Angiokeratoma corporis diffusum in a Spanish patient with aspartylglucosaminuria.

Angiokeratoma corporis diffusum (ACD), initially considered to be synonymous with Fabry's disease, represents a well-known cutaneous marker of some other lysosomal enzyme disorders. Aspartylglucosaminuria (AGU) is a rare hereditary disorder mostly affecting the Finnish population, with only a few sporadic patients of non-Finnish origin. To date, only three patients with AGU have been reported with cutaneous lesions of ACD. A 19-year-old Spanish woman presented with a 10-year history of progressive ACD affecting the limbs, buttocks and trunk. After the age of 6 years she had developed progressive mental deterioration, coarse facies and macroglossia with a scrotal appearance. Peripheral blood smears showed many vacuolated lymphocytes. Enzyme analysis in cultured fibroblasts revealed a decreased activity of aspartylglucosaminidase. By the age of 31 years the patient had developed a bipolar psychosis, polycystic ovarian disease and severe impairment of cognitive skills. This is the first case of AGU detected in a Spanish patient presenting with cutaneous lesions of ACD. To our knowledge, macroglossia with a scrotal appearance and polycystic ovarian disease have not been reported in previous cases of AGU.

Idiopathic palmoplantar hidradenitis.

Idiopathic plantar hidradenitis (IPH) is a recently described condition primarily affecting healthy children who develop tender lesions localized to the plantar or lateral aspects of the feet with histologic findings similar to those seen in neutrophilic eccrine hidradenitis (NEH), although with certain notable exceptions including the absence of syringosquamous metaplasia and the presence, in most cases, of neutrophilic abscesses adjacent to eccrine coils. Since its original description, three additional patients have been reported, one with palmoplantar lesions, extending the disease's clinical spectrum to include palmar as well as plantar surfaces. We report on a healthy 8-year-old girl with tender, erythematous nodules on palms and soles and review the literature on this subject.

Halo eczema in melanocytic nevi.

A case of halo eczema in melanocytic nevi is reported. Immunohistochemical techniques using monoclonal antibodies distinguished this condition from halo nevus. The lack of the expression of interleukin 2 receptor by T lymphocytes in the infiltrate suggest that the process is probably different from that of the allergic contact dermatitis.

Utilidad terapeutica de la incision de ovarios por la laparoscopia. / Therapeutical usefulness of ovarian incision by laparoscopy

Se estudian los expedientes clinicos de las pacientes a las cuales se les realizo incision laparoscopica y reseccion en cuna de ovarios de la consulta de esterilidad del ISMM "Dr. Luis Diaz Soto" entre los anos 1972-1977, 15 pacientes mediante incision translaparoscopica, y 20 mediante reseccion en cuna de ovarios por laparotomia, y se analizan los resultados en cuanto a los embarazos logrados. Se senala la diferencia de los resultados de cada uno de los metodos mencionados, tanto al ser usados aisladamente como cuando se asociaron con proovulatorios, se establecieron las conclusiones apropiadas de acuerdo con los resultados obtenidos

Hallazgos laparoscopicos en la paciente esteril y su relacion con la permeabilidad tubaria por laparoscopia. / Laparoscopic findings in the sterile patient and their relationship with tubal permeability by laparoscopy

Se estudian 160 pacientes a las cuales se les practico una laparoscopia como parte de su estudio de esterilidad, en las que se realizo hidrotubacion con contraste (azul de metileno) con el fin de determinar la permeabilidad tubaria, se analizaron los resultados y la probable relacion entre los hallazgos laparoscopicos encontrados y la permeabilidad observada. El factor tubo-peritoneal, el factor uterino y el factor tubario puro, fueron por orden de frecuencia los que mas se asociaron a trastornos de la permeabilidad tubaria, 26 de cada 100 pacientes presentaron obstruccion unilateral o bilateral realiconadas con cambios inflamatorios cronicos de las trompas asociados con adherencias peritoneales y en 19 de cada 100 pacientes coincidio la existencia de miomas uterinos con ausencia de permeabilidad por una o ambas trompas. Se analizan las posibilidades de la laparoscopia con respecto al diagnostico mas preciso del origen de los trastornos de la permeabilidad tubaria y su valor en la evaluacion del pronostico y conducta de estas pacientes