Gabriella Brancaccio

Bullous pemphigoid: Etiology, pathogenesis, and inducing factors: Facts and controversies

The term pemphigoids includes a group of autoimmune bullous diseases characterized by subepidermal blistering. Bullous pemphigoid (BP) is not only the most common disorder within the pemphigoid group, but also represents the most frequent autoimmune blistering disease in general. The onset and course of BP depend on a variable interaction between predisposing and inducing factors. HLA genes are the most significant genetic predisposition factor to autoimmunity mechanisms. Many studies show an association between HLA-DQβ1*0301 and distinct clinical pemphigoid variants. Imbalance between autoreactive T helper (Th) and T regulatory cells, toll-like receptor activation, and Th17/IL-17 pathway are the three possible autoimmunity triggers underlying BP. The pathomechanism of BP hinges on an autoantibody response toward structural components of the hemidesmosome (BP180 and BP230). The binding of autoantibodies leads to complement activation, recruitment of inflammatory cells, and release of proteolytic enzymes. The inflammatory cascade also may be directly triggered by activation of Th17 cells with no intervention of autoantibodies. The intervention of inducing factors in BP can be identified in no more than 15% of patients. Facilitating factors in genetically predisposed individuals are various (drug intake, physical agents, and viral infections). Drugs may act as triggers by either modifying the immune response or altering the antigenic properties of the epidermal basement membrane. Cases of induction of BP by physical agents (eg, radiation therapy, ultraviolet radiation, thermal or electrical burns, surgical procedures, transplants) are rare, but well-documented events. A contributing role in inducing BP has been suggested for infections, in particular human herpes virus (HHV) infections (cytomegalovirus, Epstein-Barr virus, and HHV-6), but also hepatitis B and C viruses, Helicobacter pylori, and Toxoplasma gondii. Unlike pemphigus, no dietary triggers have been suspected of being involved in the induction of BP. In all patients who have a diagnosis of BP, an environmental agent as a potential cause should always be considered, because the prompt discontinuation of it might result in rapid improvement or even cure of the disease.

Bullous pemphigoid: associations and management guidelines: facts and controversies.

This article has been retracted: please see Elsevier Policy on Article Withdrawal (https://www.elsevier.com/about/our-business/policies/article-withdrawal). This article has been retracted at the request of the Editor following the discovery that the text overlaps significantly with sections of several articles that are cited in the reference section, including the following: Culton DA, Diaz LA. Treatment of subepidermal immunobullous diseases. Clin Dermatol 2012;30:95–102. Meurer M. Immunosuppressive therapy for autoimmune bullous diseases. Clin Dermatol 2012;30:78–83. Ljubojevic S, Lipozencic J. Autoimmune bullous diseases associations. Clin Dermatol 2012;30:17–33. Sehgal VN, Verma. Leflunomide: dermatologic perspective. J Dermatolog Treat 2013;24:89–95. Gürcan HM, Ahmed AR. Analysis of current data on the use of methotrexate in the treatment of pemphigus and pemphigoid. Br J Dermatol 2009;16:723–31. Chen YJ, Wu CY, Lin MW, et al. Comorbidity profiles among patients with bullous pemphigoid: a nationwide population-based study. Br J Dermatol 2011;165:593–9

Phlebolymphedema: Disregarded cause of immunocompromised district

Recurrent lymphangitis, deep trauma, malignancy, and related treatments (ie surgery and radiotherapy) are usually considered the commonest causes of lymphedema. The role of chronic venous disease in this context is often overlooked; yet, all patients with advanced venous disease (stasis dermatitis) have some degree of lymph circulation impairment.1 Venous edema is assumed to be the sole consequence of increased capillary filtration from venous hypertension. Because lymph drainage is the main buffer against the formation of edema, a compensation for the increased lymph load does occur at the beginning. In the long run, however, when the transport capacity is exceeded, the lymphatics fail to keep their buffer function, and interstitial fluid accumulates. This leads to a clinical condition of mixed venous and lymphatic insufficiency that causes a progressive swelling of soft tissues, which is termed phlebolymphedema.2 There is clinical and laboratory evidence confirming the presence of microangiopathy of the lymphatic network in patients with chronic venous disease.3 Further causes of phlebolymphedema are vein stripping (saphenectomy) for varicosities, vein-harvesting procedures for coronary bypass grafting or other vascular surgery, orthopedic surgery, or penetrating trauma involving the medial aspect of a lower limb (Brodell syndrome).4 In all of these conditions, lymph stasis occurs in the involved district as a consequence of the almost inevitable surgical cutting of the lymph collectors

