Demographic and clinical properties of juvenile-onset Behçet's disease: A controlled multicenter study.

BACKGROUND: Behçet's disease (BD) is a multisystemic inflammatory disorder of unknown origin. The disease usually occurs between the second and the fourth decades, whereas it is uncommon in children. OBJECTIVE: In this multicenter study, we aimed to describe the demographic and clinical features along with severity in juvenile- versus adult-onset BD. METHODS: Patients with initial symptoms at age 16 years or younger were considered as having juvenile-onset BD. In all, 83 patients with juvenile-onset BD (38 male and 45 female; mean age 19.6 +/- 7.6 years) and 536 with adult-onset (>16 years) BD (293 male and 243 female; mean age 39.2 +/- 10.1 years) who fulfilled the classification criteria of the International Study Group for BD were involved in the study. RESULTS: Familial cases were more frequent in juvenile-onset compared with adult-onset BD (19% vs 10.3%; P = .017). The mean age of disease onset was 12.29 +/- 3.54 years in juvenile-onset BD and 31.66 +/- 8.71 years in adult-onset BD. Mucocutaneous lesions and articular symptoms were the most commonly observed manifestations in both groups. The frequency of disease manifestations was not different between juvenile- and adult-onset BD, except neurologic and gastrointestinal involvement, which were higher in juvenile-onset BD than adult-onset BD (P = .027 and P = .024, respectively). Oral ulcer was the most common onset manifestation of both juvenile-onset (86.74%) and adult-onset (89.55%) BD. The frequencies of onset manifestations of BD were similar, except genital ulcer, which was higher in adult-onset BD (P = .025). LIMITATIONS: Our study consisted of patients with BD mainly applying to dermatology and venerology departments. Therefore, it can be speculated that this study includes rather a milder spectrum of the disease. CONCLUSIONS: Although the clinical spectrum of juvenile-onset BD seems to be similar to adult-onset BD, the frequency of severe organ involvement was higher. Because of the higher prevalence of familial cases in juvenile-onset BD, it can be speculated that genetic factors may favor early expression of the disease with severe organ involvement.

Epidemiology of Pemphigus in Turkey: One-year Prospective Study of 220 Cases.

Pemphigus is a group of rare and life-threatening autoimmune blistering diseases of the skin and mucous membranes. Although they occur worldwide, their incidence shows wide geographical variation, and prospective data on the epidemiology of pemphigus are very limited. Objective of this work is to evaluate the incidence and epidemiological and clinical features of patients with pemphigus in Turkey. All patients newly diagnosed with pemphigus between June 2013 and June 2014 were prospectively enrolled in 33 dermatology departments in 20 different provinces from all seven regions of Turkey. Disease parameters including demography and clinical findings were recorded. A total of 220 patients were diagnosed with pemphigus during the 1-year period, with an annual incidence of 4.7 per million people in Turkey. Patients were predominantly women, with a male to female ratio of 1:1.41. The mean age at onset was 48.9 years. Pemphigus vulgaris (PV) was the commonest clinical subtype (n=192; 87.3%), followed by pemphigus foliaceus (n=21; 9.6%). The most common clinical subtype of PV was the mucocutaneous type (n=83; 43.2%). The mean Pemphigus Disease Area Index was 28.14±22.21 (mean ± Standard Deviation). The incidence rate of pemphigus in Turkey is similar to the countries of South-East Europe, higher than those reported for the Central and Northern European countries and lower than the countries around the Mediterranean Sea and Iran. Pemphigus is more frequent in middle-aged people and is more common in women. The most frequent subtype was PV, with a 9-fold higher incidence than pemphigus foliaceus.

Granulomatous disorders.

Almost all granulomatous skin disorders can cause red lesions on the face. Such disorders may include many bacterial, fungal, or parasitic infections, noninfectious inflammatory disorders, foreign body reactions, and even neoplasms. Clinically, they usually present with papules, plaques, nodules, and/or abscesses, which may ulcerate. It may be helpful in their differential diagnosis to define certain clinical patterns, such as multiple and discrete papules, necrotic or umbilicated papules or nodules, annular plaques, vegetative plaques or tumors, verrucous plaques or tumors, abscesses and/or sinuses, and lymphocutaneous pattern. Some disorders, such as sarcoidosis, can cause a wide variety of lesions. We accept that cutaneous leishmaniasis is also among such great imitators.

Cutaneous leishmaniasis: new dermoscopic findings.

BACKGROUND: Cutaneous leishmaniasis (CL) has a worldwide distribution. Delayed and incorrect diagnosis may cause the significant spread of this disease and consequent increases in morbidity and drug resistance. OBJECTIVES: We aimed to determine dermoscopic features of CL that may help to improve the accuracy of clinical diagnosis of the disease. METHODS: A total of 145 lesions in 102 patients were evaluated dermoscopically. Following the diagnosis of CL, all lesions were evaluated by experienced dermoscopists. RESULTS: A total of 51 papules, 40 nodulo-ulcerative lesions, 31 plaques, and 23 nodules were evaluated by dermoscopy. Generalized erythema appeared in all lesions (100%), yellow tears in 58 lesions, both crust and ulcer in 51 lesions, white starburst-like patterns in 27 lesions, ovoid salmon-colored structures in 19 lesions, and a perilesional hypopigmented halo pattern in four lesions. Various vascular structures were present in 126 lesions. The most common vascular structure observed was an irregular linear pattern in 78 lesions, followed by a tree-like pattern in 53 lesions. The rest of the vascular structures included hairpin vessels in 25 lesions, glomerulus-like vessels in 24 lesions, dotted vessels in 23 lesions, comma-shaped vessels in six lesions, and polymorphous/atypical vessels in four lesions. We did not identify the types of parasite involved. CONCLUSIONS: Yellow tears, white starburst-like patterns and salmon-colored ovoid structures seem to appear specifically in CL lesions. In geographical areas in which CL is common, dermoscopy may be utilized as a useful diagnostic tool that is practical and non-invasive.

