Vitamin deficiencies/hypervitaminosis and the skin.

Vitamins are an indispensable food source and important owing to the enzyme cofactor and catalytic roles they play in the body. Fat-soluble vitamins A, D, E, K, and B12, are stored in the body and can cause problems with their excessive accumulation. Other vitamins rarely accumulate in the body because they dissolve in water and are excreted through the kidneys. Alcoholism, strict diets, insufficient parental nutrition, and gastrointestinal absorption problems may be included in the causes of vitamin deficiencies. Although clinical findings of vitamin deficiencies display different characteristics depending on the vitamins, the signs that generally occur are cutaneous pigmentation, pigmentation on mucous membranes, palmoplantar keratoderma characterized by fissures, palmar streaking, yellow streaking on the nails, nail layering, and intranail hemorrhage.

Demographic and Clinical Features of 1,641 Patients with Alopecia Areata, Alopecia Totalis, and Alopecia Universalis: A Single-Center Retrospective Study.

BACKGROUND/AIM: Alopecia areata (AA) is a common autoimmune hair disorder which is characterized by noncicatricial hair loss. AA commonly presents with localized patches on the scalp and face but may affect any hair-bearing region of the body leading to even more generalized involvement. AA may affect any age group, gender, and race. The current study investigates the demographic characteristics of the patients with AA and subgroups of AA including alopecia totalis (AT) and alopecia universalis (AU) and the prevalence of disease, sex, and age distribution and seasonal variation retrospectively in a tertiary dermatology clinic in Turkey. MATERIALS AND METHODS: In this retrospective, cross-sectional study, 1,641 patients diagnosed with AA, AT, and AU in the dermatology clinic of a public university hospital were included. The dermatology outpatient database was reviewed retrospectively. The diagnosis of AA was based on patient history, clinical examinations, and histopathologic findings. RESULTS: Fifty-four thousand one hundred sixty-eight patients were admitted to our outpatient clinic in 4 years time, and 1,641 were diagnosed as having AA, AT, and AU. One thousand three hundred ninety-two patients (84.8%) had AA, 81 (4.9%) had AT, and 168 (10.2%) had AU. Among the 1,641 patients included in the study, 877 were females (53.4%) and 764 were males (46.6%). The mean age was 29.86 ± 14.48 years in AA, 29.50 ± 16.18 in AT, and 32.81 ± 14.48 in AU; 77.4, 72.8, and 68.5% of patients were aged under 40 years in AA, AT, and AU. There was no statistically significant difference in seasonal presentation times. CONCLUSION: AA is affecting approximately 2% of the general population without any sex, race, or age group predilection. In this study, we found a lower prevalence of AA in the pediatric age group in comparison with adults. This finding may support the hypothesis of the increasing prevalence of AA over time. The higher ratio of AA regarding this study may support that the frequency of AA and subtypes varies between regions.

A multistep approach to the diagnosis of rare genodermatoses.

Genodermatoses are heritable skin diseases that can cause significant morbidity and mortality. Most of them show characteristic cutaneous findings. Genodermatoses can be associated with extracutaneous system abnormalities. Diagnosing hereditary skin disorders is still a challenging task due to their rarity and diversity, due to diseases evolving over many years, and the initial manifestations not always being diagnostic; therefore, ongoing evaluation and surveillance is often required to make the accurate diagnosis. The algorithm for the diagnosis depends on a combination of thorough clinical and family history clinical examination, laboratory findings, consultation of multiple medical specialists, and molecular analysis. Diagnostic testing targeted at differentiation of similar genodermatoses may be required. Recognition is crucial for the initiation of the treatment for skin manifestations and detection of other extracutaneous abnormalities, including malignancy. Diagnostic accuracy and molecular diagnosis may help in providing a template for ongoing management, testing, and education and prognostication for families of children with genodermatoses.

Eruptions in life-threatening rheumatologic diseases.

Dermatologic changes occur in a variety of rheumatic diseases. Skin can be the initial site of involvement, thus providing important clues for an accurate diagnosis based on cutaneous findings. Dermatologic findings can also be an indicator of systemic involvement and prognostic outcome; however, many connective tissue disorders have a wide variety of cutaneous manifestations, with significant overlap between different diseases. These skin signs often precede systemic clinical manifestations. Careful attention to characteristic dermatologic findings in Behçet's disease, systemic lupus erythematosus, rheumatoid arthritis, and various vasculitis can provide prompt therapeutic approaches in the case of life-threatening complications of systemically involved rheumatologic diseases.

