Ectrodactyly, ectodermal dysplasia and clefting (EEC) syndrome

Rapp-Hodgkin syndrome: A review of the aspects of hair and hair color

“…Although an erosive scalp dermatitis in AEC can appear at birth or infancy, 29 alopecia starts at puberty in many cases of RHS and EEC. This onset is associated with frequent genitourinary malformations 30 and breast/nipple anomalies (Table I) [31][32][33][34][35][36][37][38][39][40][41][42][43] and might be related to sex hormones. Other speculating factors for the scalp dermatitis or alopecia, or both, are hypoplastic adnexal structures 5,44 or hypoplastic epidermis, 5 folliculitis, 45 infection, 16,27,29,46 high temperatures, 19 and atopy or immunological abnormalities.…”

Perioral Lesions in Ectrodactyly, Ectodermal Dysplasia, Clefting Syndrome

“…Perioral lesions and angular cheilitis at the oral commissures in EEC have been documented on multiple occasions (2–4). Fernandes et al (5) was first to report papillomatous perioral lesions in a 13‐year‐old boy.…”

“…Fernandes et al (5) was first to report papillomatous perioral lesions in a 13‐year‐old boy. Glorio et al (4) reported a 7‐year‐old male patient with vegetant lesions at the corners of the mouth. León‐Mateos et al (2) described lesions consistent with angular cheilitis in an adult who presented with a 3‐month history of perioral lesions.…”

Treatment of Cleft Foot Deformity With a Suture-Button Construct in the Pediatric Foot

“…It is characterized by its "lobster-claw" cleft hand and foot deformities (ectrodactyly) in addition to clefting of the lip and palate and defects in the hair, teeth, nails, sweat glands. 5,8,11 The incidence is estimated at 1:90 000 births when both hands and feet are involved and 1:150 000 with isolated foot deformities. Cleft feet dysplasia is characterized by a spectrum of complete or partial loss of the central metatarsals and phalanges, often with present first and fifth rays lending to the lobster-claw appearance.…”