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Techniques for Toddlers: Linear Band Incision for Harlequin Ichthyosis with Associated Compartment Syndrome
Author(s) -
Tontchev Gramen,
Silverberg Nanette B.,
Shlasko Edward,
Henry Carol,
Roberts Jaclyn L.,
Roth Malcolm Z.
Publication year - 2014
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.12446
Subject(s) - medicine , surgery , cloacal exstrophy , amniotic band syndrome , abdomen , lymphedema , dyskeratosis , acitretin , dermatology , hyperkeratosis , pregnancy , psoriasis , fetus , genetics , cancer , biology , breast cancer , bladder exstrophy
Harlequin ichthyosis ( HI ) is a rare autosomal recessive disorder of cornification in which children are born with an extremely thick stratum corneum that becomes a restrictive circumferential encasement around the orifices, limbs, chest, and abdomen, resulting in limb contractures. We present a neonate diagnosed in utero with HI . The infant was born with encasing bands of thickened skin creating strictures that were causing digital and limb cyanosis (compartment syndrome). We treated the child using a new technique of lysis of the encasing bands that we call linear band incision, using a new escharotomy‐like procedure while the infant was undergoing a 3‐week course oral acitretin therapy. The technique involved linear incision and lysis of encasements that resulted in reperfusion of the injured limbs and prevention of further digital necrosis. The child is currently a healthy 8‐year‐old boy with skin manifestations resembling congenital ichthyosiform erythroderma. He has use of all of the limbs that were released in the procedures and is maintained on frequent application of bland emollients. Linear band incision is a potentially life‐ and limb‐saving technique in children with HI .