Autosomal recessive cutis laxa, type 1 (ARCL1) is a generalized connective tissue disorder characterized by the association of wrinkled, redundant and sagging inelastic skin with severe systemic manifestations (lung atelectesias and emphysema, vascular anomalies, and gastrointestinal and genitourinary tract diverticuli). The disease is Monarch Disease Ontology entry MONDO_0019572 (autosomal recessive cutis laxa type 1). Also known as: ARCL1, autosomal recessive cutis laxa with severe systemic involvement, autosomal recessive cutis laxa, pulmonary emphysema type.