Clinics in Dermatology RSS feed: Current Issue. Clinics in Dermatology brings you the most practical and comprehensive information on the treatment and care of skin disorders. Each issue features a Guest Editor and is devoted to a single timely topic relating to clinical dermatology. Clinics in Dermatology provides information that is... • Clinically oriented -- from evaluation to treatment, Clinics in Dermatology covers what is most relevant to you in your practice. • Authoritative -- world-renowned experts in the field assure the high-quality and currency of each issue by reporting on their areas of expertise. • Well-illustrated -- each issue is complete with photos, drawings and diagrams to illustrate points and demonstrate techniques. Now affiliated with the International Academy of Cosmetic Dermatology (IACD). Visit the IACD web site at: www.dermato.med.br/iacd for more information. http://www.cidjournal.com/?rss=yesElsevier Inc.en © 2011 Published by Elsevier Inc. All rights reserved. Clinics in Dermatology0738-081X301January 2012 © 2011 Published by Elsevier Inc. All rights reserved. healthpermissions@elsevier.comhttp://www.cidjournal.com/article/PIIS0738081X11002999/abstract?rss=yesEditorial Board10.1016/S0738-081X(11)00299-9Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-XIFCIFChttp://www.cidjournal.com/article/PIIS0738081X11003026/abstract?rss=yesTable of Contents10.1016/S0738-081X(11)00302-6Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-Xiiiivhttp://www.cidjournal.com/article/PIIS0738081X11000800/abstract?rss=yesIn this second of a two-part issue, Bullous Disease, we address those diseases associated with subepidermal blistering. The bullous pemphigoid (BP) group represents subepidermal autoimmune blistering diseases with three clinical variants: BP, mucous membrane pemphigoid (MMP), and pemphigoid gestationis. The autoimmune response in BP is directed against two hemidesmosomal antigens: BP180 (BP antigen 2 or collagen XVII) and BP230 (BP antigen 1). Multiple antigens are included in MMP: BP180, BP230, laminin-5, laminin-6, and the integrin B4 subunit. Autoantibodies in pemphigoid gestationis are mainly reactive with BP180.Commentary: Subepidermal Bullous Skin DiseasesJasna Lipozenčić, Branka Marinović10.1016/j.clindermatol.2011.03.004Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X12http://www.cidjournal.com/article/PIIS0738081X11000812/abstract?rss=yesAbstract: Bullous pemphigoid (BP) constitutes the most frequent autoimmune subepidermal blistering disease. It is associated with autoantibodies directed against the BP antigens 180 (BP180, BPAG2) and BP230 (BPAG1-e). The pathogenicity of anti-BP180 antibodies has been convincingly demonstrated in animal models. The clinical features of BP are extremely polymorphous. The diagnosis of BP critically relies on immunopathologic findings. The recent development of novel enzyme-linked immunosorbent assays has allowed the detection of circulating autoantibodies with relatively high sensitivity and specificity. Although potent topical steroids have emerged in the past decade as first-line treatment of BP, management of the disease may be challenging.Bullous pemphigoid: From the clinic to the benchGiovanni Di Zenzo, Rocco della Torre, Giovanna Zambruno, Luca Borradori10.1016/j.clindermatol.2011.03.005Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X316http://www.cidjournal.com/article/PIIS0738081X11000824/abstract?rss=yesAbstract: The presence of one autoimmune disorder helps lead to the discovery of other autoimmune conditions. It is thought that diseases in which autoimmunity is a feature tend to be associated together more often than one can ascribe to chance. A variety of diseases have been implicated in the onset of intraepidermal and subepidermal autoimmune diseases. The presence of one autoimmune disease should alert the physician to watch for a second immunologic disorder. A list of autoimmune bullous diseases associations includes autoimmune bullous diseases, pemphigus, pemphigoid, epidermolysis bullosa acquisita, dermatitis herpetiformis (Duhring), linear immunoglobulin A disease, and multiple autoimmune syndrome.Autoimmune bullous diseases associationsSuzana Ljubojevic, Jasna Lipozenčić10.1016/j.clindermatol.2011.03.006Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X1733http://www.cidjournal.com/article/PIIS0738081X11000836/abstract?rss=yesAbstract: