Autoimmune ulceration of the leg

Autoimmune diseases are those disorders characterized by circulating autoantibodies or specific immune effector cells targeted towards a variety of self antigens. These disorders have traditionally included the so-called collagen vascular diseases, such as lupus erythematosus or scleroderma, certain vascultides, and a variety of immunologically mediated bullous diseases. The collagen vascular diseases and vasculitides, in particular, are occasionally associated with chronic, relapsing lower extremity ulcerations that may be refractory to traditional management schemes. Table 1 offers a classification of such disorders and provides a basis for the discussion which follows.

The frequency of these diseases among leg ulcer patients in general is difficult to gauge and depends in large part on the demographics of one's patient population as well as on efforts made to solicit such patients. For the last 18 months at the University of Pittsburgh, we have seen 303 new patients with leg ulcerations as part of a multidisciplinary wound healing and limb preservation clinic. Of this number, 20 patients (6.6%) have had leg ulcerations that were felt to be directly attributable to a variety of collagen vascular diseases or vasculitides. An understanding of these disorders and their potential to form ulcerations of different types is important in the differential diagnosis of patients with leg ulcerations in general, and management of these patients in particular.

The following is a discussion of the most important autoimmune disorders associated with leg ulcerations, with emphasis on practical aspects of diagnosis and management.     

Proctology – diseases of the anal region

Proctology is a medical subspecialty that encompasses diseases of the perianal region, anal canal, and rectum. Dermatologists play a pivotal role in this realm, as inflammatory perianal disorders, infectious and sexually transmitted diseases, as well as perianal tumors and their precursor lesions fall within the core competency of dermatology. In a concise manner, the present article highlights all relevant disease groups in the field of proctology. With a particular focus on aspects pertinent to dermatologists, this includes inflammatory disorders, “classic” proctologic diseases, sexually transmitted diseases, malignancies of the anal region, as well as pathogen‐induced diseases. Despite the wide variety of disorders, there are only five key symptoms prompting patients to consult a proctologist, including anal pruritus and burning, discharge, bleeding, pain, and foreign body sensation. A simple algorithm, which incorporates these symptoms as well as key clinical features, may assist in quickly establishing the correct diagnosis in everyday clinical practice.     

Changing a concept--controversy on the confusing spectrum of the reticulate pigmented disorders of the skin

Galli-Galli disease (GGD) and Dowling-Degos disease (DDD) are inherited skin diseases with variable progressive course. They are of benign and harmless behaviour but aesthetically annoying. They are subsumed within the group of reticulate pigmented disorders of the skin to which, additionally, Kitamura's and Haber's diseases are counted. Clinical appearance is approximately similar, with slight differences in age of onset and associated disorders. Histopathological features are almost similar aside from the unique hallmark of suprabasal acantholysis, which can exclusively be observed in GGD. We report four typical cases of reticulate pigmented disorders, clinically accordable to DDD but histopathologically allocated to GGD. In conclusion, we purpose the idea of a wide spectrum of reticulate pigmented disorders in which Morbus Galli-Galli should probably be reclassified as a subset of DDD.     

The prevalence of skin diseases in the elderly: analysis of 4099 geriatric patients

BACKGROUND: The geriatric population is composed of persons over 65 years of age, and few studies are available on the dermatologic diseases in this group. METHODS: Data on a total of 4099 geriatric patients admitted between the years 1999-2003 were analyzed. Hospital-based patient registry records were used for data collection. The data were analyzed according to age, sex, and time of admittance. RESULTS: The five most frequently encountered diseases in elderly patients were eczematous dermatitis, fungal infections, pruritus, and bacterial and viral infections. The most common disorders in males were fungal, bacterial, and viral infections, disorders of the feet, cutaneous ulcers, and vesiculo-bullous diseases, whereas, in females, they were immune-rheumatologic diseases and disorders of the mucous membranes. The five most frequently encountered diseases were significantly different in geriatric age subgroups. In the younger age group, pruritus, disorders due to sun exposure, and precancerous lesions and skin carcinomas were less common, whereas eczematous dermatitis was more common. The frequencies of some diseases showed significant seasonal variations. Infestations were more common in spring and summer, fungal infections were more common in summer but less so in winter, pruritus was more common in autumn but less so in spring, disorders due to sun exposure were more common in spring, and benign neoplasia were more common in autumn. In 2003, benign neoplasia, precancerous lesions and skin carcinomas, and immune-rheumatic disorders were more common, but vesicular and bullous diseases, fungal infections, and cutaneous lymphomas were less common when compared with the year 1999. CONCLUSIONS: This study provides important data on the frequency of dermatologic diseases in elderly patients, and shows variations in the frequency depending on age, gender, and season. We believe that this study will create awareness about the extent and patterns of dermatologic problems in geriatric patients.     