Changes in serum TNF-like weak inducer of apoptosis (TWEAK) levels and Psoriasis Area Severity Index (PASI) scores in plaque psoriasis patients treated with conventional versus anti-TNF treatments.

BACKGROUND: Psoriasis is a chronic dermatologic disease affecting 2% of the general population. Tumour necrosis factor (TNF)-like weak inducer of apoptosis (TWEAK) is a newly defined member of the TNF family. Increased serum levels of TWEAK were reported in inflammatory diseases. The relationship between serum TWEAK levels and severity of psoriasis has not yet been proven. Our aim was to clarify the change in serum TWEAK levels in response to conventional and anti-TNF treatments. MATERIAL AND METHODS: Blood samples were collected from 103 moderate or severe chronic plaque psoriasis patients with or without arthritis who were referred to the Department of Dermatology, Istanbul University Cerrahpasa Medical Faculty between the years 2016 and 2018. Psoriasis Area and Severity Index (PASI) scores were calculated, and serum TWEAK levels were assessed with TWEAK ELISA kit. SPSS 20 was used for statistics. RESULTS: Serum TWEAK levels increased significantly and PASI scores decreased significantly after both conventional and anti-TNF treatments, but the two variables were not correlated. There was no significant difference between conventional and anti-TNF treatments, between patients with or without comorbid arthritis and between genders. CONCLUSIONS: Lower serum TWEAK levels induce psoriasis and higher levels of TWEAK are observed after treatment. It is important to determine a threshold value. Such a cutoff value of serum TWEAK levels could not be calculated in our study similar to previous studies. If its serum levels were to be standardized in further studies, TWEAK can be used as a follow-up marker in psoriasis patients with the PASI score.

Pathergy testing: prospective comparison of dermatoscopic evaluation and naked eye examination.

Pathergy phenomenon is a non-specific tissue hyperreactivity reaction due to trauma and is a minor diagnostic criterion of Behcet's disease. In this study, 100 patients with a suspicion of Behcet's disease who were referred to Cerrahpasa Medical Faculty Dermatology department between 01.11.2014 and 31.01.2015 are included. Skin pathergy tests were applied to all the patients and results were evaluated by two dermatologists separately at 48th hour, each with naked eye and with dermatoscopy. Test results were scored on a scale of 0-6. At the end of the study, score results of naked eye and dermatoscopy for doctor number 1 were statistically similar. Same results applied for doctor number 2. However, naked eye results of doctor number 1 and 2 for the same patients were significantly different from each other (p 0.0372) and with dermatoscopy examination this difference was eliminated (p > 0.05). This study revealed that naked eye evaluation of pathergy test results can yield different results among different interpreters. Use of dermatoscopy during the evaluation process decreases interobserver variation and subjectivity of the test.

The color of skin: brown diseases of the skin, nails, and mucosa.

Brown diseases comprise disorders leading to hyperpigmentation in skin and nails. Melasma is an acquired skin disorder that is characterized by brownish macules that typically occur on the face. Schamberg disease, also known as progressive pigmented purpura, is characterized by brown pigmentation with pepper spots on their edges. We summarize the epidemiology, pathogenesis, histologic features, and treatment choices for additional brown diseases, including melasma, pigmented purpuric dermatoses, postinflammatory hyperpigmentation, drug-induced hyperpigmentation, and pigmentations due to systemic or physiologic conditions.

The Pathergy Test as a Diagnostic Tool.

The pathergy test produces a nonspecific hyperreactive lesion in Behçet's disease (BD), a finding that has been known since 1937. Pathergy refers to the development of new skin lesions or the aggravation of existing ones after trivial trauma. In clinical practice, the pathergy test induces a skin response by needleprick, with positive reactions manifesting as a papule or pustule developing by 48 hours. The pathergy test is one of the major features and diagnostic criteria of the disease. It is very similar to the erythematous papules or pustules that appear spontaneously in patients with BD. There is no standardized method for conducting the pathergy test. Intradermal, intravenous, and subcutaneous applications are used. There is no generally accepted opinion on which form of the test yields a higher positivity rate. The pathergy reaction is also reported in pyoderma gangrenosum, and has been noted in other neutrophilic dermatoses such as Sweet syndrome. The overall objective of this contribution is to provide a review of the available information, literature, and research relating to the pathergy test.

