Psoriasis in autoimmune polyendocrine syndrome type I: A possible complication or a non-endocrine minor component.

Introduction: Autoimmune polyendocrine syndrome type I (APS I) is an autosomal recessive systemic autoimmune disorder, affecting primarily endocrine glands, in which chronic mucocutaneous candidiasis is an early and prominent manifestation. We describe the rare occurrence of unstable psoriasis (with onset of pustular lesions) in a case of APS I without mucocutaneous candidiasis. A patient presenting with unstable psoriasis (with onset of pustular lesions) was detected to have persistent hypocalcemia which led to the diagnosis of hypoparathyroidism. Subsequently he was found to have hypergonadotrophic hypogonadism, primary adrenal insuffi ciency (compensated), and coeliac disease, thus confi rming the diagnosis of APS I. Psoriasis is very rarely reported in APS I, possibly due to the protective effect of antibodies to Th17 cytokines, which are responsible for the occurrence of candidiasis in this syndrome. However, psoriasis could occur in APS I patients without mucocutaneous candidiasis, who lack these antibodies. In our patient, possible factors aggravating psoriasis include hypocalcemia due to hypoparathyroidism as well as coeliac disease via anti-tissue transglutaminase antibodies. However, defi ning psoriasis as a possible minor component of APS I would require further studies of the autoimmune regulator (AIRE) gene functions.

Síndrome autoinmune poliglandular tipo II: reporte de un caso y revisión de la literatura / Autoinmune polyglandular syndrome type II: a case report and review of literature

El síndrome autoinmune poliglandular (SAP) corresponde a una infrecuente entidad clínica que se reconoce por el compromiso de dos o más glándulas endocrinas basados en mecanismos autoinmunes, siendo posible además la afectación de órganos no endocrinos por esta misma causa. Debido a esto último el término síndrome autoinmune "poliendocrino", antes utilizado frecuentemente, ya no es completamente aceptado para referirse a esta patología. Actualmente existen dos grupos clásicos (SAP tipo I y tipo II) los cuales se clasifican de acuerdo a la edad de presentación de la enfermedad, combinaciones características de órganos comprometidos y patrón hereditario, entre otros. Además se describen otros dos grupos de menor importancia clínica llamados SAP tipo III y IV. A continuación se presenta un caso clínico pediátrico de SAP tipo II diagnosticado en el Hospital Herminda Martín de Chillán y una revisión actualizada de la literatura correspondiente.

Vitiligo y tiroiditis de Hashimoto: asociación o síndrome autoinmune? / Vitiligo and Hashimoto's thyroiditis: an association or an autoimmune syndrome?

Se sospecha de ciertos virus y otros agentes como causantes de la tiroiditis de Hashimoto, siendo bien conocida la asociación con otras entidades inmunes, entre ellas el vitiligo. Una vez disparados los fenómenos inmunológicos se generan anticuerpos contra los antígenos microbianos (o de otro tipo), pero también Ac. contra antígenos propios como los de la estructura tiroidea y la piel. En el vitiligo hay alta incidencia de anticuerpos organoespecíficos, pero también otros anticuerpos no organoespecíficos, sugiriéndose para los síndromes autoinmunes múltiples y las colagenopatías un fuerte parentesco clínico, laboratorial y quizá etiológico. En este caso clínico en particular la tarea del dermatólogo cobró importancia pues toda la investigación generada se debió a la repentina aparición de vitiligo en un paciente con síntomas vagos

Schmidt's syndrome in Saudi Family

We present 3 patients from a Saudi family who are presented with polyglandular autoimmune syndrome type 2. They have Addison's disease with either autoimmune thyroid disease or insulin dependent diabetic mellitus. Although this syndrome is rare, the incidence among Saudi Arabia or the Arab population is not known

Polyglandular autoimmune (PGA) syndromes: report of three cases and review of the literature.

Polyglandular autoimmune (PGA) syndrome is caused by autoimmune process in multiple endocrine glands. This usually results in endocrine gland hypofunction, except for the thyroid gland, in which both hyper or hypofunction may occur. The syndrome can be classified into two types, type I and type II, each with distinct clinical characteristics. We report three cases of PGA syndromes. The first patient had type I PGA syndrome, characterized by hypoparathyrodism, primary adrenal insufficiency and primary ovarian failure. She also had chronic mucocutaneous candidiasis, which is the distinct feature in this syndrome. The second patient had type II PGA syndrome, with primary adrenal insufficiency, Hashimoto's thyroiditis and primary ovarian failure. She also had widespread vitilgo. The last patient also had type II PGA syndrome. She had insulin dependent diabetes mellitus, Graves' disease and alopecia areata.