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

Area of Science:

• Dermatology
• Genetics
• Neonatal Care

Background:

• Harlequin ichthyosis (HI) is a severe autosomal recessive disorder of cornification.
• Affected neonates present with a thick stratum corneum causing restrictive encasement, leading to limb contractures and compartment syndrome.

Purpose of the Study:

• To present a novel treatment approach for Harlequin ichthyosis.
• To describe the efficacy of linear band incision combined with oral acitretin therapy in a neonate with HI.

Main Methods:

• A neonate diagnosed prenatally with HI was treated with a new technique: linear band incision.
• This escharotomy-like procedure involved lysis of encasing skin bands.
• Treatment was administered concurrently with a 3-week course of oral acitretin therapy.

Main Results:

• The linear band incision technique resulted in reperfusion of cyanotic limbs.
• The procedure prevented further digital necrosis and limb compromise.
• The child, now 8 years old, has full limb use and skin resembling congenital ichthyosiform erythroderma.

Conclusions:

• Linear band incision is a potentially life- and limb-saving intervention for neonates with Harlequin ichthyosis.
• This technique, alongside acitretin therapy, offers a promising management strategy for severe HI cases.