Case reportĀ 
A 15-year-old male was born to unaffected, second degree consanguineous parents. He was referred to our outpatient departement for palmoplantar keratoderma evolving over the last few months. During the childhood, his hair was light colored and coarse. He began to lose it since his teenage years. He also complains of heat intolerance and a reduced ability to sweat. The patient had dental prosthesis due to caries and dental malformation. A cleft palate was repaired in childhood. A dacryocystorhinostomy and a nephrectomy were practiced too. There was no history of similar cases in the family.
On physical examination, the patient measured 161 cm in height. We noticed diffuse bilateral palmoplantar keratoderma with palmoplantar pits. Bilateral ulcerations covered by thick and honey-colored crusts with underlying erythema were observed on the palms and mainly on the carpus (Fig.1) .
He also demonstrated a striking facial morphology consisting of a frontal bossing, a short philtrum, a thin upper lip, a mildly dysmorphic auricles and prognathism (Fig.2) . Skin was dry with discrete scaling. An occasional light-colored, wiry hair was found on an otherwise alopecic scalp. His eyebrows and eyelashes were scarce.
All his nails were dysplastic. They were narrow and small with pterygium and longitudianl ridging (Fig.3 ).
In view of hypohidrotic ectodermal dysplasia associated with orofacial clefting, genitourinary abnormalities, hypodontia, hair involvement and lacrimal duct anomaly, the diagnosis of RHS was made.