Multiple Pterygium Syndrome (MPS) is a rare congenital multiple-anomaly disorder characterized by webbing of the neck, elbows, knees, armpits, and fingers, multiple joint contractures, vertebral defects, growth retardation, several musculoskeletal abnormalities, and minor facial defects. Various cardiovascular, respiratory, gastrointestinal, and genitourinary system abnormalities may also be seen in association with these characteristic findings. The Escobar variant of MPS a non-lethal form of the condition, is an autosomal recessive form of distal arthrogryposis characterized by arthrogryposis multiplex congenita, typical facial appearance, ophthalmologic anomalies, atrophic calf muscles, and interdigital, neck, and axillary pterygia. Other features also include furrowed tongue and enlarged corneal nerves.
It is important to differentiate this condition from the Popliteal Pterygium Syndrome (PPC) and Arthrogyposis Multiplex Congenita (AMC). This can be easily done based on the joint contractures in PPS being limited to the knee, and by the progressive nature of the Escobar Syndrome as compared to AMC.
