Isabel Rodríguez-Blanco

Essential Progressive Telangiectasia

by well-established clinical criteria.5 The abnormality is located on chromosome 17 and exhibits autosomal dominant inheritance. Most cases of Sturge-Weber syndrome are sporadic, but a familial distribution has been reported. As a result, the syndrome is believed to follow paradominant inheritance, such that the individual is heterozygotic for this inherited characteristic and phenotypically normal, and will only suffer from the disease should further mutation occur during the early phases of embryonic development.6 In neurofibromatosis type 1 the underlying alteration encodes a protein, neurofibromin, that is responsible for the pathogenesis of the condition. Sturge-Weber syndrome is explained by increased expression of fibronectin, which regulates angiogenesis and constitutes the cerebral response to chronic ischemic damage. Therefore, there does not appear to be a relationship between the 2 neurocutaneous syndromes in either pathogenesis, transmission, or the underlying genetic defect. We raise the question of whether this case could be the result of the simultaneous occurrence of both process—an extraordinarily rare occurrence. Another explanation could lie in the pathogenesis of neurofibromatosis itself, since numerous articles link this condition with angioma and other vascular abnormalities.7 Thirdly, it is possible that this could correspond to the most common neurocutaneous syndrome of all, Pascual Castroviejo type II syndrome, which encompasses neurologic abnormalities and various vascular abnormalities, including angioma. Reports of Pascual Castroviejo II syndrome include a description of a patient with neurofibromatosis type 1 and cutaneous and hepatic hemangioma.8

Dermatitis en párpados

Resumen —Introduccion. La dermatitis de los parpados constituye un proceso inflamatorio frecuente, ya que la piel que los recubre es especialmente susceptible a la accion de agentes irritantes y/o alergenicos. Material y metodos Se trata de un estudio retrospectivo de pacientes con dermatitis palpebral entre los anos 1998-2003 a los que se han realizado pruebas epicutaneas. Resultados En 1.976 pacientes atendidos en consulta, 84 individuos (4,25 %) presentaron dermatitis palpebral. La principal causa fue la dermatitis alergica de contacto (70,23 %), seguida por otras como la dermatitis irritativa (25 %), seborreica (1,19 %) o atopica (1,19 %). Conclusiones En nuestro centro, coincidiendo con otros estudios, la principal causa de dermatitis palpebral en pacientes remitidos a la unidad de contacto la constituye la dermatitis alergica de contacto, en la cual destacan los cosmeticos como principal grupo de alergenos.

Ulcerated dermatofibroma with osteoclast-like giant cells

Background: Ulceration and osteoclast‐like giant cells are two pathological features uncommonly seen in dermatofibromas. To our knowledge, the presence of these features has not been previously described within the same lesion.

Necrolisis epidérmica tóxica por lamotrigina

Toxic epidermal necrolysis (TEN) is a serious entity consisting of a generalized eruption with the formation of flaccid blisters. It affects at least 30% of the cutaneous surface and is usually related to the prior ingestion of medications. We describe TEN symptoms related to the introduction of lamotrigine, an anti-epileptic drug prescribed in this case for the prevention of migrainous episodes.

P76 Allergic contact dermatitis vs. psoriasis

Background:  Psoriasis prevalence in the Spanish population is around 1.4%(2000). The last epidemiological enquire of the GEIDC (2001) showed that 55.11% of the patients revised in specialized units presented Allergic Contact Dermatitis (ACD). Concomitance of both processes has been widely studied being the opinions of the different authors controversial. Palmo‐plantar psoriasis seems to be the most frequent type in patients presenting delayed hypersensitivity.

Malignant Degeneration of Linear Hidroacanthoma Simplex

We describe an 88-year-old woman with various lesions in the right submammary region that had remained stable for more than 40 years. She reported rapid growth and ulceration of one of the lesions in the past year. The examination showed a firm tumor of diameter 7 cm below the right breast. The surface of the tumor was keratotic at the periphery and ulcerated in the middle with foul-smelling serous exudate. Adjacent to the lesion, there were various smaller erythematous brownish tumors with a velvety surface, and with a linear distribution (Figure 1). No enlarged local or regional lymph nodes were palpated. Laboratory workup, chest x-ray, electrocardiogram, bilateral mammography, and right axillary ultrasound were all normal. An incisional biopsy of the larger tumor and another biopsy of one of the adjacent lesions were taken. In the first case, the hematoxylin-eosin stain showed irregular, anastomosed islets composed of intraepidermal tumor cells, some of them pigmented, with a clearer cytoplasm than the surrounding keratinocytes. Abundant atypical cells with large, irregular, hyperchromatic nuclei were observed inside the tumor masses. In some sections, cystic spaces within these nests of basaloid cells could be seen. The epidermis presented hyperkeratosis, foci of parakeratosis, and irregular acanthosis (Figure 2 A and B). A biopsy of the smaller lesion showed well-defined nests of uniform cuboidal cells with rounded, basophilic nuclei showing no atypia, and with cystic structures in the interior (Figure Malignant Degeneration of Linear Hidroacanthoma Simplex

Linfocitoma cutáneo en labio: a propósito de un caso tratado con cloroquina

Resumen —Se presenta el caso de un paciente varon de 42 anos con una lesion tumoral en labio superior cuyo estudio dermatopatologico fue compatible con el diagnostico de linfocitoma cutaneo. Dado el fracaso de la terapeutica local previa empleada, se administro cloroquina en dosis de 500 mg/dia, que indujo la remision de la lesion. Destacamos la capacidad inmunomoduladora de los antipaludicos de sintesis en un caso en el que los corticoides locales no fueron efectivos.