Autoimmune blistering diseases in the elderly

Autoimmune blistering diseases are a significant cause of morbidity and mortality in the elderly population. Given the advancing age of the population, the incidence of these disorders, particularly bullous pemphigoid, is expected to rise. This contribution reviews autoimmune immunobullous disorders of particular relevance in the elderly population. These include bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita, pemphigus, paraneoplastic pemphigus, and linear immunoglobulin A bullous dermatosis. Because therapy and management of individual immunobullous dermatoses differ, establishing the diagnosis is often critically important. An overall approach to bullous diseases in the elderly, as well as key clinical features, appropriate diagnostic tests, microscopic findings, immunofluorescence microscopy patterns, and molecular targets for select disorders are reviewed. Elucidation of antigenic targets at the molecular level has allowed for development of serum enzyme-linked immunofluorescence assays, which have enhanced diagnostic accuracy for several autoimmune blistering disorders. Given the relative rarity of these diseases, large randomized trials evaluating efficacy of various treatments are few, and therapy for most immunobullous disorders in the elderly has not been standardized. Despite this, appropriate therapeutic considerations for each condition are presented and the evidence for them is reviewed.     

Skin diseases of the lower extremities in the elderly

Routine care of the legs and feet in the elderly is important in helping to prevent infections, malignancies, and further deterioration. The lower extremities are of particular interest because of the increasing amount of diseases and disorders involving the legs and feet. Foot disorders in the elderly are associated with poor choices in footwear, structural changes, brought on by aging, and inadequate knowledge about prevention and treatment. Conservative treatments along with gait modification provide positive long-term results, although sometimes surgery is necessary for severe foot disorders. Onychomycosis and other nail disorders commonly infect the elderly despite the wide variety of treatment options. The increase in malignancy formation in the elderly is caused by the increase in the elderly population and inadequate treatment of leg ulcers and other chronic lesions. Aging is associated with many dermatologic changes; many of the disorders and diseases of the lower extremities can be managed if detected and treated early. Proper awareness of the signs, symptoms, and care is important.     

Epidemiology of skin disorders in Ethiopian children and adolescents: an analysis of records from the Italian Dermatological Centre, Mekelle, Tigray, Ethiopia, 2005 to 2009

Skin disorders are an important problem in children living in developing countries, but only a few epidemiologic investigations on pediatric dermatoses are available in the literature. Our study is an analysis of the range and frequency of skin diseases presenting to the Italian Dermatological Center in a pediatric Ethiopian population. A retrospective analysis was performed on 17,967 medical records of children aged 0 to 18 years attending the Italian Dermatological Centre in Mekele (Ethiopia) from January 2005 to December 2009. Infections and infestations accounted for 47% of the disorders seen; fungal infections were the most common (44.1%), followed by bacterial and parasitic diseases. Dermatitis constituted the second most common diagnostic category (24.7%) of the disorders seen, and contact dermatitis was the most common diagnosis (48.8%). Pigmentary disorders and disorders of skin appendages were more common in girls, whereas fungal and parasitic infections were more common in boys. Bacterial and parasitic infections were more common in children younger than 1 year old, fungal infections in those aged 1 to 5.9, and disorders of skin appendages and pigmentary disorders in those aged 15 to 18. These findings demonstrate that most of the disorders seen could be easily managed in clinical practice with appropriate skill development. It is crucial to ensure that training of medical students and pediatricians focuses on accurate recognition, diagnosis, and management of these common skin diseases and that families, teachers, health workers, and nurses be educated about the most common signs of prevalent skin diseases to help facilitate appropriate care.     

Skin diseases unique to the pregnant woman

Skin disorders are often associated with pregnancy. Most of these disorders are physiologic but some are pathologic. The following review focuses on skin diseases that are unique to pregnancy.     

The skin and the eye

Many systemic diseases may involve the skin and the eye. In some of these disorders, with both prominent ocular and cutaneous manifestations, the clinical findings, pathology, and pathophysiology may be quite similar; in other instances, quite diverse. Most dermatologists are well aware of the cutaneous manifestations of these disorders, but many of us may be unaware of the associated ophthalmologic findings. This article emphasizes and reviews the ocular manifestations of these diseases, hopefully enabling the dermatologist to make timely and appropriate referral to the ophthalmologic consultant.     

Immunosuppressive and cytotoxic drugs in the treatment of rheumatic skin disorders

Cytotoxic and immunosuppressive drugs are regularly used to treat proliferative, immunologically mediated inflammatory disorders and some neoplastic diseases of the skin. Methotrexate, azathioprine, mycophenolate mofetil, cyclosporin cyclophosphamide, chlorambucil, and other related drugs have potential benefits in the treatment of severe and/or recalcitrant rheumatic skin diseases. The therapeutic window for these agents is narrow. The major uses of these drugs are for life-threatening cutaneous disorders or as steroid-sparing agents.     

