| • | AKA bullous congenital ichthyosiform erythroderma |
| • | keratins 1 and 10 gene mutation (accelerated rate of epidermal turnover) |
clinical:
| • | newborn – widespread bullae, erythroderma, denuded skin |
| • | later infancy to adult – dark warty scale with spiny ridges, increased in flexures; secondary bacterial infection with foul odor |
ddx:
| • | newborn – epidermolysis bullosa, staph scalded skin syndrome, TEN, other causes of blistering |
pearls:
| • | metronidazole cream for odor (kills anaerobes) |
| • | (Dr. Fisher) may have relatives with ichthyosis hystrix |
mnemonic: the terminal differentiation between a hot girl and an ugly girl is a 1 and a 10 (keratin 1 and 10 are the terminal differentiation specific keratins; i.e. expressed in the most superficial keratinocytes in the stratum corneum)
see Ichthyoses
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