Information on Lamellar Ichthyosis

Lamellar ichthyosis is a rare skin condition passed down through families (inherited), which affects a newborn. The newborn is born encased in a collodion membrane that sheds within 10-14 days. The shedding of the membrane reveals generalized scaling with variable redness of the skin. The scaling may be fine or plate like, resembling fish skin. It is also characterized by generalized, abnormally red (erythroderma), dry, rough, thick skin that may cover the redness of the erythroderma. Newborns may be covered with a transparent, membrane that has the appearance of a plastic wrap and that will be sloughed off, leaving large plate-like scales.

 This includes a alteration in the gene for transglutaminase 1 (TGM1). The transglutaminase 1 enzyme is involved in the formation of the cornified cell envelope. A rare phenotype of lamellar Ichthyosis has recently been described in South Africa. The term bathing-suit ichthyosis describes the characteristic distribution of the lesions, which involve the trunk, the proximal parts of the upper limbs, the scalp, and the neck, with sparing of the central face and extremities.

This form of lamellar ichthyosis is caused by a homozygous missense mutation in TGM1. Topical calcipotriol cream has been used with success; however, this vitamin D derivative cans consequence in hypercalcemia when used over broad areas, especially in small children. Long-term treatment with oral isotretinoin has resulted in bony exostoses in some patients, and other long-term adverse effects may arise. In any Lamellar Ichthyosis, minimizing bathing or showering is helpful. Blotting with a towel removes excess applied material. The employ of emollients remains a cornerstone of treatment for Lamellar Ichthyosis.

Regular employ of soaps containing chlorhexidine may also reduce the bacteria. Topical retinoid and vitamin D3 derivatives may also be helpful. Other helpful agents comprise salicylic acid gel, hydrophilic petrolatum and water (in equal parts), and cold cream and the a-hydroxyls acids in various bases. To remove the scale in inherited ichthyoses, patients can apply a preparation containing 50% propylene glycol in water under occlusion every night after hydration of the skin. In children, the propylene glycol preparation should be applied bid without an occlusive dressing overnight.