Résumé
Abstract Ectodermal dysplasias are conditions that present primary defects in two or more tissues of ectodermal origin and can be classified as hypohidrotic and hidrotic. Hidrotic ectodermal dysplasia or Clouston syndrome is an autosomal dominant genodermatosis and appears as a triad of clinical findings: palmoplantar keratoderma, nail dystrophy, and hypotrichosis. The hair is sparse and brittle. The nails become thickened and dystrophic, which is an essential characteristic of the syndrome. The diagnosis is made based on clinical findings. This study reports a case of a patient who began with changes in hair, nails and palmoplantar keratoderma in early childhood.
Sujets)
Humains , Femelle , Adolescent , Dysplasie ectodermique/diagnostic , Kératose palmoplantaire/diagnostic , Onychopathies/diagnostic , SyndromeRésumé
Intravascular papillary endothelial hyperplasia is a benign vascular lesion caused by proliferation of endothelium. It is reactive to thrombotic or inflammatory stimuli in the vessel wall.We report the case of a 14-yearold male patient with a violet-colored erythematous tumoral lesion of progressive growth in the occipital region. The diagnosis of intravascular papillary endothelial hyperplasia (IPEH) was confirmed by clinical and histopathological findings. Total lesion exeresis was performed with no recurrence up to date. IPEH presents clinical importance due to its clinical and histological resemblance to angiosarcoma. In order to differentiate it from angiosarcoma, distinguishing features of the benign disease should be considered, such as lack of cellular atypia and rare mitotic activity.Prognosis is good.
Sujets)
Humains , Mâle , Adolescent , Cuir chevelu/anatomopathologie , Tumeurs cutanées/anatomopathologie , Endothélium vasculaire/anatomopathologie , Tumeurs vasculaires/anatomopathologie , Tumeurs de la tête et du cou/anatomopathologie , Pronostic , Tumeurs cutanées/chirurgie , Résultat thérapeutique , Tumeurs vasculaires/chirurgie , Diagnostic différentiel , Érythème , Tumeurs de la tête et du cou/chirurgie , Hyperplasie/chirurgie , Hyperplasie/anatomopathologieRésumé
Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the pilosebaceous follicle and sebaceous glands. It presents in two forms, a primary or idiopathic form and a secondary form associated with various benign or malignant processes. Among the malignant processes, the main association is with mycosis fungoides. The frequent overlap of clinical, histopathological, immunohistochemical and molecular biology characteristics makes the correct classification of these conditions difficult, therefore a long follow-up of all cases is recommended. We report the case of an adolescent with disseminated lesions and discuss the difficulty of early identification of secondary follicular mucinosis associated with cutaneous lymphoma.