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Incontinentia pigmenti with NEMO mutation in a Japanese family

Dear Editor, Incontinentia pigmenti (IP) is a rare X-linked dominant genodermatosis characterized by skin lesions and disorders of various organs. Mutations of NEMO (nuclear factor-kappa B essential modulator), located at Xq28, are responsible for IP. The NEMO codes are a regulatory component of the inhibitor of the kappa-B kinase complex that is required for the activation of the nuclear factor kappa-B (NF-jB) pathway. A large deletion of NEMO exon 4–10 is reported in approximately 80% of IP patients; however, other NEMO mutations such as missense mutations and small deletions have been identified. The patient was a 6-day-old Japanese girl, who was the first child of non-consanguineous parents. She was born at 39 weeks of gestation in a good condition. She had skin lesions at birth characterized as linear and whorled patterns of erythematous vesiculo-bullous lesions following Blaschko’s lines on her lower extremities (Fig. 1a,b) and upper extremities. Histopathological examination showed acanthosis and intraepidermal vesicles with numerous eosinophils, which indicate ‘‘eosinophilic spongiosis’’ (Fig. 1c,d). Physical examination showed linear hairless lesions. Complete blood examination revealed leukocytosis of 20000 cells ⁄ mm and mild eosinophilia of 3200 eosinophils ⁄ mm (16% of total white blood cells). The results of magnetic resonance imaging, electroencephalogram, chest radiography and echocardiography showed