Mucous membrane pemphigoid, a heterogeneous group of autoimmune blistering diseases, affects primarily the mucous membranes. Although oral and ocular mucosae can both be affected in a given patient, patients with involvement restricted to oral mucosae tend to have a benign outcome, whereas those with ocular disease commonly face treatment resistance, resulting in scarring and blindness. Diagnosis requires direct immunofluorescence microscopy to demonstrate a linear deposition of immunoglobulin (Ig) G or IgA, or complement component 3 (C3), at the epithelial basement membrane. Although the target antigens vary, subsets of patients affected exclusively by oral and ocular mucosal diseases have autoantibodies targeting α-6 and β-4 integrins, respectively.Ocular and oral mucous membrane pemphigoid (cicatricial pemphigoid)Lawrence S. Chan10.1016/j.clindermatol.2011.03.007Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X3437http://www.cidjournal.com/article/PIIS0738081X11000848/abstract?rss=yesAbstract: Linear immunoglobulin A (IgA) bullous dermatosis, also known as linear IgA disease, is an autoimmune mucocutaneous disorder characterized by subepithelial bullae, with IgA autoantibodies directed against several different antigens in the basement membrane zone. Its immunopathologic characteristic resides in the presence of a continuous linear IgA deposit along the basement membrane zone, which is clearly visible on direct immunofluorescence. This disorder shows different clinical features and distribution when adult-onset of linear IgA disease is compared with childhood-onset. Diagnosis is achieved via clinical, histopathologic, and immunopathologic examinations. Two common therapies are dapsone and sulfapyridine, which reduce the inflammatory response and achieve disease remission in a variable period of time.Linear immunoglobulin A bullous dermatosisGiulio Fortuna, M. Peter Marinkovich10.1016/j.clindermatol.2011.03.008Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X3850http://www.cidjournal.com/article/PIIS0738081X1100085X/abstract?rss=yesAbstract: Pemphigoid gestationis is a rare autoimmune subepidermal bullous dermatosis that occurs during pregnancy and postpartum. Diagnosis is made on the basis of the presence of a subepidermal vesicle on routine histologic examination and of linear deposition of complement along the basement membrane zone of perilesional skin. The disorder is accompanied by severe pruritus and polymorphous bullous skin lesions. Clinical diagnosis is confirmed by histology and positive cutaneous immunofluorescence and immunoelectron microscopy tests (linear deposition of C3, with or without immunoglobulin G, along the basement membrane zone, within the lamina lucida, and localized to the proximal part of anchoring filaments of the epidermal fragment of salt-split skin). Enzyme-linked immunosorbent assay for pemphigoid gestationis antibody (BP180) is commercially available. If local treatment fails, systemic corticosteroid therapy should be administered. Oral corticosteroids are the therapeutic mainstay in pregnancy and postpartum. The prognosis is good for mother and child, except that there is a risk of preterm delivery and of moderate fetal growth restriction. Recurrence is possible during subsequent pregnancies. There is no significant maternal morbidity or mortality.Pemphigoid gestationisJasna Lipozenčić, Suzana Ljubojevic, Zrinka Bukvić-Mokos10.1016/j.clindermatol.2011.03.009Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X5155http://www.cidjournal.com/article/PIIS0738081X11000861/abstract?rss=yesAbstract: Dermatitis herpetiformis (DH) is a chronic, polymorphic, pruritic skin disease that develops mostly in patients with latent gluten-sensitive enteropathy. DH patients usually present with skin manifestations only and are not aware of the underlying small-bowel problems. Owing to the granular immunoglobulin (Ig) A deposition at the tips of the papillary dermis and to the subepidermal blister formation associated with neutrophilic accumulations underlying the basement membrane, DH is considered to be an autoimmune blistering disease. Contrary to the other bullous disorders, DH patients have no circulating autoantibodies binding to the cutaneous basement membrane components or to other adherent structures