Desquamating Rash in a Patient with Undiagnosed Netherton Syndrome

A 9-year-old girl presented with a 3-week history of a desquamating rash that affected ~20% of her body surface area. Physical examination revealed diffuse desquamative skin patches, of which the patches on the extremities showed a double border scale consistent with ichthyosis linearis circumflexa (Figure, A and B) and sparse, short hair on the patient's head (Figure, C). Laboratory studies were remarkable for an IgE level of 27 294 IU/mL (reference range 0-100). Collectively, these findings raised suspicion of a diagnosis of Netherton syndrome.

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