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IL36RN gene analysis of two Japanese patients with generalized pustular psoriasis

distinct phenotypic feature of acquired CL. We recommend that this phenotype should be designated as acquired CL with papular lesions. The condition should be differentiated from mid-dermal elastolysis (MDE), which is another rare acquired disorder of elastic tissue that manifests clinically as either a fine wrinkling of the skin parallel to the skin cleavage or as perifollicular papules. Unlike acquired CL, MDE is characterized histopathologically by a band-like loss of elastic fibers limited to the mid-dermis. The basic pathology common to all forms of CL is a deficiency of elastic fibers. Elastic fibers are composed of two distinct components: an amorphous ELN core and microfibrils. Fibulin-5 localizes to the surface of elastic fibers, which represents an essential determinant of elastic fiber organization. Molecular defects underlying inherited CL have been well identified. Mutations in ELN and FBLN5 have been shown to be responsible in the majority of cases. By contrast, the precise etiology of acquired CL remains unclear, although it has been reported de novo or following cutaneous eruption, antibiotic therapy, or febrile illness. In 2006, Hu et al. reported a patient with acquired CL preceded by Toxocara canis parasitism and chronic urticaria. Mutational analysis showed the patient to be heterozygous for an inherited FBLN5 allele G202R, and compound heterozygous for ELN alleles A55V and G773D. This would imply that the pathogenesis of acquired CL may also involve an underlying genetic susceptibility and highlights the importance of molecular genetic analysis in patients with acquired CL. The present patient was screened for both ELN and FBLN5, but no mutations were found. It is possible that not only ELN and FBLN5 but also other genes associated with the biosynthesis of elastic fibers may be involved in acquired CL. Further studies are needed to clarify the exact pathogenesis of acquired CL.