Actas dermo-sifiliográficas
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Actas Dermosifiliogr · Oct 2013
ReviewUpdate on the classification and treatment of localized scleroderma.
Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. However, classification is difficult because the boundaries between the different types of morphea are blurred and different entities frequently overlap. ⋯ With certain exceptions, the disorder does not have serious systemic repercussions, but it can cause considerable morbidity. In the case of lesions affecting the head, neurological and ocular complications may occur. There is no really effective and universal treatment so it is important to make a correct assessment of the extent and severity of the disease before deciding on a treatment approach.
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Actas Dermosifiliogr · Sep 2013
Review Case ReportsPurpura fulminans associated with Streptococcus pneumoniae septicemia in an asplenic pediatric patient.
Purpura fulminans is a rapidly progressive syndrome of small-vessel thrombosis and hemorrhagic necrosis of the skin accompanied by disseminated intravascular coagulation. We describe a case of Streptococcus pneumoniae septicemia in an asplenic 5-year-old boy on oral tacrolimus, with a past medical history of multivisceral organ transplantation and subsequent development of purpura fulminans on his chest and distal extremities. ⋯ Our patient had both, which likely increased his susceptibility, and he responded well to antimicrobial therapy in addition to prophylactic coverage in the setting of his immunosuppression. We review the literature for similar cases due to S. pneumoniae in the pediatric population and discuss the etiology and treatment of purpura fulminans.
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Actas Dermosifiliogr · Sep 2013
ReviewAdvances in the diagnosis and treatment of tumor necrosis factor receptor-associated periodic syndrome.
Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is a rare autosomal dominant disease included in the group of autoinflammatory syndromes. It is characterized by recurrent episodes of fever and inflammation in different regions of the body. The main clinical manifestations are myalgia, migratory erythematous rash, periorbital edema, and abdominal pain. ⋯ Tumor necrosis factor inhibitors and corticosteroids are the most widely used treatments. In recent years, significant advances have been made in the diagnosis and treatment of TRAPS, thanks to a better understanding of its pathogenesis. Dermatologists must be aware that the skin manifestations of TRAPS are particularly important, as they are often diagnostic.
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Actas Dermosifiliogr · Jul 2013
Infantile hemangiomas treated by sequential application of pulsed dye laser and Nd:YAG laser radiation: a retrospective study.
Infantile hemangiomas are the most common benign tumor in children. They have 3 phases of development: a proliferative phase, an involuting phase, and involution. Although active treatment is often not required, it is necessary in some cases. Of the possible treatments for hemangiomas, lasers have been shown to be effective in all phases of development. We report our experience with dual-wavelength sequential pulses from a pulsed dye laser and an Nd:YAG laser. ⋯ We believe that treatment with dual-wavelength light from a pulsed dye laser and a Nd:YAG laser is a viable treatment option for infantile hemangiomas when first-line therapies are ineffective or contraindicated.
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The term autosomal recessive congenital ichthyosis (ARCI) refers to a group of rare disorders of keratinization classified as nonsyndromic forms of ichthyosis. This group was traditionally divided into lamellar ichthyosis (LI) and congenital ichthyosiform erythroderma (CIE) but today it also includes harlequin ichthyosis, self-healing collodion baby, acral self-healing collodion baby, and bathing suit ichthyosis. ⋯ ARCI is genetically highly heterogeneous and has been associated with 6 genes to date: TGM1, ALOXE3, ALOX12B, NIPAL4, CYP4F22, and ABCA12. In this article, we review the current knowledge on ARCI, with a focus on clinical, histological, ultrastructural, genetic, molecular, and treatment-related aspects.