Infantile hemangiomas and structural anomalies: PHACE and LUMBAR syndrome.
Authors: Luu M, Frieden IJ Abstract In a subset of patients, infantile hemangiomas may be associated with structural anomalies. Structural anomalies may affect the upper body (PHACE Syndrome) or the lower body (LUMBAR Syndrome). Since their initial descriptions, improved understanding of these two syndromes has lead to recognition of a broader phenotypic spectrum than initially recognized. This paper aims to provide an updated review of the presentation, associated anomalies, and management of PHACE and LUMBAR syndromes, two well-characterized syndromes associated with IH. PMID: 27607319 [PubMed -...
Source: Seminars in Cutaneous Medicine and Surgery - September 10, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Congenital hemangiomas.
Authors: Boull C, Maguiness SM Abstract Congenital hemangiomas are rare solitary vascular tumors that do not proliferate after birth. They are characterized as either rapidly involuting congenital hemangiomas (RICHs) or noninvoluting congenital hemangiomas (NICHs) based on their clinical progression. NICHs have no associated complications, but are persistent. RICH, while usually asymptomatic, may ulcerate or bleed early in their presentation, but involute quickly during the first few months of life. Hepatic RICHs are not associated with cutaneous RICHs, but may result in high-output cardiac failure due to ...
Source: Seminars in Cutaneous Medicine and Surgery - September 10, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Genetic basis for vascular anomalies.
Authors: Kirkorian AY, Grossberg AL, Püttgen KB Abstract The fundamental genetics of many isolated vascular anomalies and syndromes associated with vascular anomalies have been elucidated. The rate of discovery continues to increase, expanding our understanding of the underlying interconnected molecular pathways. This review summarizes genetic and clinical information on the following diagnoses: capillary malformation, venous malformation, lymphatic malformation, arteriovenous malformation, PIK3CA-related overgrowth spectrum (PROS), Proteus syndrome, SOLAMEN syndrome, Sturge-Weber syndrome, phakomatosis p...
Source: Seminars in Cutaneous Medicine and Surgery - September 10, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Histopathology of vascular anomalies: update based on the revised 2014 ISSVA classification.
Authors: Miller DD, Gupta A Abstract Precise diagnosis of childhood vascular anomalies is challenging, and requires careful correlation of clinical findings, diagnostic imaging, histopathology and genetic analysis. Skin and soft tissue biopsies remain an important element in the complete evaluation of many vascular anomalies included in the revised 2014 International Society for the Study of Vascular Anomalies (ISSVA) classification. Here we present an overview of the light microscopic and immunohistochemical features of the entities in this updated classification scheme, with emphasis on newly-included di...
Source: Seminars in Cutaneous Medicine and Surgery - September 10, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

The clinical spectrum of kaposiform hemangioendothelioma and tufted angioma.
Authors: Croteau SE, Gupta D Abstract Kasposiform hemoangioendothelioma (KHE) and tufted angioma (TA) are classifed as vascular tumors with locally aggressive and benign growth potential, respectively, within the classification schema proposed by the International Society for the Study of Vascular Anomalies. A unique feature of these vascular tumors is the risk of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenia with mild to moderate coagulopathy resulting from intralesional platelet trapping. As with many vascular anomalies, accurate description of clinical course, responses to therapy, and lo...
Source: Seminars in Cutaneous Medicine and Surgery - September 10, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Multifocal vascular lesions.
Authors: Levin LE, Lauren CT Abstract Multifocal vascular lesions are important to recognize and appropriately diagnose. Generally first noticed on the skin, multifocal vascular lesions may have systemic involvement. Distinguishing among the different types of multifocal vascular lesions is often based on clinical features; however, radiological imaging and/or biopsy are frequently needed to identify distinct features and guide treatment. Knowledge of the systemic associations that can occur with different vascular anomalies may reduce life-threatening complications, such as coagulopathy, bleeding, cardiac...
Source: Seminars in Cutaneous Medicine and Surgery - September 10, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Overgrowth syndromes with vascular malformations.
Authors: Hagen SL, Hook KP Abstract This review provides a clinically-oriented summary of the most commonly encountered overgrowth syndromes associated with vascular malformations. This manuscript will outline morphologic features, clinical evaluation and management of this complex group of patients. Recent genetic advances have aided in classification and help to explain overlapping clinical features in many cases. PMID: 27607325 [PubMed - in process] (Source: Seminars in Cutaneous Medicine and Surgery)
Source: Seminars in Cutaneous Medicine and Surgery - September 10, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Vascular anomalies: differential diagnosis and mimickers.
Authors: Garzon MC, Weitz N, Powell J Abstract Vascular anomalies are very common in children and encompass a wide spectrum of diseases. Many vascular anomalies can be mistaken for infantile hemangioma (IH). In addition, there is a variety of rare disorders including benign and malignant tumors that may mimic IH and other types of vascular anomalies. Understanding the clinical features, natural history, and typical clinical course of different types of vascular anomalies is essential in order to make the correct diagnosis and guide management. Radiologic imaging plays an important role in establishing the ...
Source: Seminars in Cutaneous Medicine and Surgery - September 10, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Medical management of vascular anomalies.
Authors: Trenor CC Abstract We have entered an exciting era in the care of patients with vascular anomalies. These disorders require multidisciplinary care and coordination and dedicated centers have emerged to address this need. Vascular tumors have been treated with medical therapies for many years, while malformations have been historically treated with endovascular and operative procedures. The recent serendipitous discoveries of propranolol and sirolimus for vascular anomalies have revolutionized this field. In particular, sirolimus responses are challenging the dogma that vascular malformations are n...
Source: Seminars in Cutaneous Medicine and Surgery - September 10, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