of the skin, but they have gluten-induced IgA autoantibodies against transglutaminase (TG) 2 and TG3. The serum IgA against tissue TG2 is a most specific and sensitive serologic marker of gluten-sensitive enteropathy and is equivalent to the perviously described IgA endomysium antibodies. DH could be a cutaneous IgA-epidermal TG3 immunocomplex disease, developing only in a few patients with gluten-sensitive enteropathy as a second gluten-dependent disease. The main treatment of DH today is a strict, life-long gluten-free diet. Untreated DH patients should be regularly monitored for malabsorption and lymphomas. Associated autoimmune diseases are more common among DH patients. Family screening for gluten sensitivity is also strongly suggested.Dermatitis herpetiformisSarolta Kárpáti10.1016/j.clindermatol.2011.03.010Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X5659http://www.cidjournal.com/article/PIIS0738081X11000873/abstract?rss=yesAbstract: Epidermolysis bullosa acquisita (EBA) is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type VII collagen (C7) structures, a major component of anchoring fibrils, which attach the epidermis to the dermis. EBA patients have tissue-bound and circulating antitype C7 autoantibodies that attack type C7 and result in a reduction or perturbation of normally functioning anchoring fibrils. Patients with EBA have skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic epidermolysis bullosa. These immunoglobulin G antitype C7 antibodies are pathogenic, because when they are injected into mice, the mice develop an EBA-like blistering disease. In addition to the classical mechanobullous presentation, EBA also has several other distinct clinical syndromes similar to bullous pemphigoid, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. Although treatment for EBA is often unsatisfactory, some therapeutic success has been achieved with colchicine, dapsone, plasmapheresis, photopheresis, infliximab, and intravenous immunoglobulin.Epidermolysis bullosa acquisitaRishu Gupta, David T. Woodley, Mei Chen10.1016/j.clindermatol.2011.03.011Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X6069http://www.cidjournal.com/article/PIIS0738081X11000885/abstract?rss=yesAbstract: Epidermolysis bullosa (EB) is a group of inherited, mechanobullous disorders caused by mutations in various structural proteins in the skin. There have been several advances in the classification of EB since it was first introduced in the late 19th century. We now recognize four major types of EB, depending on the location of the target proteins and level of the blisters: EB simplex (epidermolytic), junctional EB (lucidolytic), dystrophic EB (dermolytic), and Kindler syndrome (mixed levels of blistering). This contribution will summarize the most recent classification and discuss the molecular basis, target genes, and proteins involved. We have also included new subtypes, such as autosomal dominant junctional EB and autosomal recessive EB due to mutations in the dystonin (DST) gene, which encodes the epithelial isoform of bullouspemphigoid antigen 1. The main laboratory diagnostic techniques—immunofluorescence mapping, transmission electron microscopy, and mutation analysis—will also be discussed. Finally, the clinical characteristics of the different major EB types and subtypes will be reviewed.Inherited epidermolysis bullosa: New diagnostic criteria and classificationLizbeth R.A. Intong, Dédée F. Murrell10.1016/j.clindermatol.2011.03.012Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X7077http://www.cidjournal.com/article/PIIS0738081X11000897/abstract?rss=yesAbstract: Adjuvant immunosuppressive drugs are widely used to minimize corticosteroid-related adverse effects in the short-term and long-term management of cautoimmune bullous diseases. In bullous pemphigoid and pemphigus vulgaris, azathioprine and mycophenolate mofetil seem to be equally effective when used in combination with oral corticosteroids, but mycophenolate mofetil is less myelosuppressive and hepatotoxic. Due to a better safety profile, mycophenolate mofetil or enteric-coated mycophenolate sodium may gradually replace azathioprine as the first-line adjuvant of choice in the treatment of moderate to severe autoimmune bullous diseases, including epidermolysis bullosa acquisita and cicatricial pemphigoid. Cyclophosphamide