without familial background of alopecia complains because patchy irregular and diffuse alopecia are presenting in determinate seasons of the year (specially in summer time). The girls were born with atrichia and the 3 patients started outbreaks of partial alopecia in temporal and occipital areas with a cyclic evolution, mimicking trichotillomania. Routinary laboratory data were normal in both patients including byotin and byotinidasa activity, aminoacidaemia/ aminoaciduria and oligoelements. In the boy, cutaneous signs of ectodermal dysplasia as dolichocephaly, shortening of 1º and 5º fingers and toes, as well as diffuse epidermal nevus of trunk and limbs were present. In the outbreaks the plunk test were positive and the trichogram revealed dystrophic roots without ruffling. Sporadically, proximal trichorrhexis nodosa were found in the boy. SEM and X-ray microanalysis were not contributives. Scalp biopsies showed decrease of mature hair follicles and absence of inflammatory infiltrate. Comment: The above mentioned data allowed us to rule out a well defined hair disorder of children: alopecia areata, loose anagen hair, arginosuccinuria, byotin deficiency, trichothiodysthrophy and any specific type of ectodermal dysplasia or hypotrichosis simplex. Our patients seem to fill into a peculiar, but characteristic, group of congenital, non familial, patchy alopecia of chronic and cyclic behaviour.

Reference: Ferrando J & Grimalt R. Atlas of Diagnosis in Paediatric Trichology. I.M. & C. Ed., Madrid 2000.