Psoriasiform drug eruption associated with sodium valproate.

As psoriasis is a common skin disorder, knowledge of the factors that may induce, trigger, or exacerbate the disease is of primary importance in clinical practice. Drug intake is a major concern in this respect, as new drugs are constantly being added to the list of factors that may influence the course of the disease. We report a patient with a psoriasiform drug eruption associated with the use of sodium valproate. Physicians should be aware of this type of reaction. Early detection of these cases has practical importance since the identification and elimination of the causative drug are essential for therapy success.

Severe inflammatory and keloidal, allergic reaction due to para-phenylenediamine in temporary tattoos.

Hair coloring with henna has been popular in Turkey for years. In recent years since the tattoos are applied by the street vendors in most of the beach places in Turkey, skin coloring with henna has also increased. Henna can be used alone or in combination with other coloring agents. Henna alone can be safe but due to additives such as para-phenylenediamine (PPD), p-toluenediamine and various essential oils, allergic contact reactions may occur. We report a 22-year-old man who developed severe inflammatory and keloidal, moderately bullous allergic reaction after henna paint-on tattoo. We did a patch test separately with these painting products (henna powder, PPD) and with the European standard series. PPD was strongly positive (+++) on day 2 and remained positive for following days. After treating with topical clobetasol-17 butyrate, resolution was obtained in two weeks. But some keloidal reaction remained.

The influence of training on the recognition of gross features of dermoscopy images.

BACKGROUND: In a dermoscopic examination, besides structural components, inexperienced clinicians should also be able to recognize the gross features of the images. AIM: The aim of this study is, whether or not an inexperienced clinician has problems in the recognition of gross features of the images on dermoscopic examination. METHODS: Two dermatologists, of whom one was experienced in the field of dermoscopy and the other was not, examined 161 dermoscopic images of melanocytic lesions in the gross features of their borders. Inner and outer borders were defined for each lesion. Both dermatologists separately evaluated the borders of the lesions for irregularity, asymmetry, and wideness of fading. For subjective image analysis they scored each lesion by using the four-point ordinal scale. For computerized image analysis they manually marked borders with dots, by using a computer program. We used quadratic-weighted kappa for interobserver reliability assessments for subjective scores and intraclass correlation coefficients (ICC) for automatically calculated scores. RESULTS: In a subjective evaluation the inexperienced observer used a higher score than the experienced observer and the kappa values were between 0.241-0.286. ICC for the automatically calculated scores were between 0.357 and 0.522. According to both the outer and the inner borders, the concordance between experienced and inexperienced observers was almost perfect in measurements of diameter, perimeter, and area (ICC scores were between 0.948 and 0.990). CONCLUSIONS: An inexperienced person, in comparison with an experienced person, sees lesions in the same sizes, but in different shapes on dermoscopy. Therefore, it is advisable that making learners familiar with the borders of lesions should be included in the training on dermoscopy.

Interrater agreement in rating of pigmented skin lesions for border irregularity.

The clinical ABCD criteria are still recommended to both physicians and laymen when checking moles. The aim of this study was to determine the level of interrater reliability and therefore objectivity in rating for one of these criteria, namely border irregularity. Five professors, five residents, five nurses, and 10 students rated a set of 54 clinical images of pigmented skin lesions for border irregularity. After a descriptive presentation, rating was again carried out on another set of 54 images. In all groups, the agreement was moderate or substantial before the presentation and increased after the presentation. An almost perfect agreement was achieved by the professors after the presentation. Although both experience and receiving information could increase the level of interrater reliability, the disagreement was usually sufficient to suggest subjectivity in rating for border irregularity.

Pemphigus in the Mediterranean region of Turkey: a study of 148 cases.

BACKGROUND: The purpose of this study was to describe the epidemiological and clinical features, course, response to treatment, and prognosis of pemphigus in the Mediterranean region of Turkey. METHODS: All patients with confirmed pemphigus were prospectively enrolled in two major dermatology departments in the cities of Adana and Antalya in the Mediterranean region between March 1998 and March 2004. Details including demography, findings of clinical examinations, treatment, course, and prognosis were recorded. RESULTS: One hundred and forty-eight patients with pemphigus were diagnosed during the 6-year period, with a prevalence of 1.46 and an annual incidence of 0.24 per 100,000 in this region. There was a female predominance with a male to female ratio of 1:1.4. Pemphigus vulgaris (PV) was the most common clinical subtype, identified in 123 patients (83%). The mean age of onset was 43. In 101 (82%) patients with PV, disease began as persistent oral ulcers. The majority of the patients with PV could be managed with middle or high-dose steroids (60-140 mg/day). Complete clinical remission was obtained in 41 (39.4%) patients. The mortality rate was 4.8%. CONCLUSIONS: A moderately high incidence of pemphigus was found in the Mediterranean region of Turkey as compared with that encountered in other countries. The commonest clinical subtype was PV with a 9.5-fold higher incidence than pemphigus foliaceus. It is more frequent in middle-aged people and has a female predominance. Although a relatively higher dose of steroid was needed to control the PV, the disease completely remitted in a significant proportion of the patients.