Psychotrichology: psychosomatic aspects of hair diseases

Hair growth and hair disorders with changes in hair density or quality not only influence an individualís appearance but also often lead to an enormous emotional burden with low self‐confidence, impaired quality of life, and even psychological disorders. Psychosomatic hair diseases cover a wide spectrum of specific psycho‐dermatological disease patterns. This review provides an overview and classification of psychosomatic hair diseases based on primary and secondary disorders. Somatoform disorders are among the primary psychiatric diseases, especially body dysmorphic disorder in which patients have an exaggerated and excessive preoccupation of normal and physiological hair loss. Self‐inflicted skin diseases as trichotillomania, often with an impairment of impulse control, also belong to this group. Secondary/reactive psychosocial disorders may occur in congenital and acquired hair disorders. These may be accompanied by feelings of disfigurement, depressive and anxiety disorders including social avoidance. Furthermore, psychosomatic comorbidity could complicate coping with hair loss. Psychosomatic therapy and coping are based on an early and accurate differential diagnostic approach to psychosomatic disorders. Psychotrichological disorders need to be treated promptly with psychosomatic basic care, improvement of coping strategies, behavior therapy, depth psychology, and/or appropriate psychopharmacotherapy with antidepressants or anxiolytics.     

Skin diseases in rural Nyala,Sudan (in a rural hospital,in 12 orphanages,and in two refugee camps)

Skin diseases are considered to be common in Nyala, Sudan. This study was carried out to verify the prevalence of skin diseases in Nyala. This prospective observational study included skin examination of a total of 1802 people: 620 patients who were evaluated in the outpatient clinics (OC) and 1182 people from orphanages and refugee camps (ORC) in Nyala, Sudan. χ² test was used. The total prevalence of skin disorders in the sample was 92.6% (1670/1802). One thousand and fifty of 1182 (88.8%) people from ORC had a skin disorder. The most common skin diseases in this community were: fungal infections (32.6%), dermatitis/eczema (10.5%), bacterial skin infections (10.3%), disorders of skin appendages (8.7%), parasitic infestations (7.7%), atrophic skin disorders (7.4%), disorders of pigmentation (7.4%), hypertrophic skin disorders (6.4%), viral infections (5.8), benign neoplasm (1.9%), dermatoses due to animal injury (0.4%), bullous dermatoses (0.1%), and malignant neoplasm (0.1%). Hypertrophic and atrophic disorders of the skin were mainly lesions of scarification (mostly atrophic) (5.7%) and keloids (5.6%). Fungal infection, bacterial infection, and parasitic infestation were more common in the ORC group, while dermatitis and eczema, disorders of skin appendages, hypertrophic and atrophic disorders of the skin, disorders of pigmentation, and benign neoplasm were more common in the OC group. The prevalence of skin diseases in the rural Nyala was more than our expectation and was dominated by infectious skin diseases. In addition, infectious skin diseases were more common in ORC rather than OC.     

ALOPECIA AREATA AND AUTOIMMUNITY: A CLINICAL STUDY

Alopecia areata (AA) frequently occur in association with other autoimmune diseases such as thyroid disorders, anemias and other skin disorders with autoimmune etiology. Despite numerous studies related to individual disease associations in alopecia areata, there is paucity of literature regarding comprehensive studies on concomitant cutaneous and systemic diseases. The present study has been designed to determine if there is a significant association between alopecia areata and other autoimmune diseases. This study covers 71 patients with the diagnosis of alopecia areata as the case group and 71 patients with no evidence of alopecia areata as the control group. Among the cutaneous diseases associated with AA, atopic dermatitis (AD) showed maximum frequency with an O/E ratio of 2.5, which indicates that it is two to three times more common in patients with alopecia areata. In our study, thyroid disorders showed the highest frequency with on O/E ratio of 3.2 and a P value of 0.01, which is statistically highly significant. Among the thyroid disorders, hypothyroidism was the most frequent association (14.1%) in our study. Since systemic involvement is not infrequent in patients with alopecia areata, it is imperative to screen these patients for associated disorders, particularly atopy, thyroid diseases, anemias and other autoimmune disorders, especially if alopecia areata is chronic, recurrent and extensive.     

