Th17 cells and their produced cytokine IL-22 were elevated in the peripheral blood of patients with AGEP. As IL-17 and IL-22 cooperatively stimulate keratinocytes to produce IL-8, IL-8 may contribute to the accumulation of neutrophils in the lesional epidermis of AGEP.

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protein in embryonic heart, neuroepithelium, skin and vasculature. Development 128: 929-41 Garcia-Gras E, Lombardi R, Giocondo MJ et al. (2006) Suppression of canonical Wnt/betacatenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy. J Clin Invest 116:2012-21 Green KJ, Simpson CL (2007) Desmosomes: new perspectives on a classic. J Invest Dermatol 127:2499-515 Jonkman MF, Pasmooij AM, Pasmans SG et al. (2005) Loss of desmoplakin tail causes lethal acantholytic epidermolysis bullosa. Am J Hum Genet 77:653-60 Mahoney MY, Sadowski S, Brennan D et al. (2010) Compound heterozygous desmoplakin mutations result in a phenotype with a combination of myocardial, skin, hair, and enamel abnormalities. J Invest Dermatol 130:968-78 Meng JJ, Bornslaeger EA, Green KJ et al. (1997) Two-hybrid analysis reveals fundamental differences in direct interactions between desmoplakin and cell type-specific intermediate filaments. J Biol Chem 272:21495-503 Nishimura T, Takeichi M (2009) Remodeling of the adherens junctions during morphogenesis. Curr Top Dev Biol 89:33-54 Norgett EE, Hatsell SJ, Carvajal-Huerta L et al. (2000) Recessive mutation in desmoplakin disrupts desmoplakin-intermediate filament interactions and causes dilated cardiomyopathy, woolly hair and keratoderma. Hum Mol Genet 9:2761-6 Abbreviations: OTR, organ transplant recepient; SCC, squamous cell carcinoma; UTR, untranslated region 2686

Extramammary Paget's disease that occurs in non-apocrine-bearing regions is referred to as ectopic and has been rarely reported. A 62-year-old man presented with a slowly progressive, asymptomatic light-brown plaque on his back. Histopathological examination revealed the presence of large pale cells with prominent nuclei, which proliferated diffusely and focally in the epidermis. Immuno-histochemically the tumour cells were positive for CK7, GCDFP-15, CEA, and p63. Based on these findings, we diagnosed the tumour as ectopic extramammary Paget's disease. We reviewed the English and Japanese literature and found 29 previously reported cases of ectopic extramammary Paget's disease, including our case, with a predominance of occurrence in the Asian population. The germinative milk line is known to be a possible site where extramammary Paget's disease occurs. Like-wise, some germinative apocrine-differentiating cells might exist on the trunk preferentially in Asians. Attention should be paid to the development of ectopic as well as triple or quadruple EMPD in Asians.

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