[Auto-inflammatory disease with hyperimmunoglobulinemia D of late onset].

Background: The term "auto-inflammatory diseases" encompasses a group of disorders characterised by febrile episodes of sudden onset associated with joint, abdominal, lymph node and cutaneous signs, each presenting a genetic and/or laboratory specificity allowing their identification. Polyclonal elevation of serum IgD is highly suggestive of hyper-IgD syndrome, a disease with autosomal recessive transmission that usually begins before the age of one year.

Case Report: We report the case of a 46-year-old woman presenting a disease since the age of 30 years clinically very similar to hyper-IgD syndrome except for ocular and pulmonary involvement.

[Unilateral focal dermal hypoplasia].

Introduction: Goltz's syndrome or focal dermal hypoplasia is an X-linked dominant disease. Patients suffer from cutaneous, bone, dental and ocular disorders. The mutated gene has not been identified.