Techniques for toddlers: Linear band incision for harlequin ichthyosis with associated compartment syndrome

Gramen Tontchev, Nanette B. Silverberg, Edward Shlasko, Carol Henry, Jaclyn L. Roberts, Malcolm Z. Roth

Research output: Contribution to journalArticlepeer-review

11 Scopus citations


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.

Original languageEnglish
Pages (from-to)625-629
Number of pages5
JournalPediatric Dermatology
Issue number5
StatePublished - 2014


Dive into the research topics of 'Techniques for toddlers: Linear band incision for harlequin ichthyosis with associated compartment syndrome'. Together they form a unique fingerprint.

Cite this