No clear genotype-phenotype correlation was found. Patients' biochemical profiles did not reveal a relationship between sterol profiles and severity of disease. A skewed X-chromosome inactivation may explain the clinical phenotype in CDPX2 in some familial cases.

We report on the case of a male who from the start of life displayed vesicular lesions; on the trunk these were clustered and on the limbs they adopted a linear configuration. After biopsy of one such lesion, the histopathological study was compatible with a diagnosis of incontinentia pigmenti (IP). In the following months, hyperkeratotic lesions appeared which later became pigmented. The mother and other female members of the family also showed different degrees of alteration related to the same disease. The karyotype study showed the existence of 47,XXY (Klinefelter syndrome). The exceptional nature of this case is that although it is the third case reported in the literature of a male affected by incontinentia pigmenti with a previous family history, it is the only one combining this characteristic with the presence of a 47,XXY karyotype.

Follicular mucinosis (FM) is an uncommon reaction pattern in which the accumulation of mucin in the follicular epithelium is the main pathologic finding. FM may be idiopathic (primary follicular mucinosis [PFM]), in association with mycosis fungoides or cutaneous T-cell lymphoma, or in association with other neoplastic and inflammatory conditions. Herein we report a case of PFM with identical T-cell clone rearrangement at anatomically distinct sites, supporting the idea that some authors have proposed, that FM may represent a low-grade lymphoproliferative disease related to mycoses fungoides with favorable prognosis.

Resumen: El presente artículo tiene por objeto principal el estudio de los ritos funerarios solicitados por las élites toledanas al Cabido de Curas y Beneficiados, entre 1436 y 1488. En concreto, este trabajo se centra en el papel que jugaron las mandas fúnebres en la construcción de la memoria y escenificación del poder de las oligarquías urbanas, durante la Baja Edad Media. Mediante el estudio de siete obituarios, pretendemos mostrar cómo las honras fúnebres sirvieron como reflejo de su estatus social. A su vez, prestaremos una especial atención al impacto que tales encargos supusieron en la evolución económica e institucional del Cabildo de Curas y Beneficiados de Toledo.

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Following a recent report of xanthomas arising in a patient treated with etoposide for mycosis fungoides (MF), we present a similar patient with xanthomas and MF, who had not received any treatment. Further studies are needed to elucidate the origin and pathophysiology of normocholesterolaemic xanthomas associated with MF.

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ResumenEste trabajo tiene como objeto fundamental el estudio de las cofradías clericales en la Baja Edad Media, en concreto el Cabildo de Curas y Beneficiados de Toledo en la segunda mitad del siglo XV. Mediante el estudio de cuatro obituarios de esta institución, se puede conocer la composición y el desarrollo institucional de estas corporaciones y su importancia en el medio social, donde desarrollaron sus actividades. AbstractThe main purpose of this article is to study clerical brotherhoods in the late Middle Ages, specifically the Chapter of Priests and Incumbents of Toledo in the second half of the fifteenth century. The composition and the institutional development of these corporations and its meaning in the social environment, where they carried out its activities, can be determined through the study of four of this institution's obituaries.