TY - JOUR
T1 - Rapp-Hodgkin syndrome and AEC syndrome
T2 - Are they the same entity?
AU - Cambiaghi, S.
AU - Tadini, G.
AU - Barbareschi, M.
AU - Menni, S.
AU - Caputo, R.
PY - 1994
Y1 - 1994
N2 - Rapp-Hodgkin syndrome and AEC syndrome are two disorders in which ectodermal dysplasia and clefting are associated. Rapp-Hodgkin syndrome is an autosomal dominant condition characterized by cleft lip and palate, peculiar craniofacial features, and ectodermal dysplasia, consisting of abnormalities of teeth, hair, nails and sweating. AEC syndrome manifests the same defects plus ankyloblepharon and a higher frequency of scalp dermatitis. A child affected by ectodermal dysplasia associated with clefting, ankyloblepharon, severe scalp dermatitis, and the characteristic Rapp-Hodgkin facies is reported. The overlap between Rapp-Hodgkin syndrome and AEC syndrome is discussed. Critical review of both disorders suggests that AEC syndrome and Rapp-Hodgkin syndrome represent the same entity.
AB - Rapp-Hodgkin syndrome and AEC syndrome are two disorders in which ectodermal dysplasia and clefting are associated. Rapp-Hodgkin syndrome is an autosomal dominant condition characterized by cleft lip and palate, peculiar craniofacial features, and ectodermal dysplasia, consisting of abnormalities of teeth, hair, nails and sweating. AEC syndrome manifests the same defects plus ankyloblepharon and a higher frequency of scalp dermatitis. A child affected by ectodermal dysplasia associated with clefting, ankyloblepharon, severe scalp dermatitis, and the characteristic Rapp-Hodgkin facies is reported. The overlap between Rapp-Hodgkin syndrome and AEC syndrome is discussed. Critical review of both disorders suggests that AEC syndrome and Rapp-Hodgkin syndrome represent the same entity.
UR - http://www.scopus.com/inward/record.url?scp=0028300567&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0028300567&partnerID=8YFLogxK
U2 - 10.1111/j.1365-2133.1994.tb06891.x
DO - 10.1111/j.1365-2133.1994.tb06891.x
M3 - Article
C2 - 8305327
AN - SCOPUS:0028300567
VL - 130
SP - 97
EP - 101
JO - British Journal of Dermatology
JF - British Journal of Dermatology
SN - 0007-0963
IS - 1
ER -