still has a place in the treatment of severe relapsing autoimmune bullous diseases. Continuous oral cyclophosphamide provides optimal immunosuppression, but it also produces the highest cumulative dose. Several pulsed cyclophosphamide regimens have, therefore, been developed and are reported to be effective in severe forms of pemphigus. Randomized controlled studies are needed to compare the efficacy and safety of cyclophosphamide with newer treatment options, such as rituximab and immunoapheresis, and to define optimal dose ranges and duration of available immunosuppressive treatments in different stages of autoimmune bullous diseases.Immunosuppressive therapy for autoimmune bullous diseasesMichael Meurer10.1016/j.clindermatol.2011.03.013Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X7883http://www.cidjournal.com/article/PIIS0738081X11000903/abstract?rss=yesAbstract: Treatment of pemphigus patients is still challenging and, in some cases, conventional therapy with systemic corticosteroids in combination with adjuvant corticosteroid-sparing immunosuppressive drugs is not sufficient to induce clinical remission. More recently, high-dose intravenous immunoglobulins, immunoadsorption, and the monoclonal anti-CD20 antibody, rituximab, have been established as additional successful therapeutic options. This contribution covers both conventional therapies and most current treatment strategies for pemphigus.Current therapy of the pemphigus groupMichael Kasperkiewicz, Enno Schmidt, Detlef Zillikens10.1016/j.clindermatol.2011.03.014Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X8494http://www.cidjournal.com/article/PIIS0738081X11000915/abstract?rss=yesAbstract: The subepidermal immunobullous diseases are a group of autoimmune blistering disorders of the skin and mucous membranes that share the common features of autoantibody deposition and blister formation at the dermal-epidermal junction or basement membrane. This group includes bullous pemphigoid, linear IgA disease, dermatitis herpetiformis, and epidermolysis bullosa acquisita, among others. Although these disorders share some common features, each disease is unique in its clinical presentation, histopathology, and immunofluorescence patterns, which allows for accurate diagnosis and disease-specific treatment strategy. Treatment of these disorders is complex and requires expert knowledge of disease pathogenesis. We review common treatment approaches for each of these disorders.Treatment of subepidermal immunobullous diseasesDonna A. Culton, Luis A. Diaz10.1016/j.clindermatol.2011.03.015Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X95102http://www.cidjournal.com/article/PIIS0738081X11000927/abstract?rss=yesAbstract: Genetic and acquired bullous dermatoses can severely affect multiple domains of a patient's quality of life (QOL). Integrating formal evaluation of QOL into the clinical evaluation of patients facilitates an objective assessment of disease severity, mapping of disease trajectory, and captures therapeutic intervention outcomes. There have been 5 studies evaluating QOL in autoimmune dermatoses and 4 studies reviewing QOL in the genodermatoses. All literature to date indicates a significant disease burden in this setting. The development of formal QOL instruments has facilitated quantification of QOL deficits in this arena and offers promising tools for patient assessment in the future.Quality of life in patients with bullous dermatosesDeshan F. Sebaratnam, James R. McMillan, Victoria P. Werth, Dédée F. Murrell10.1016/j.clindermatol.2011.03.016Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X103107http://www.cidjournal.com/article/PIIS0738081X11000939/abstract?rss=yesAbstract: Scoring systems are used to assess the severity of a disease and the response to treatment. The main severity scoring indexes are the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and the Pemphigus Disease Area Index (PDAI). They have been validated and are already used in the evaluation of pemphigus and in clinical trials. They quantify disease severity by performing a global assessment of all lesions. In recent years, other severity scoring systems have been developed for pemphigus and other autoimmune blistering diseases.Severity score indexes for blistering diseasesBenjamin S. Daniel, Michael Hertl, Victoria P. Werth, Rüdiger