Liquen plano familiar / Familial lichen planus

ResumenEl liquen plano familiar es una forma rara de liquen plano que se caracteriza por inicio precoz, curso prolongado, afectación de mucosa oral y formasclínicas atípicas. Se han investigado posibles factores ambientales o genéticos en su patogénesis. Presentamos tres miembros de una familia que padecenliquen plano, con características clínicas típicas de la variante familiar. Se realizó un tipaje de HLA en dos miembros afectos. No se identificó ningúnfactor ambiental ni infeccioso

Familial lichen planus is a rare form of lichen planus characterized by an early onset, prollonged course, involvement of oral mucosa and atypical clinicalforms. Different enviromental or genetic factors have been investigated in its pathogenesis. Herewith three members of a familiy affected withlichen planus, with typical clinic features of the familiar variant are reported. HLA typing was performed made in two affected members. No enviromentalnor infectious agent was identified

Reticular telangiectatic erythema associated with an implantable cardioverter defibrillator.

Reticular telangiectatic erythema (RTE) is a skin reaction associated with implantable cardiac devices (ie, pacemakers and cardioverter defibrillators). We present a patient who developed an erythematous patch over the implantable cardioverter defibrillator site. We discuss the clinical features, histologic findings, and patch testing of this entity.

[Stevens-Johnson syndrome and toxic epidermal necrolysis]. / Síndrome de Stevens-Johnson y necrólisis epidérmica tóxica: experiencia clínica y revisión de la literatura especializada.

INTRODUCTION: The aim of this work is to reflect the clinical experience of the Dermatology Department of Hospital General in Valencia with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) or Lyell's syndrome over the last 15 years. METHODS: Data on epidemiology, likely causal agents, complementary tests, concomitant pathologies, management, evolution and complications was gathered through a retrospective study. RESULTS: Thirteen patients were included, with a mean age of 53 years. The most frequently involved drugs were antibiotics (50 %), followed by anti-convulsants (16.6 %). The mucous membranes were involved in 84.6 % of the cases. 61.5 % of the patients presented with systemic symptoms. The most frequent laboratory finding was hypoproteinemia. Corticosteroids were used in 69 % of the cases, and intravenous immunoglobulins in 15 %. Two oncological patients with a diagnosis of TEN died (15 % overall mortality). CONCLUSIONS: SJS and TEN are infrequent mucocutaneous reactions, often drug induced, with significant associated morbidity and mortality. Their pathogenesis is still partially unknown, and no specific treatment has been proven to be clearly beneficial; therefore, the best treatment consists of early diagnosis, the withdrawal of the suspect drug and support therapy.

[Pseudovascular squamous cell carcinoma]. / Carcinoma epidermoide pseudovascular.

The adenoid form of squamous cell carcinoma is a neoplasm that is characterized by the fact that it presents a pseudoglandular pattern in the histological study. The biological phenomenon that explains this histological pattern is acantholysis; when the latter is massive, the tumor may even mimic a vascular proliferation, and is known as pseudovascular squamous cell carcinoma. This tumor has the clinical characteristics of a squamous cell carcinoma, but histologically, it may mimic an angiosarcoma. Most cases of pseudovascular squamous cell carcinoma of the skin have a poor prognosis.

Carcinoma epidermoide pseudovascular / Pseudovascular squamous cell carcinoma

La forma adenoide del carcinoma epidermoide es una neoplasia que se caracteriza por presentar un patrón pseudoglandular en el estudio histológico. El fenómeno biológico que explica este patrón histológico es la acantolisis que, cuando es masiva, puede incluso simular una proliferación vascular, denominándose carcinoma epidermoide pseudovascular. Este tumor tiene las características clínicas de un carcinoma epidermoide, pero histológicamente puede simular un angiosarcoma. La mayoría de los casos de carcinoma epidermoide pseudovascular cutáneo presentan un mal pronóstico

The adenoid form of squamous cell carcinoma is a neoplasm that is characterized by the fact that it presents a pseudoglandular pattern in the histological study. The biological phenomenon that explains this histological pattern is acantholysis; when the latter is massive, the tumor may even mimic a vascular proliferation, and is known as pseudovascular squamous cell carcinoma. This tumor has the clinical characteristics of a squamous cell carcinoma, but histologically, it may mimic an angiosarcoma. Most cases of pseudovascular squamous cell carcinoma of the skin have a poor prognosis

[Papillary dermal elastolysis similar to pseudoxanthoma elasticum]. / Elastolisis dérmica papilar similar a pseudoxantoma elástico.

