OBSOLETE: Ectodermal dysplasia-absent dermatoglyphs syndrome

Ectodermal dysplasia-absent dermatoglyphs syndrome, also known as Basan syndrome or ectodermal dysplasia with absent dermatoglyphics, is an extremely rare genetic condition characterized by the congenital absence of dermatoglyphics (fingerprints and other skin ridge patterns on the hands and feet) combined with features of ectodermal dysplasia. This condition primarily affects the skin and its appendages. Key clinical features include absent or reduced fingerprints (adermatoglyphia), facial milia (small white cysts on the skin, particularly the face) that may appear in infancy and resolve over time, and blistering of the skin, especially on the hands and feet. Some affected individuals may also have nail abnormalities and reduced sweating. The condition is present from birth, with blistering often noted in the neonatal period. This entry is marked as OBSOLETE in Orphanet, suggesting it may have been reclassified or merged with another condition. The phenotype overlaps with what has been described as adermatoglyphia or 'immigration delay disease,' and some cases may now be classified under SMARCAD1-related conditions. The syndrome was first described by Basan in 1965 in a large family. There is no specific treatment for this condition; management is supportive and focuses on skin care, prevention of blistering, and monitoring of ectodermal features. Genetic counseling is recommended for affected families.

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