Palmoplantar psoriasis.

Palmoplantar psoriasis refers to a localized psoriasis variant. The disease can be associated with many clinical forms, including predominantly pustular lesions to thick scaly, hyperkeratotic plaques, or an overlapping of both of them. Palmoplantar psoriasis accounts for 3-4% of all psoriasis cases in most studies. Although it is localized only on the palms and the soles, the fissures, the hardening of the tissue, and hyperkeratosis affect daily routine activities. Taking the body surface area as a measure of severity can sometimes be misleading. In clinical practice, the level of functional impairment should be taken into account rather than relying on traditional instruments to evaluate the severity. Palmoplantar psoriasis is usually managed with topical therapy as a first step. Systemic therapy is needed when the topicals fail or when the disease becomes more severe. Sometimes, biologic agents are required for adequate maintenance of clinical response.

Occupational acral dermatitis and where the twain shall meet.

This contribution, which is somewhat of a departure from the usual format, will focus on dermatologic conditions that are consequences of the patients' choices of employment, hobby, or even other forms of recreation. Most of the cutaneous changes involve the hands and feet, and each is labeled according to the anatomic location.

Acral manifestations of Sweet syndrome (neutrophilic dermatosis of the hands).

Neutrophilic dermatosis of the hand (NDH) is a rare localized variant of the syndrome, originally described two decades ago by Strutton et al. The lesions of NDH and Sweet syndrome are similar, as indicated in the first report of NDH. Both diagnoses are characterized by an acute onset of fever, leukocytosis, and tender, erythematous infiltrated plaques. There are also bullae and ulceration in NDH, in contrast to Sweet syndrome, in which bullae are quite uncommon, especially at the early stages. Similar to Sweet syndrome, the majority of NDH patients are women (69%). Patients with NDH present with fever, peripheral neutrophilia, leukocytosis, and/or an elevated erythrocyte sedimentation rate or C-reactive protein level, but at a significantly lower rate than those in Sweet syndrome (33%). Similar to Sweet syndrome, NDH has been associated with the following conditions: Malignancies (particularly hematological [21%], most common of which is acute myelogenous leukemia, but many other malignancies as well), inflammatory bowel disease (19%), medication and vaccination-related eruptions, bacterial and viral infections, rheumatologic diseases, and others. The clues to the diagnosis of NDH are the same as for Sweet syndrome. Awareness of this diagnosis is important not only to avoid unnecessary medical and surgical therapy and to expediently initiate the administration of steroids for this highly responsive dermatosis, but also to conduct an appropriate workup to exclude associated diseases, especially malignancies.

Inflammatory Lipid Mediators in Common Skin Diseases.

Lipid mediators play a main role in the complex course of cutaneous inflammatory reactions. They regulate a wide spectrum of cellular processes such as cell proliferation and apoptosis. In the early phase of inflammation, excessive amounts of lipid mediators are released and play a major role in the pathogenesis of skin diseases. Recent data suggest that lipid mediators are able to interfere with the pathogenesis of certain dermatologic diseases, seriously affecting patient quality of life. Acne, psoriasis, and atopic dermatitis are specific examples of skin diseases that may respond to treatment with medication affecting these metabolic pathways. The authors briefly present the current knowledge about the role of lipid mediators in common skin pathologies.

Topical immunotherapy with diphenylcyclopropenone-induced vitiligo.

Topical immunotherapy made by diphenylcyclopropenone (DPCP) is an alternative treatment that can be used safely and efficaciously in recalcitrant alopecia areata patients. DPCP-induced vitiligo is a rare, but documented, unwanted side effect. The real mechanism of DPCP-induced vitiligo is not well known.

Histopathological and clinical evaluation of papulopustular lesions in Behçet's disease.

