A patient with alopecia, nail dystrophy, palmoplantar hyperkeratosis, keratitis, hearing difficulty and micrognathia without GJB2 or GJB6 mutations: a new type of hidrotic ectodermal dysplasia?

tion (Fig. 2). The main problems are potential technical difficulties, infection, longer-term complications such as glaucoma, and, in situations such as cicatricial pemphigoid or a patient such as ours with largely mucosal IgA-EBA, there is a potential problem of obtaining an adequate nonscarred mucosal graft. Although the field of vision is reduced (to about 40 ) compared with the normal eye (about 160 ), this is a life-altering procedure for a dry keratinized eye and should be more widely recognized by dermatologists as a treatment option for such patients. IgA-EBA is a rare condition, and blindness due to this has only been described occasionally, but it is a further indication for this procedure.