Crohn's disease and idiopathic thrombocytopenic purpura in a patient with ectodermal dysplasia and immunodeficiency.
In this case report we will describe a rare association between anhyrotic ectodermal dysplasia (AED) and immunodeficiency and autoimmunity [in our case: Idiopathic Thrombocytopenic Purpura (ITP) and Crohn disease]. AED is a rare congenital disorder characterized by sparse hair, abnormal teeth and anhidrosis due to lack of eccrine glands. The survey of 87 cases with (AED) revealed only one Irritable Bowel Disease (IBD). AED has only two relevancies with immunodeficiency: (EDA-ID: Ectodermal Dysplasia Anhyrotic with Immunodeficiency) and APE-CED (Autoimmune polyendocrinopathy, Candidiasis and Ectodermal Dysplasia) that in our case EDA-ID is strongly suspected.
Motamed
Farzaneh
F
Department of Gastroenterology, Tehran University of Medical Sciences, Tehran, Iran.
Zandieh
Fariborz
F
Sedighi
Mostafa
M
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002675:Child, Preschool; D003424:Crohn Disease / Q000175:diagnosis*; D004476:Ectodermal Dysplasia / Q000175:diagnosis*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis*; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000175:diagnosis* / Q000188:drug therapy
D000893:Anti-Inflammatory Agents; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D011241:Prednisone