代谢综合征与皮肤病

代谢综合征（MS）是一组以多种代谢性疾病合并出现为特点的临床症候群,是个体发展为2型糖尿病和心血管疾病的重要危险因素。其发病机制不明,可能涉及胰岛素抵抗、慢性炎症反应及氧化应激等。研究表明,MS与丘疹鳞屑性皮肤病、皮肤附属器疾病、皮炎湿疹类皮肤病、色素性疾病密切相关。本文综述MS与相关皮肤病的联系。     

Granulomatous vasculitides and the skin

Wegener's granulomatosis, lymphomatoid granulomatosis, and Churg-Strauss granulomatosis may all have cutaneous involvement. The morphology of cutaneous lesions in these disorders varies from macular erythema to frank gangrenous ulceration. Most often lesions are located on the extremities; however, truncal or facial involvement has been reported, the latter especially in Wegener's granulomatosis. A common histologic finding in these cutaneous lesions is necrotizing vasculitis. However, it is also possible to see Churg-Strauss extravascular granulomas and even periarteritis. Cutaneous involvement with these three forms of systemic granulomatosis generally parallels the systemic course. The treatment for the cutaneous lesions is dictated by the treatment for the systemic vasculitis. It is important to recognize that the cutaneous extravascular granuloma and cutaneous granulomatous vasculitis can be associated with other disorders in addition to systemic granulomatosis. These disorders include most importantly lymphoproliferative diseases, inflammatory disorders such as arthritis, autoimmune diseases, and other inflammatory disorders such as sarcoidosis. Cutaneous involvement with giant cell or temporal arteritis is not common, but ulcerative temporal-parietal scalp lesions are distinctive. Although not common in the United States, Takayasu's arteritis may have several cutaneous manifestations, including erythema nodosum-like lesions. Granulomatous vasculitides have a myriad of cutaneous manifestations. Knowledge of these manifestations may allow for prompt diagnosis in many cases and increased surveillance in other cases for associated systemic illnesses.     

Patterned disorders in dermatology

The skin gives us an opportunity to study pathologies unapparent in other systems such as patterned disorders. Among the best-identified patterns of skin disorders are the well-known lines of Blaschko, but other types of skin-patterned lesions have also been recognized. This short review will describe and discuss these different patterns and their pathophysiologic mechanisms, such as somatic mosaicism and X-chromosome associated mosaicism. Cutaneous patterned disorders are thought to be associated usually with inherited diseases per se, but in fact they are also reported in so-called acquired diseases. These cases suggest the existence of an underlying defect in a gene closely associated with the disease pathogenesis. The study of these acquired patterned disorders in the future may help us to understand the biologic foundations and pathogenesis of common human diseases.     

Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia

Linear immunoglobulin (Ig)A bullous dermatosis is a rare autoimmune subepidermal bullous dermatosis caused by circulating IgA autoantibodies directed against the antigens at the basement membrane zone. Most linear IgA bullous dermatosis cases are idiopathic, but some are associated with the use of certain drugs, infections, lymphoproliferative disorders, internal malignancies, autoimmune disorders, collagen diseases or, very rarely, other skin diseases, including autoimmune bullous diseases. Acquired hemophilia is also rare; it is a coagulation disease caused by anti-factor VIII IgG antibodies. Acquired hemophilia has been reported to be associated with malignant tumors, pregnancy or postpartum, drug reactions, collagen diseases such as rheumatoid arthritis, autoimmune disorders, and skin diseases such as psoriasis and pemphigus. We report a case of hemophilia acquired during the course of linear IgA bullous dermatosis and review reported cases of autoimmune bullous dermatoses associated with acquired hemophilia.     

Pemphigus vulgaris and pemphigus foliaceus: an overview of the clinical presentation,investigations and management

Pemphigus diseases are cutaneous and mucous membrane blistering diseases, related to the key antigens of desmoglein 1 and 3. This article reviews the topic, including diagnosis, and provides the physician with guidance on the treatment of these difficult to control disorders.     

Zivilisatosen

Psychosocial disorders and lifestyle trends have become more important in dermatology. Lifestyle diseases are a biopsychosocial phenomenon that can only be diagnosed and treated by paying attention to the quickly changing sociocultural aspects. The naming and popularization of the particular lifestyle diseases takes place by the media, but there is only an imprecise medical classification of these phenomena. This article gives an overview of the current situation and medical conditions of lifestyle diseases and try to assign them to an established psychosomatic diagnosis, based on the clinical symptomatic. Most often somatoform disorders, somatization disorders with a repeated presentation of physical symptoms which cannot be medically objectified or depressive disturbances are found.     

Pediatric skin disorders encountered in a dermatology outpatient clinic in Turkey

Abstract:  This study documents the spectrum and frequency of skin disorders in a total of 2370 children encountered in a dermatology outpatient clinic in Turkey. Infectious skin diseases were most frequently observed (27.6%) followed by, eczemas (17.9%), acne (14,5%), papulosquamous diseases (6.9%), hair diseases (4.1%), pigmentation disorders (3.8%), generalized pruritus (2.8%), urticaria (2.7%), and insect bite (2.3%). Epidemiologic data are useful in planning of the health care and taking preventive measures to decrease the prevalence of skin disorders in children.