SYNDROMIC ICHTHYOSES
SYNDROMIC ICHTHYOSES ICD-10: Q80.9
• These are a number of rare syndromic ichthyoses where ichthyotic skin changes are associated with metabolic and/or functional and structural abnormalities.
• For erythrokeratodermia variabilis (Fig. 4-14), keratitis–ichthyosis–deafness (KID) syndrome (Fig. 4-15), Child syndrome, and Netherton syndrome (Fig. 4-16), see P Fleckman, JJ DiGiovanna, in L Goldsmith et al: Fitzpatrick’s Dermatology in General Medicine, 8th ed. New York, NY: McGraw-Hill, pp. 507–538, 2012.
FIGURE 4-14 • Erythrokeratodermia variabilis Note hyperkeratotic plaques on the face associated with migrating erythemas on the neck (arrow).
FIGURE 4-15 • Keratitis-ichthyosis-deafness (KID) syndrome Hyperkeratosis on the cheeks, and the tip of the nose and ear and sparse hair are characteristic for this syndrome as are hyperkeratosis in the flexural folds, dorsa of hands. In addition, there is keratitis and loss of hearing.
FIGURE 4-16 • Netherton syndrome Ichthyosis linearis circumflexa consists of serpiginous psoriasiform erythema with scaling and is associated with trichorrhexis nodosa (bamboo hairs).