The physician suspected that the patient had X-linked ichthyosis. This condition is inherited in an X-linked recessive manner, and thus is found almost exclusively in males. These males have an increased incidence of cryptorchidism and are at an increased risk of testicular cancer. Often, patients with this condition were delivered by cesarean section because a placental sulfatase deficiency results in a failure of labor to progress.
Most of the body is involved, except for the typical sparing of the flexures, face, palms, and soles. There is an accentuation noted on the neck, giving these patients a characteristic “dirty neck” appearance.
X-linked ichthyosis is rare and treatments are based on the clinical experience, rather than large studies. Frequent application of emollients, humectants, and keratinolytics are the mainstay of therapy. There are many effective over-the-counter and prescription products that contain propylene glycol, urea, or lactic acid. Salicylic acid products should be used only on a limited body surface area, as systemic absorption has led to salicylate toxicity in some patients. The physician in this case prescribed 12% ammonium lactate and educated the family about the genetics of this disorder.
Text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. Photo courtesy of the University of Texas Health Sciences Center, Division of Dermatology. This case was adapted from: Babcock M. Genodermatosis. In: Usatine R, Smith M, Mayeaux EJ, et al, eds. The Color Atlas of Family Medicine. New York, NY: McGraw-Hill; 2009:881-885.
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