Introduction:
The clinical presentation of inborn errors of immunity (IEI) is variable and includes severe or unusual infections, autoimmune diseases, and neoplastic diseases. The skin constitutes a target organ of multiple IEI; dermatoses may be its first manifestation or the most evident, hence its relevance for the diagnostic approach.
Objective: to determine the frequency and type of skin manifestations in pediatric patients with IEI

Method:
Observational, descriptive, and cross-sectional study, carried out in a cohort of pediatric patients diagnosed with IEI, from western Mexico.
Results: 18 patients with IEI and skin manifestations were included. Men: 11, women: 7, with the following diagnoses: Hyper IgE Syndrome: 2, X-linked agammaglobulinemia: 4, Hereditary Angioedema: 2, Mucocutaneous Candidiasis: 1, Chronic Granulomatous Disease: 1, Severe Combined Immunodeficiency: 2 (1 Omenn), Wiskott-Aldrich Syndrome: 3, Phenocopy Defect: 1, Pneumococcal vaccine Antibody Response Deficiency: 2 one with ectodermal Dysplasia: 1. The average age at the time of skin manifestations was: 49 months. The presentation of skin manifestations was: infectious: 9 patients (molluscum contagiosum: 1, oral candidiasis: 3, nail mycosis: 1, vaginal candidiasis: 1, skin abscesses: 3, periorbital cellulitis: 1, BCGitis: 3, autoimmune skin manifestations: alopecia universalis: 1, scleroderma: 1, allergic skin manifestations: 8: eczema mild 3, severe eczema 2, prurigo: 1, angioedema: 2 Hematological skin manifestations: 4 patients: petechiae and bruising. Three patients died: one patient with Omenn syndrome, one with severe combined immunodeficiency, and one with Wiskott-Aldrich syndrome. Fourteen patients received gamma globulin therapy. One patient received Icatibant, one patient interferon gamma 1-b. Currently, seven patients receive antimicrobial prophylaxis.
Conclusions: In patients with IEI there may be cutaneous manifestations not only of infectious origin but also of autoimmune and allergic origin. Skin abscesses and BCGitis should lead to suspicion of IEI.