A meta-analysis of published trials on the treatment of alopecia areata (AA) came to the conclusion that most have been reported poorly and so small that any important clinical benefits are inconclusive, and considering the possibility of spontaneous remission, especially for those in the early stages of the disease, the options of not being treated or depending on individual preference, and of wearing a wig may be alternative ways of dealing with the condition. And yet, from clinical practice, we know that depending on patient age, surface area, disease duration, and comorbidities an empiric treatment algorithm can be designed that is successful in a significant proportion of patients. More recently, it has been suggested that Janus kinase (JAK) inhibitors may represent the drug of choice for AA, based on robust scientific background and preliminary clinical study results. The fact is that a sophisticated treatment of AA cannot be reduced to one drug, while in many patients, depending on disease duration and surface area, either intravenous methylprednisolone pulse therapy, intralesional triamcinolone acetonide or subcutaneous methotrexate will achieve remission rates in the range of the efficacy of the JAK inhibitors. Moreover, at this time point, affordability of the JAK inhibitors for long-term treatment, sustainability of treatment result, and long-term safety are major issues with regard to the treatment of AA with JAK inhibitors.
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.
Frontal fibrosing alopecia (FFA) represents a distinctive condition with a marginal scarring alopecia along the frontal and temporal hairline. Since its original description, the condition has been recognized to represent a more generalized than localized process, with extension beyond the frontotemporal hairline to include the parieto-occipital hairline and involve peculiar facial papules as evidence of facial vellus hair involvement and loss of peripheral body hair. Finally, the association of FFA with oral lichen planus, nail involvement, and concomitant lichen planopilaris (LPP) points to a close relationship to lichen planus. The Koebner phenomenon or isomorphic reaction has been described in lichen planus, LPP, and ultimately FFA, with face-lift procedures and hair restoration surgery having been implicated as the culprits in the latter. We report the first case of FFA in whom LPP developed at the sites of wig attachments, providing the evidence for Koebner phenomenon. Therefore, wigs are to be included to the list of procedures for hair restoration at risk of eliciting an isomorphic reaction in patients with FFA. Ultimately, the association of Koebner phenomenon with LPP-type lesions in FFA may provide further insight into the underlying pathology and nosology of the condition.
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