Papillary dermal elastolysis similar to pseudoxanthoma elasticum is an elastolytic disorder characterized by cutaneous lesions on the neck and in the supraclavicular region that are clinically similar to pseudoxanthoma elasticum, with no systemic complications. The histological examination shows a loss of elastic fibers in the papillary dermis. We report a case in a 76-year-old woman with typical lesions on the neck.

Síndrome de Stevens-Johnson y necrólisis epidérmica tóxica: experiencia clínica y revisión de la literatura especializada / Stevens-Johnson syndrome and toxic epidermal necrolysis

Introducción. El objetivo de este trabajo es reflejar la experiencia clínica del Servicio de Dermatología del Hospital General de Valencia en el síndrome de Stevens-Johnson (SSJ) y necrólisis epidérmica tóxica (NET) o síndrome de Lyell durante los últimos 15 años. Métodos. A través de un estudio retrospectivo se recogieron datos sobre epidemiología, agentes causales probables, pruebas complementarias, patologías concomitantes, tratamiento, evolución y complicaciones. Resultados. Se incluyeron 13 pacientes con edad media de 53 años. Los fármacos más frecuentemente implicados fueron los antibióticos (50 %) seguidos de los anticomiciales (16,6 %). Las mucosas se afectaron en el 84,6 % de los casos. El 61,5 % de los pacientes presentó clínica sistémica. El hallazgo de laboratorio más frecuente fue la hipoproteinemia. Los corticoides se emplearon en el 69 % de casos y las inmunoglobulinas intravenosas, en el 15 %. Dos pacientes oncológicos con diagnóstico de NET fallecieron (15 % de mortalidad global). Conclusiones. El SSJ y la NET son reacciones mucocutáneas poco frecuentes a menudo inducidas por fármacos que asocian una importante morbimortalidad. La patogenia permanece en parte desconocida y ningún tratamiento específico se ha demostrado claramente beneficioso, por lo que la mejor terapéutica consiste en el diagnóstico precoz, la retirada del fármaco sospechoso y la terapia de soporte

Introduction. The aim of this work is to reflect the clinical experience of the Dermatology Department of Hospital General in Valencia with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) or Lyell's syndrome over the last 15 years. Methods. Data on epidemiology, likely causal agents, complementary tests, concomitant pathologies, management, evolution and complications was gathered through a retrospective study. Results. Thirteen patients were included, with a mean age of 53 years. The most frequently involved drugs were antibiotics (50 %), followed by anti-convulsants (16.6 %). The mucous membranes were involved in 84.6 % of the cases. 61.5 % of the patients presented with systemic symptoms. The most frequent laboratory finding was hypoproteinemia. Corticosteroids were used in 69 % of the cases, and intravenous immunoglobulins in 15 %. Two oncological patients with a diagnosis of TEN died (15 % overall mortality). Conclusions. SJS and TEN are infrequent mucocutaneous reactions, often drug induced, with significant associated morbidity and mortality. Their pathogenesis is still partially unknown, and no specific treatment has been proven to be clearly beneficial; therefore, the best treatment consists of early diagnosis, the withdrawal of the suspect drug and support therapy

[Treatment of severe refractory pemphigus vulgaris with rituximab]. / Tratamiento de pénfigo vulgar grave resistente con rituximab.

Pemphigus vulgaris is a potentially fatal autoimmune bullous disease. High doses of immunosuppressive drugs are used in managing severe cases of pemphigus. Rituximab, an anti-CD20 monoclonal antibody, has proven to be effective in patients with refractory pemphigus vulgaris and pemphigus foliaceus. We review cases of pemphigus vulgaris and pemphigus foliaceus not associated with lymphoma that were treated with rituximab, and we report a new case of severe refractory pemphigus vulgaris successfully treated with rituximab.

