Loreto Carrasco

Postirradiation pseudosclerodermatous panniculitis.

Pseudosclerodermatous panniculitis is an unusual variant of panniculitis that results as a complication of megavoltage radiotherapy. Four women developed this unusual entity on the anterior chest and abdominal skin after receiving megavoltage therapy for either breast carcinoma or painful bone metastases from breast carcinoma. Histopathologically, the epidermis and dermis of the involved area showed little or no evidence of radiodermatitis. The main findings were confined to the subcutaneous tissue and consisted of thickened, sclerotic septa composed of both thick and thin collagen bundles, and a lobular panniculitis characterized by lipophagic granulomas and scattered lymphocytes and plasma cells. Additionally, one of the cases showed markedly dilated vascular spaces with the appearance of lymphatics in the upper part of the dermis. Pseudosclerodermatous panniculitis after irradiation is an unusual cutaneous complication of megavoltage radiotherapy that should be distinguished from subcutaneous metastatic disease, cellulitis, or connective tissue diseases involving the subcutaneous fat. The differential diagnosis can be established on the basis of the characteristic histopathologic features of postirradiation pseudosclerodermatous panniculitis.

Olmsted syndrome: report of a case with study of the cellular proliferation in keratoderma.

Olmsted syndrome is a rare disorder that consists of sharply marginated keratoderma of the palms and soles, constriction of digits and toes that may result in spontaneous amputation of the distal phalanges, hyperkeratotic plaques around the body orifices, onychodystrophy, and other less common cutaneous and extracutaneous anomalies. Although some patients had other affected family members, most cases of Olmsted syndrome seem to be of sporadic occurrence. We describe a patient with the characteristic features of Olmsted syndrome. The symptoms consisted of diffuse transgrediens palmoplantar keratoderma and keratotic plaques around the mouth and nose. Our patient also had the associated anomalies of hyperhidrosis of the palms and soles and congenital deaf-mutism. Histopathologic study of the keratoderma demonstrated epidermal hyperplasia with acanthosis, papillomatosis, and orthokeratotic hyperkeratosis. Immunohistochemical study showed more basal and suprabasal keratinocytes of the epidermis with immunoreactivity for Ki-67 marker when compared with the keratinocytes of the epidermis of the adjacent non-involved skin. These results support the notion that Olmsted syndrome is a hyperproliferative disorder of the epidermis.

Acral arteriovenous tumor developed within a nevus flammeus in a patient with Sturge-Weber syndrome

The Sturge-Weber syndrome consists of a large facial nevus flammeus in the distribution of the ophthalmologic division of the trigeminal nerve accompanied by ipsilateral leptomeningeal angiomatosis. Usually, when angiomatous nodules develop in a nevus flammeus of a patient with Sturge-Weber syndrome they are pyogenic granulomas. We describe an acral arteriovenous tumor developed within the nevus flammeus of a patient with Sturge-Weber syndrome. To our knowledge, acral arteriovenous tumor has not been previously described in the cutaneous vascular malformation of patients with Sturge-Weber syndrome. The development of acral arteriovenous tumor within the vascular malformation of a nevus flammeus in this patient with Sturge-Weber syndrome probably results from a vascular proliferation secondary to underlying arteriovenous shunts.

Chloracne: histopathologic findings in one case

Background: Chloracne is an acneiform eruption due to poisoning by halogenated aromatic compounds having a specific molecular shape. This condition is always a symptom of systemic poisoning by chemical chloracnegens and not just a cutaneous disorder.

Miasis forunculoide: descripción de dos casos con estudio histológico de las diferentes larvas

Resumen —Miasis es un termino que significa la infestacion de organos y tejidos de vertebrados por dipteros, habitualmente moscas. Presentamos dos casos de miasis forunculoide, poco frecuentes en nuestro pais, ya que las especies de moscas que producen este tipo de miasis no habitan en Espana. El primero se trataba de una miasis producida por Cordylobia anthropophaga o mosca tumbu que habita en Africa, en el sur del Sahara. El segundo de los casos estaba producido por Dermatobia hominis, conocida como torsalo, berne o moscardon de los humanos, comun en America Central y Sudamerica. Las lesiones consistian en nodulos cutaneos, con ulceracion superficial de la que emergian las larvas. Estas larvas fueron estudiadas histopatologicamente, prestando especial atencion a las caracteristicas que permiten diferenciar las dos especies.

Enfermedad de Grover en un paciente con insuficiencia renal crónica en hemodiálisis

Resumen —Se presenta un caso de enfermedad de Grover, con patron histopatologico tipo enfermedad de Darier, en un paciente varon de 69 anos de edad con antecedentes personales de insuficiencia renal cronica secundaria a un carcinoma urotelial multicentrico de larga evolucion que habia sido tratado con sucesivas intervenciones quirurgicas. El paciente referia que el inicio de las lesiones cutaneas se habia producido poco tiempo despues del comienzo del tratamiento de hemodialisis. Se discute la posible implicacion de la insuficiencia renal cronica y/o la hemodialisis en la patogenia de la enfermedad de Grover.

Melanocitosis dérmica de la parte alta de la espalda

Resumen —Se han descrito numerosas variantes clinicas de melanocitosis dermica que incluyen el nevo azul, la mancha mongolica, el nevo de Ota y el nevo de Ito, asi como otros tipos menos frecuentes que se denominan genericamente melanocitosis dermicas atipicas. Presentamos el caso de una melanocitosis dermica persistente adquirida que afecta la region interescapular de un paciente de raza blanca y 44 anos de edad, y que corresponderia a la denominada melanocitosis dermica de la parte alta de la espalda.

Síndrome de Schnitzler

Resumen —El sindrome de Schnitzler se define como una constelacion unica de signos clinicos y biologicos que incluye la presencia de urticaria cronica, fiebre intermitente, dolores oseos, artralgia o artritis y una gammapatia monoclonal de tipo IgM. Presentamos el caso de una paciente con brotes diarios de lesiones urticarianas evanescentes muy refractarias al tratamiento con antihistaminicos y esteroides orales, asociadas a picos febriles y dolores oseos. La paciente presentaba leucotrombocitosis, anemia asociada a trastorno inflamatorio cronico y aumento de la velocidad de sedimentacion globular. El proteinograma revelo la presencia de una paraproteinemia monoclonal de tipo IgM con cadenas ligeras kappa en la inmunofijacion. Mediante tecnicas de radiodiagnostico se identificaron focos de osteocondensacion en la columna vertebral. El estudio histopatologico de las lesiones cutaneas demostro hallazgos de urticaria sin vasculitis. De acuerdo con estos hallazgos clinicos, analiticos y radiologicos se establecio el diagnostico de sindrome de Schnitzler.