Ichthyosis is a heterogeneous group of inherited skin disorders characterized by a defect of keratinization. Patients diagnosed with lamellar ichthyosis (LI) and some ichthyosiform syndromes, such as the Conradi-Hünermann-Happle syndrome (CHHS), usually present with hair loss. Even though only few dermatologic complaints carry as many emotional overtones as hair loss, there are very few data available in the literature regarding scalp histopathological features in ichthyosis. A better understanding of scalp changes in such context may result in new therapeutic strategies that in turn would enhance patients' self-esteem and quality of life. The aim of this paper is to describe the scalp histopathological findings of four young patients with cicatricial alopecia: three diagnosed as having LI and the fourth with CHHS. K E Y W O R D S alopecia, dermatopathology, hair follicle 1 | INTRODUCTION Ichthyosis is a heterogeneous group of inherited skin disorders characterized by a defect of keratinization 1-4 with an estimated incidence of 1:200000 ± 300 000. 3 Patients bearing lamellar ichthyosis (LI) and some ichthyosiform syndromes, such as the Conradi-Hünermann-Happle syndrome (CHHS), present with ichthyosiform skin lesions and also scalp involvement, sometimes manifested as unambiguous cicatricial alopecia. In fact, few dermatological complaints carry as many emotional overtones as hair loss. 5 Yet, despite the considerable psychological impairment recognized in patients suffering from ichthyosis, there are very few data available in the literature regarding scalp histopathological features. Hence, a better understanding of scalp changes may result in new therapeutic strategies that in turn would enhance patients' self-esteem and quality of life. In order to highlight histopathological findings, the authors present four cases of overt cicatricial alopecia in young females who have been followed since childhood for ichthyosiform skin changes. Three out of the four patients were diagnosed as having LI and the fourth with CHHS. 2 | CASE REPORTS 2.1 | Cases 1 and 2 Two sisters with LI complained of long-term duration of hair loss and scalp scaling. Both had been followed by a pediatric dermatologist since their childhood. On physical examination, the 15-year-old sister showed frontotemporal hair loss, a central area of patchy cicatricial alopecia and dry, brittle hair (Figure 1). The 24-year-old sister exhibited frontotemporal hairline recession and dry, brittle hair (Figure 2). Dermoscopic and histopathological features are shown in Figures 3-6 and 7.2.2 | Case 3 A 22-year-old female patient with LI complained of hair loss since her early adolescence. On physical examination, there were frontotemporal hairline recession, occipital patchy cicatricial alopecia, sparse pustules and dry, brittle hair (Figure 8). Dermoscopic and histopathological features are shown in Figures 9-10 and 11.