Ichthyoses are a heterogeneous group of diseases that are classified as hereditary cornification disorders. Usually ichthyoses are not life-threatening but in most cases are disfiguring. Because of the early onset of the disease during childhood, patients not only have to cope with the dermatological symptoms, but also with psychological problems concerning their self-assessment and self-esteem.
A 7-year-old Moroccan girl presented with severe nonerythrodermic lamellar ichthyosis. The mother described her daughter as having psychosocial problems in preschool and kindergarten. On physical examination the girl was found to be physically retarded but without intellectual deficits. There was no evidence of ichthyosis in the family for the last 2 generations. The diagnosis was based on the clinical picture in addition to findings from histopathological and biochemical examinations of the girl. Since the disease had been unresponsive to standard topical therapy, including pretreatment with urea-containing ointments and ciclopiroxolamine, we enrolled her in a therapeutic trial with the new topical receptor-selective retinoid tazarotene. Tazarotene gel was topically applied to one side of her chest (9% of body surface area) in a concentration of 0.05% daily for 3 weeks. The results of hematologic, blood chemistry, and urinalysis tests did not show any significant changes. The patient reported only mild pruritus in treated skin areas. Clinically, an almost complete remission of hyperkeratosis was noted after only 3 weeks of treatment (Figure 1). This clinical result encouraged us to treat not only one side of her body but the whole trunk and neck region, after defining a maximal area of 10% of the whole body surface for the daily application. After 6 more weeks of treatment, no ichthyosiform skin lesions were detected clinically. The therapeutic benefit was limited to treated skin areas, and systemic effects were not found either clinically or by laboratory analysis.
Stege H, Hofmann B, Ruzicka T, Lehmann P. Topical Application of Tazarotene in the Treatment of Nonerythrodermic Lamellar Ichthyosis. Arch Dermatol. 1998;134(5):640–641. doi:https://doi.org/
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: