Xerosis cutis and associated co-factors in women with prurigo nodularis

Abstract: Background: Current data regarding the associated factors of prurigo nodularis are still uncertain, except for atopic predisposition. Objectives: The purposes of this study were to (1) determine the frequencies of xerosis and other accompanying diseases of female patients with prurigo nodularis; (2) compare the demographic, clinical and accompanying disease characteristics by grouping these patients according to whether they have associated xerosis (who were subsequently subgrouped as atopic or non-atopic) or not. Methods: In this retrospective descriptive study, 80 females with PN were categorized according to the accompanying diseases (dermatological, systemic, neurological, psychogenic, mixed, or undetermined origin). Results: A total of 45 associated co-factors including dermatological in 63 (78.8%), systemic in 57 (71.3%), psychological in 33 (41.3%) and neurological co-factors in 14 (17.5%) of all patients with prurigo nodularis were detected. Xerosis was observed in 48 (60%) patients (non-atopic co-factors in 66.7% of them). The ratio of patients with mixed co-factors, dermatological+systemic co-factors and dermatological+systemic+psychological co-factors were found to be significantly higher in patients with xerosis compared to those without xerosis. Study limitations: Our study has certain limitations such as the absence of an age-matched control group, absence of follow-up data and the fact that the diagnosis of xerosis has not been based on objective methods. Conclusions: Xerosis has been identified in more than half of the patients with PN and it has been determined that in most patients xerosis is associated especially with diabetes mellitus and other conditions related to prurigo nodularis.

Ictiosis sindrómicas / Syndromic ichthyosis

Las ictiosis hereditarias son un grupo de desórdenes mendelianos, con manifestaciones clínicas y alteraciones genéticas heterogéneas caracterizadas por la presencia de escamas y/o hiperqueratosis. Las ictiosis sindrómicas son aquellas en las que el defecto genético se manifiesta en la piel y también en otros órganos. Presentamos 7 pacientes con ictiosis sindrómicas: un síndrome IFAP (ictiosis folicular, atriquia, fotofobia), un síndrome de Conradi-Hünermann-Happle (CHH), dos síndromes de Netherton (SN), dos síndromes de Sjögren-Larsson (SSL) y un síndrome KID (queratitis, ictiosis, sordera). Se analizan las características clínicas y diagnósticas de nuestros pacientes (AU)

Inherited ichthyosis are a group of clinical and genetic heterogeneous disorders characterized by the presence of scales, hyperkeratosis or both. In syndromic ichthyosis, the genetic defect involves the skin and other organs. We present 7 patients with syndromic ichthyosis: a case of IFAP syndrome (ichthyosis follicularis with alopecia and photophobia), a case of Conradi-Hünermann-Happle (CHH) syndrome, two cases of Netherton syndrome, two cases of Sjögren-Larsson syndrome and a case of KID syndrome (keratitis, ichthyosis and deafness). We analyze the diagnostic and clinical features of our patients (AU)

Biotinidase deficiency: An atypical presentation.

Biotinidase deficiency is a rare metabolic disorder which can cause dermatological manifestations and lead to severe neurological sequelae if untreated. Holocarboxylase synthetase deficiency also has similar manifestations and needs to be differentiated. We present a neonate who had atypical early onset symptoms and was diagnosed to have biotinidase deficiency.

Familial clustering of a rare syndrome.

Ectrodactyly, ectodermal dysplasia and cleft palate syndrome is a rare autosomal dominant multiple congenital anomaly syndrome with variable expressivity and reduced penetration. The cardinal features are cleft palate/lip, lobster hand deformity, sparse hypopigmented hair, dry scaly skin, and lacrimal and urogenital anomalies. A neonate presented to us with typical features, his mother and other two siblings were also affected.

Paraneoplastic Addisonian pigmentation and acquired ichthyosis as presenting features of multiple myeloma

Black brown hyperpigmentation of the mucosae, sunexposed skin, palmar creases and frictional sites [Addisonian pigmentation] is characteristic of Addison disease. However, it can also occur as a paraneoplastic manifestation of tumours like bronchogenic carcinoma. Acquired ichthyosis starts later in life and can also be a paraneoplastic presentation.We report a unique combination of paraneoplastic Addisonian pigmentation and acquired ichthyosis as presenting features in a patient with undiagnosed multiple myeloma. To the best of our knowledge this combination of paraneoplastic dermatosis has not been documented before in multiple myeloma. It is concluded that the presence of more than one suspicious dermatosis may be an indicator of being paraneoplastic requiring necessary work-up

Harlequin ichthyosis: four cases

Harlequin icthyosis [HI] is inherited as autosomal recessive gene. Many cases are sporadic, but others occur in consanguineous families, in which than one may be affected child

Manifestaciones cutáneas en pacientes dializados por insuficiencia renal crónica / Cutaneous manifestations in patients on dialysis for chronic renal failure

La hemodiálisis proporciona a los pacientes con insuficiencia renal crónica (IRC) una supervivencia prolongada, pero a su vez condiciona el desarrollo de diferentes manifestaciones cutáneas. Se evaluaron 130 pacientes bajo tratamiento hemodialítico por un período mayor a seis meses, encontrándose las siguientes manifestaciones cutáneas: prurito en 100 pacientes (76,92 por ciento), xerosis en 99 (76,15 por ciento), manifestaciones ungulares en 82 pacientes (63,07 por ciento), cambios en la coloración de la piel en 82 pacientes (63.07 por ciento), alteraciones del pelo en 81 (62,30 por ciento), hiperqueratosis folicular urémica en 24 pacientes (18,46 por ciento). En un caso se observó calcinosis cutis (0,76 por ciento)

Hiperqueratosis epidermolítica: a propósito de tres casos / Epidermolythic hyperkaratosis: about three cases

La hiperqueratosis epidermolítica es una alteración hereditaria, con un patrón autosómico dominante. El espectro clínico es muy variable e incluye varias formas de presentación, que anteriormente se consideraban como una forma de ictiosis (ictiosis hystrix, eritrodermia ictiosis forme ampollosa congénita). Estudios recientes señalan mutaciones en los genes 12q y 17q que codifican las queratinas K1 y K10 respectivamente, como los posibles factores causales. Se comunican tres casos con formas clínicas diferentes

Colangitis esclerosante / Sclerosing cholangitis

La colangitis esclerosante primaria es una enfermedad poco comun, de etiologia desconocida y de caracter cronico y progresivo, eventualmente fatal, caracterizada por ictericia obstructiva causada por fibrosis inflamatoria de segmentos de la submucosa, con engrosamiento de la pared y estenosis de la luz de los canales biliares extra e intrahepaticos, lo cual produce cirrosis, hipertension portal e insuficiencia hepatica. El sindrome es de dificil tratamiento quirurgico y el manejo medico de poca utilidad. Se revisan los criterios para establecer el diagnostico, la patologia, la presentacion clinica y caracteristicas radiologicas, y se describen en mayor detalle las tecnicas de dilatacion biliar percutanea, solas o en combinacion con colangioyeyunostomias, con un asa abierta a la piel o colocada en el espacio subcutaneo para acceso para dilatacion intraluminal con cateteres, preconizada por Hutson y asociados de Miami