OBJECTIVES: Behçet's disease (BD) is a chronic inflammatory disorder characterised by aphthous stomatitis, genital ulcerations, erythema nodosum-like manifestations and papulopustular lesions. While a neutrophilic vasculitis accompanies most skin lesions it is usually regarded that the papulopustular lesions in BD are similar to acne vulgaris (AV). The aim of our current study was to further assess the clinical and histopathological features of papulopustular lesions in BD and how these features compared to those of AV. METHODS: To analyse the histopathological features of BD and AV, 89 punch biopsies were taken from 58 BD (52 male, 6 female) and 31 AV patients (26 male, 5 female). Sections were evaluated in a blind manner by two different pathologists. A dermatologist who was blind to the patients' diagnosis counted the number of papules, pustules, comedones, folliculitis, cysts and nodules on the face, chest, back, upper and lower extremities. RESULTS: The number of papules, pustules and comedones was higher on the face in the AV group, whereas in the BD group, both number of papules and folliculitis on the back and that of folliculitis were higher on the lower extremities in the AV group. With the exception of comedone formation, which was more frequent among the AV patients [20/31 (64.5%) vs. 23/58 (39.6%), p=0.025] the presence of suppurative folliculitis/perifolliculitis, intrafollicular abscess formation, leukocytoclastic vasculitis or microorganisms were not useful in differentiating BD from AV. However, the interobserver agreement for histologic assessment was low. Kappa was 0.17 for suppurative foliculitis/perifol¬liculitis; 0.39 for intrafollicular abscess formation; 0.51 for leukocytoclastic vasculitis. CONCLUSIONS: In the BD group, although the inflammatory lesions located on the face were less than those in the AV group inflammatory lesions such as folliculitis on the legs were only seen, again in the BD group. The papulopustular lesions of BD could not be distinguished from AV by histology. Some of this might be due to high interobsever variation in interpretation. Acne is an inherent manifestation of BD.

Pseudo-Kaposi sarcoma (acroangiodermatitis): occurring after bullous erysipelas.

Pseudo-Kaposi sarcoma is a benign reactive vascular proliferative disorder, which can be seen at any age. It occurs when the chronic venous pressure changes result in vascular proliferation in the upper and mid dermis. This disease is divided into two subtypes: the most frequent subtype is the Mali type and seen in early ages. The Mali type is seen in chronic venous insufficiency and in those patients with arteriovenous shunts. The rare subtype is the Stewart-Bluefarb type. This disease must be distinguished from Kaposi sarcoma because of their clinical resemblance. Herein, we present a patient with pseudo-Kaposi sarcoma, which developed after bullous erysipelas.

Bacterial infections of the folds (intertriginous areas).

The axillary, inguinal, post-auricular, and inframammary areas are considered skin folds, where one skin layer touches another. Skin fold areas have a high moisture level and elevated temperature, both of which increase the possibility of microorganism overgrowth. A massive amount of bacteria live on the surface of the skin. Some are purely commensal; thus, only their overgrowth can cause infections, most of which are minor. In some cases, colonization of pathogenic bacteria causes more serious infections. This contribution reviews the bacterial infections of the skin fold areas.

Darier disease: A fold (intertriginous) dermatosis.

Darier disease, also known as Darier-White disease, is characterized by yellow to brown, oily keratotic papules and plaques in the seborrheic areas of the face and chest. This disorder may show different clinical manifestations, such as palmoplantar pits and nail abnormalities. The trigger factors are mechanical trauma, heat, humidity, ultraviolet B, and pyogenic infections. The disease usually becomes apparent in the second decade of life. The ATP2 A2 (SERCA2) gene mutation was detected in all patients. Histopathologic changes include epidermal adhesion loss, acantholysis, abnormal keratinization, eosinophilic dyskeratotic cells in the spinous layer known as corps ronds, and the presence of grains in the stratum corneum. Although the treatment for Darier disease is unsatisfactory, some relief has been achieved with the use of corticosteroids and retinoids.

Hailey-Hailey disease: A fold (intertriginous) dermatosis.

Hailey-Hailey disease, also called benign familial pemphigus, is a late-onset blistering disorder that affects the flexures. There are typically painful erosions and cracks in affected areas. Lesions generally begin between 20 and 40 years of age. In two third of all cases, positive family history is detected. In pathogenesis, there is a defect in keratinocyte adhesion due to ATP2 C1 gene mutation. The result of the desmosomal decomposition is acantholysis. Menstruation, pregnancy, skin infections, physical trauma, excessive sweating and exposure to ultraviolet radiation are important triggering factors. Histopathologic changes are suprabasal acantholysis and formation of intraepidermal bullae. In the epidermis, a partial acantholysis that looks like broken bricks is observed.