Eming, Dédée F. Murrell10.1016/j.clindermatol.2011.03.017Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X108113http://www.cidjournal.com/article/PIIS0738081X11000940/abstract?rss=yesAbstract: Autoimmune blistering disorders are a heterogeneous group of diseases that result from autoantibodies generated against target antigens found in the skin and mucous membranes. This process leads to a variety of disruptions in keratinocyte adhesion and cellular integrity, resulting in fluid accumulation and development of blisters. Physicians should have an appreciation and understanding of autoimmune blistering disorders in the pediatric population when formulating a differential diagnosis of a patient who presents with skin blistering. Early detection and discrimination between the varied autoimmune blistering disorders can change the course of treatment and outcome. Due to the similarity in clinical presentation among different diseases within this category, histopathologic evaluation and, especially, immunofluorescence studies are necessary to establish the definitive diagnosis.Autoimmune bullous diseases in childhoodFreda Sansaricq, Sarah L. Stein, Vesna Petronic-Rosic10.1016/j.clindermatol.2011.03.018Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-X114127http://www.cidjournal.com/article/PIIS0738081X1100109X/abstract?rss=yesAbstract: Jan Mikulicz-Radecki (1850-1905) was the cofounder of modern surgery, aseptic techniques, and the inventor of gastroscopy, but his professional accomplishments go far beyond the field of surgical treatment. Various medical achievements are named in his honor, including the name of the cells that he discovered in rhinoscleroma, the name of Mikulicz disease, and the name of an ointment that he developed and continues to be used in the treatment of wounds and ulcers in surgery and dermatology. Noteworthy are also his interdisciplinary approach towards diagnosis and treatment, and his cordial attitude towards his patients.Jan Mikulicz-Radecki (1850-1905): His impact on modern medicineAndrzej Grzybowski, Jarosław Sak10.1016/j.clindermatol.2011.05.004Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-XCaretaker of the Skin129136http://www.cidjournal.com/article/PIIS0738081X11001088/abstract?rss=yesAbstract: This adventure in dermatology took place between 1960 and 1962. An immigration ruling forced Dr Bernard Gordon, a Canadian, to spend 2 years abroad before he could legally live and practice in the United States. Dr Gordon had just completed a 3-year residency at the New York University Skin and Cancer Unit. Dr Gordon met with his mentor, Dr Marion Sulzberger, for advice. This world-famous dermatologist took an interest in his situation and was able to arrive at an extraordinary solution. Dr Sulzberger consulted with his many dermatology colleagues and made arrangements for Dr Gordon to work abroad. He would spend 1 year in Caracas, Venezuela, teaching and conducting research in tropical skin diseases. The second year would be spent in Europe, beginning with 3 months in Madrid, Spain, followed by 3 months in Geneva, Switzerland, and concluding with 6 months at the Hôpital Saint Louis in Paris, France. Dr Gordon worked with the leading dermatologists at each hospital. These 2 years abroad led to a deeper understanding of world dermatology and lasting friendships.An adventure in dermatology: A personal historyBernard I. Gordon10.1016/j.clindermatol.2011.05.003Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-XClio Dermatologica137140http://www.cidjournal.com/article/PIIS0738081X10002579/abstract?rss=yesDermatologists have opportunities as communicators that are unique to our specialty. Consumers and patients have a never-ending hunger for information about their skin and how to keep it healthy and beautiful. Accordingly, we are sought after as speakers at medical meetings, for community gatherings, and by the media. In fact, no other medical specialty is so integrally tied to the mainstream media. According to the American Academy of Dermatology, dermatologists generated 1.4 billion media impressions in 2009.Effective communications using key messagingPatricia K. Farris10.1016/j.clindermatol.2010.12.001Clinics in Dermatology 30, 1 (2012)2012-01-01Clinics in Dermatology2012-01-01301S0738-081X(11)X0007-XDermatologic Disquisitions and Other Essays141143