X-linked hyper-IgM syndrome and hypohydrotic ectodermal dysplasia (Nemo deficiency)

Nemo defect affects multiple organs and presents with vesiculo-bullous lesions with erythema with linear arrangement on the extremities and lateral aspects of the trunk. These bullous and erythematous lesions evolve into verrucous lessions after weeks to month, and then are followed by atrophy or depigmentation with a slate-brown or blue-gray pigmentation. In adulthood, there are hypopigmented linear macules on the extremities and the trunk with a lack of skin appendages. This disorder is usually prenatally lethal in males and contributes to abnormalities of skin, hair, nails, teeth and central nervous system in carrier females.