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.

[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.

[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.

[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.

[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.

[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.