New Therapies for Psoriasis.
Authors: Strober BE Abstract Among the newer medications for treating psoriasis are the interleukin-17A inhibitor secukinumab and the phosphodiesterase 4 inhibitor apremilast. Secukinumab offers a level of efficacy greater than that of the tumor necrosis factor-α inhibitor adalimumab. Apremilast is associated with lower levels of efficacy than the biologic therapies for psoriasis. Apremilast may cause diarrhea and nausea and is associated with weight loss and rare instances of depression. Semin Cutan Med Surg 35(supp4):S71-S73. PMID: 27602700 [PubMed - as supplied by publisher] (Source: Seminars i...
Source: Seminars in Cutaneous Medicine and Surgery - September 8, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Translating the Science of Psoriasis.
Authors: Gordon KB Abstract Knowledge about the pathophysiology of psoriasis has evolved substantially in recent years, since the identification of the T helper 17 (Th17) cells. Cytokines produced by these cells appear to play major roles in psoriatic inflammation. The cytokine interleukin (IL)-23 appears to promote regulatory T cells to differentiate into Th17 cells. Available and investigational therapies act on targets within these pathways. Semin Cutan Med Surg 35(supp4):S64. PMID: 27603734 [PubMed - as supplied by publisher] (Source: Seminars in Cutaneous Medicine and Surgery)
Source: Seminars in Cutaneous Medicine and Surgery - September 8, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Small Molecules for Psoriasis.
Authors: Leonardi CL Abstract Acitretin is an older, oral, non-immunosuppressive medication for the treatment of psoriasis. Tofacitinib is an oral Janus kinase inhibitor that has been studied for use in psoriasis. Each offers efficacy in certain settings and patient types but carries substantial safety risks. Semin Cutan Med Surg 35(supp4):S65-S66. PMID: 27604063 [PubMed - as supplied by publisher] (Source: Seminars in Cutaneous Medicine and Surgery)
Source: Seminars in Cutaneous Medicine and Surgery - September 8, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Introduction.
Authors: Kerdel FA PMID: 27551697 [PubMed - in process] (Source: Seminars in Cutaneous Medicine and Surgery)
Source: Seminars in Cutaneous Medicine and Surgery - August 25, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Antibodies in the Treatment of Psoriasis: IL-12/23 p40 and IL-17a.
This article reviews the data documenting the efficacy and safety of three types of biologics. PMID: 27551698 [PubMed - in process] (Source: Seminars in Cutaneous Medicine and Surgery)
Source: Seminars in Cutaneous Medicine and Surgery - August 25, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research

Update on TNF Inhibitors in Dermatology.
Authors: Sobell JM Abstract Emerging data describe new potential indications for tumor necrosis factor (TNF) inhibitors in dermatology, including pediatric psoriasis and hidradenitis suppurativa. New biosimilar TNF agents are in late stages of development and may be available in the United States in the near future. Biosimilar agents are similar but not identical to available TNF inhibitors, and approval requires extensive analytic, toxicity, pharmacokinetic, pharmacodynamic, and clinical testing. Semin Cutan Med Surg 35(supp6):S104-S106. PMID: 27537073 [PubMed - as supplied by publisher] (Source: ...
Source: Seminars in Cutaneous Medicine and Surgery - August 19, 2016 Category: Dermatology Tags: Semin Cutan Med Surg Source Type: research