Sparse hair-short stature-skin anomalies syndrome

Sparse hair-short stature-skin anomalies syndrome combines short stature, sparse hair, skin hyperpigmentation and urticaria-like reactions on the hands and arms. An upper central incisor, hypoplastic thumbs and/or palmoplantar hyperkeratosis may also be present. It is thought to be a rare form of ectodermal dysplasia and has been described at least once in a mother and her three sons. Transmission is autosomal dominant, or X-linked.

Single median maxillary incisor

The presence of a single, median maxillary incisor, affecting both the primary maxillary incisor and the permanent maxillary incisor.


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