Etanercept in the Treatment of Generalized Annular Pustular Psoriasis

1. Some authors consider a separate variant of generalized psoriasis, well described by Lapiere, as recurrent circinate erythematous psoriasis. It presents with erythematous, annular or polycyclic lesions, and an eruption of small sterile pustules and fine desquamation. The patches extend from the center and resolve within some weeks, leaving scales and changes in pigmentation and pigmentary changes. Frequent relapses are described in the bordering areas 2,3 . In this study, we report the case of a woman affected by generalized annular pustular (Lapiere) psoriasis. This patient had previously been treated with conventional therapeutics, and demonstrated a significant improvement after treatment with etanercept. This 70-year-old Caucasian woman, described in our report, has a 35-year history of psoriasis. Physical examination revealed the presence of small erythematous papules, centered by a pustule, a few millimeters in diameter (Fig. 1). Histological examination showed Kogoj-Lapiere spongiform multilocular typical pustules (an epidermal pustule formed by infiltration of neutrophils into necrotic areas of the epidermis, where the cell walls form a swampy network), features compatible with the clinical diagnosis of Lapiere psoriasis. The patient had previously been treated with other topical and systemic drugs (colchicine, acitretin, ciclosporin, methotrexate) and ultraviolet B narrow band phototherapy, with partial and temporary benefits, side effects, and frequent relapses. Differential diagnosis of our case included other generalized pustular psoriasis: acute generalized exanthematous pustolosis (AGEP) was perhaps the most important differential diagnosis. AGEP, which occurs as an acute, spontaneously healling reaction to drugs (usually antibiotics), was excluded on the basis of the absence of vasculitis associated with spongiform pustules and based on the presence of psoriatic anamnesis. Lapiere psoriasis can be differentiated from pustular lesions caused by prolonged application of topical steroids or tar ointments on the periphery of pre-existent psoriatic plaques. Unlike the Von Zumbusch generalized form, the general state of health is not compromised. The patient was treated with 50 mg of etanercept twice weekly subcutaneously for three months. There was an

RETRACTED: Bullous pemphigoid: Associations and management guidelines: Facts and controversies

This article has been retracted: please see Elsevier Policy on Article Withdrawal (https://www.elsevier.com/about/our-business/policies/article-withdrawal). This article has been retracted at the request of the Editor following the discovery that the text overlaps significantly with sections of several articles that are cited in the reference section, including the following: Culton DA, Diaz LA. Treatment of subepidermal immunobullous diseases. Clin Dermatol 2012;30:95–102. Meurer M. Immunosuppressive therapy for autoimmune bullous diseases. Clin Dermatol 2012;30:78–83. Ljubojevic S, Lipozencic J. Autoimmune bullous diseases associations. Clin Dermatol 2012;30:17–33. Sehgal VN, Verma. Leflunomide: dermatologic perspective. J Dermatolog Treat 2013;24:89–95. Gürcan HM, Ahmed AR. Analysis of current data on the use of methotrexate in the treatment of pemphigus and pemphigoid. Br J Dermatol 2009;16:723–31. Chen YJ, Wu CY, Lin MW, et al. Comorbidity profiles among patients with bullous pemphigoid: a nationwide population-based study. Br J Dermatol 2011;165:593–9

Melanoma: clinical and dermoscopic diagnosis.

Missing the diagnosis of a melanoma is the worst dermatologist nightmare, especially when melanomas have a non-alarming clinical appearance and imitate a completely benign lesion. The use of dermoscopy has provided an effective tool to facilitate the differential diagnosis and to increasingly allow an early diagnosis of melanoma. The aim of this article was to summarize the most recent and important clinical and dermoscopic pearls to recognize melanoma at the earliest stages of its development.

Evolution of pigmented Spitz naevi with starburst pattern during childhood

Spitz nevi (SN) are benign melanocytic lesions typical of childhood/adolescence that can mimic melanoma on a clinical, dermoscopic, and histopathologic level. Moreover, SN are often characterized by rapid growth and worrisome changes. Thus, their diagnosis and management may be challenging. The knowledge on prognosis and natural history of SN improved substantially in the last few years thanks to dermoscopy and a better understanding of clinic-pathologic correlations. This article is protected by copyright. All rights reserved.

Obligate and facultative paraneoplastic dermatoses: an overview

Dermatological paraneoplastic syndromes are a group of cutaneous diseases associated with malignancy, but not directly related to the primary tumor itself or to its metastases. It is of utmost importance for the dermatologist to recognize the major cutaneous paraneoplastic syndromes to diagnose the underlying tumors that trigger them as early as possible. In this overview, skin conditions that are highly correlated with malignancy, whose recognition implies a mandatory investigation of internal cancer, are described.

Dermatoscopy of Vascular Lesions

Cutaneous vascular lesions (VLs) represent a very common reason for dermatologic consultation for patients. In most cases, VLs are benign and self-limiting. However, because they often mimic malignant skin tumors, their correct and prompt identification is very important in daily practice. Dermoscopy may play a key role in achieving that purpose. This article reviews current knowledge of dermoscopic features of the most frequent VLs.

A new direction for Dermatology Practical & Conceptual

Establishing the journal, Dermatology Practical & Conceptual (DPC), has been a tremendous challenge for us. Previously named Dermatopathology Practical & Conceptual by Bernie Ackerman, who was the visionary founder, DPC became more and more relevant under the editorship of Drs. Kittler, Zalaudek, Marghoob and Marchetti. When the journal became the official organ of the International Dermoscopy Society, DPC became a great arena to share the visions and experiences in the field of dermoscopy. Research in dermoscopy is of uppermost importance, given that this technique is still relatively young, and its applications in the context of skin tumors, but especially of general dermatology, are growing day by day. However, DPC is not only that. It is indeed unique in that it is open access, free to both authors and readers. In other words, at DPC there are no fees for anyone who wants to read or publish scientific articles in the entire field of clinical and experimental dermatology! This is the same spirit that has guided the International Dermoscopy Society for the last 15 years, namely, an open access platform devoted to physicians, aiming exclusively to promote the science behind dermatology, to spread the available knowledge, and to assist doctors in improving their efficiency in diagnosing and treating patients with skin disorders. With this in mind, we feel privileged and deeply committed to take Dermatology Practical & Conceptual in a brand new course aimed to further spread the readership, facilitate publication of important new ideas, and finally take the journal to the next step!