Elastolisis dérmica papilar similar a pseudoxantoma elástico / Papillary dermal elastolysis similar to pseudoxanthoma elasticum

La elastolisis dérmica papilar similar a pseudoxantoma elástico es un trastorno elastolítico caracterizado por lesiones cutáneas en el cuello y en la región supraclavicular que clínicamente se asemejan al pseudoxantoma elástico, sin complicaciones sistémicas. El examen histológico muestra una pérdida de fibras elásticas en la dermis papilar. Comunicamos un caso en una mujer de 76 años con lesiones típicas en el cuello

Papillary dermal elastolysis similar to pseudoxanthoma elasticum is an elastolytic disorder characterized by cutaneous lesions on the neck and in the supraclavicular region that are clinically similar to pseudoxanthoma elasticum, with no systemic complications. The histological examination shows a loss of elastic fibers in the papillary dermis. We report a case in a 76-year-old woman with typical lesions on the neck

Tratamiento de pénfigo vulgar grave resistente con rituximab / Treatment of severe refractory pemphigus vulgaris with rituximab

El pénfigo vulgar es una enfermedad ampollosa autoinmune potencialmente mortal. En el tratamiento de los casos graves de pénfigo se utilizan fármacos inmunosupresores en dosis altas. Rituximab, un anticuerpo monoclonal anti-CD20, ha demostrado eficacia en pacientes con pénfigo vulgar y pénfigo foliáceo refractarios. Revisamos los casos de pénfigo vulgar y de pénfigo foliáceo tratados con rituximab no asociados a linfoma, y comunicamos un nuevo caso de pénfigo vulgar grave refractario tratado con éxito con rituximab

Pemphigus vulgaris is a potentially fatal autoimmune bullous disease. High doses of immunosuppressive drugs are used in managing severe cases of pemphigus. Rituximab, an anti-CD20 monoclonal antibody, has proven to be effective in patients with refractory pemphigus vulgaris and pemphigus foliaceus. We review cases of pemphigus vulgaris and pemphigus foliaceus not associated with lymphoma that were treated with rituximab, and we report a new case of severe refractory pemphigus vulgaris successfully treated with rituximab

Escleredema de Buschke asociado a diabetes mellitus. Estudio de cuatro casos / Scleredema of Buschke associated with diabetes mellitus. Study of four cases

El escleredema de Buschke se caracteriza por un engrosamiento de la dermis con depósito de mucina entre las fibras de colágeno, que se manifiesta como un endurecimiento de la piel, predominantemente en la mitad superior del tronco. Se ha relacionado con diabetes mellitus de larga evolución y mal controlada, gammapatías monoclonales e infecciones estreptocócicas. Se estudiaron los pacientes con el diagnóstico clínico de escleredema confirmado mediante estudio histopatológico y asociado a diabetes mellitus. Se revisaron las enfermedades asociadas, las características clínicas e histopatológicas, el curso evolutivo y la respuesta al tratamiento. El escleredema se caracteriza por un endurecimiento cutáneo que afecta a la mitad superior del tronco. En una gran proporción de casos se asocia a diabetes mellitus mal controlada, de curso prolongado durante años y con escasa respuesta al tratamiento

Scleredema of Buschke is characterized by a thickening of the dermis with mucin deposits among the collagen fibers, which manifests as a hardening of the skin, predominantly on the upper trunk. It has been associated with long-standing, poorly controlled diabetes mellitus, monoclonal gammopathies and streptococcus infections. We studied patients with a clinical diagnosis of scleredema confirmed by means of a histopathological study, and associated with diabetes mellitus. We reviewed associated diseases, clinical and histopathological characteristics, evolution and response to treatment. Scleredema is characterized by hardening of the skin, affecting the upper trunk. A large proportion of cases are associated with long-standing, poorly controlled diabetes mellitus, with little response to treatment

[Scleredema of Buschke associated with diabetes mellitus. Study of four cases]. / Escleredema de Buschke asociado a diabetes mellitus. Estudio de cuatro casos.

Scleredema of Buschke is characterized by a thickening of the dermis with mucin deposits among the collagen fibers, which manifests as a hardening of the skin, predominantly on the upper trunk. It has been associated with long-standing, poorly controlled diabetes mellitus, monoclonal gammopathies and streptococcus infections. We studied patients with a clinical diagnosis of scleredema confirmed by means of a histopathological study, and associated with diabetes mellitus. We reviewed associated diseases, clinical and histopathological characteristics, evolution and response to treatment. Scleredema is characterized by hardening of the skin, affecting the upper trunk. A large proportion of cases are associated with long-standing, poorly controlled diabetes mellitus, with little response to treatment.

Angioedema asociado a Iosartán / Angioedema asociated with Losartan

El losartán es un fármaco antihipertensivo del grupo de los antagonistas de los receptores de angiotensina. Estos medicamentos actúan sobre el sistema renina-angiotensina bloqueando el receptor AT1 de la angiotensina. De forma ocasional, se han descrito casos de angioedema asociados a estos medicamentos. Presentamos un paciente que desarrolló un angioedema secundario a la ingesta de losartán por hipertensión arterial (AU)