key: cord-0989572-7xypceh4 authors: Callea, Michele; Willoughby, Colin Eric; Perry, Diana; Holzer, Ulrike; Fedele, Giulia; Cárdenas Tadich, Antonio; Cammarata‐Scalisi, Francisco title: COVID‐19 and ectodermal dysplasias. Recommendations are necessary date: 2020-05-30 journal: Dermatol Ther DOI: 10.1111/dth.13702 sha: 83990a87869065bcdb64a1a93738fa2a8b55bcfc doc_id: 989572 cord_uid: 7xypceh4 nan Francisco Cammarata-Scalisi: MD, MSc in Genetics, Servicio de Pediatría, Hospital Regional de Antofagasta, Chile francocammarta19@gmail.com Cell: +56 9 57411721 The term ectodermal dysplasias (EDs) refers to a heterogeneous group of rare congenital conditions affecting the normal development and/or homeostasis of two or more ectodermal derivatives including skin, teeth, hair, nails, and eccrine glands. [1] [2] [3] Hypohidrotic ectodermal dysplasia is estimated to affect at least 1/5,000-10,000 newborns. 1 X-linked hypohidrotic ectodermal dysplasia (XLHED; OMIM 305100), 3 is the most common subtype of EDs, with an incidence of 1/50,000-100,000 males. 1-3 XLHED is characterized by a clinical triad of hypotrichosis, hypo-, oligo-or anodontia, and hypo-or anhidrosis. 2, 3 XLHED is associated with the EDA gene located at (Xq12-q13.1), leading to loss or dysfunction of the signaling protein EDA (Wohlfart et al., 2020), a critical signaling unit involved in the interaction between the ectoderm and the mesoderm. [1] [2] [3] The dominant (OMIM 129490) and recessive (OMIM 614941) subtypes involve the EDAR (2q13), and EDARADD (1q42.3) genes, respectively. Moreover, X-linked anhidrotic ectodermal dysplasia with immunodeficiency (XL-EDA-ID, OMIM 300291) is a primary immunodeficiency disease. This is due to the mutation of IKBKG that encodes NF-κB (nuclear factor-kappa B essential modulator). 4, 5 Mutations in this gene can cause others disorders, including incontinentia pigmenti (OMIM 308300) and hypohidrotic ectodermal dysplasia with immunodeficiency associated with osteopetrosis and lymphoedema (OMIM 300301). 6 Clinical expressions of the disease are characterized by abnormal teeth, hypohidrosis, sparse hair, and the immunological defects of impaired antibody response to polysaccharides, hypogammaglobulinemia, and impaired natural killer cell cytotoxicity. Patients are susceptible to infections with pyogenic bacteria, mycobacteria, parasites, viruses, and fungi. 4 This article is protected by copyright. All rights reserved. The deficient development of other eccrine glands results in recurrent respiratory infections. 2 The presence of abnormal cilia and glands explains the pooling of secretions in the nasal cavities. Allergic rhinitis often worsen nasal symptoms and predispose to sinonasal infections. In order to reduce the nasal symptoms and infections, it is necessary to humidify the environment where patients normally live and to periodically and gently remove nasal crusting. Alcoholic hand disinfectants preserve the epidermal barrier better than hand washes with soap. 7 The use of face masks may also be necessary. The global ED patient organizations came together in 2007 to form the International Ectodermal Dysplasia Network https://edinetwork.org/ providing a leading role in patient advice and support to ED patients and their families. The global registry of patients with EDs affected by COVID-19, their prognosis and clinical evolution will be necessary to study the presentation of the This article is protected by copyright. All rights reserved. The characterization of hypodontia, hypohidrosis, and hypotrichosis associated with X-linked hypohidrotic ectodermal dysplasia: A systematic review EDAR, EDARADD and WNT10A allelic variants in patients with ectodermal derivative impairment in the Spanish population Natural history of X-linked hypohidrotic ectodermal dysplasia: a 5-year follow-up study Hemorrhagic pneumonia as the first manifestation of anhidrotic ectodermal dysplasia with immunodeficiency Mycobacterium abscessus infection in a boy with X-linked anhidrotic ectodermal dysplasia, immunodeficiency Ear nose throat manifestations in hypoidrotic ectodermal dysplasia Challenges of COVID-19 pandemic for dermatology infection in patients who have this genetic condition and thus be able to establish management recommendations.