A multicentre prospective analysis of the incidence of pemphigoid diseases in Turkey.

BACKGROUND: The differentiation between the pemphigoid diseases is essential for treatment and prognosis. In Turkey, data on the incidence of these diseases are insufficient. Our aim in this study is to determine the incidence, demographics and clinical characteristics associated with diseases of the pemphigoid group. METHODS: We prospectively analysed 295 patients with pemphigoid who visited dermatology clinics of tertiary referral hospitals in 12 different regions of Turkey within a year. The diagnosis was based on clinical, histopathological, direct immunofluorescence (DIF) and serological (multivariant enzyme-linked immunosorbent assay [ELISA], indirect immunofluorescence and mosaic-based BIOCHIP) examinations. Clinical and demographic findings, aetiological factors and concomitant diseases observed in the patients were recorded. RESULTS: A total of 295 (female/male ratio: 1.7/1) patients with pemphigoid were diagnosed in 1-year period. The overall incidence rate of pemphigoid diseases was found to be 3.55 cases per million-years. The ratio of pemphigoid group diseases to pemphigus group diseases was 1.6. The most common pemphigoid type was bullous pemphigoid (BP, 93.2%). The others were epidermolysis bullosa acquisita (3.1%), pemphigoid gestationis (2.4%), linear IgA disease (1%) and mucous membrane pemphigoid (0.3%). The most common (26.8%) possible trigger of the bullous pemphigoid was gliptin derivative drugs. The most common concomitant diseases with pemphigoid were cardiovascular (27.8%) and neurological diseases (23.7%). CONCLUSIONS: This study showed that the increased frequency of bullous pemphigoid reversed the pemphigoid/pemphigus ratio in Turkey. Further studies are warranted regarding the reasons for this increase.

Evaluation of the laboratory parameters in hidradenitis suppurativa: Can we use new inflammatory biomarkers?

Hidradenitis suppurativa (HS) is a chronic, recurrent, and debilitating skin disease. Recent studies showed that inflammatory biomarkers, such as neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), Lymphocyte/HDL ratio (LHR), Neutrophil/HDL ratio (NHR), and Monocyte/HDL ratio (MHR) are an indicator of inflammatory diseases and may be associated with disease severity and disease activity. To investigate NLR, PLR, LHR, NHR, and MHR in HS patients. In addition, to compare erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), complete blood count, leukocyte profile, and biochemical parameters between the control and the patient group. Clinical and biochemical data of patients and healthy subjects were collected from medical records, retrospectively. In total, 166 patients with HS and 124 control subjects were included. We found no significant difference in NLR (P = .207) and PLR (P = .257). LHR (P < .001), NHR (P < .001), and MHR (P < .001) were significantly higher in the patient group. No positive correlation was found between any of these markers and disease severity according to Hurley staging system. However, MCV (Mean corpuscular volume), RDW (Red cell distribution width), and CRP showed a significant positive correlation with disease severity. Among these markers, only MHR was positively correlated with disease duration. Our study shows that CRP still maintains its value for HS patients compared to new inflammation markers. Unlike the studies in other inflammatory diseases, no significant relationship was found with most of these inflammatory parameters. MHR may be more useful in patients with HS as an indicator of inflammation compared to other parameters.

Patients' knowledge about psoriasis and comorbidities; their participation in treatment decisions.

BACKGROUND: Psoriasis is a common chronic inflammatory disease. Having more information about psoriasis and comorbid diseases means that patients have more involvement in treatment. AIM: Evaluate the knowledge levels of psoriasis patients about the disease and comorbidities and also to determine participation in treatment decisions. METHODS: Totally 103 patients over the age of 18 with diagnosed psoriasis were included into the study. A questionnaire including items on knowledge about disease and treatment options, comorbid diseases were filled out by the patients. Total knowledge score (TKS) was found by giving 1 point to the questions that the patients knew correctly. RESULTS: Patients were well informed about their risk of depression (64.1%), but were less well informed about their risk of atherothrombotic disease (14.6%). About 50.4% of the patients thought that psoriasis may be due to an internal organ disease. There was a negative correlation between the patient's age, the age of diagnosis and TKS. About 42.7% wanted to leave the decision entirely to the physician. CONCLUSION: The results of this study showed that majority of patients with psoriasis are not aware of the increased risk of comorbid diseases and do not have enough knowledge about psoriasis.

Successful treatment of severe refractory post-cesarean pyoderma gangrenosum with intravenous immunoglobulin.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by rapidly progressing necrolytic ulceration of the skin. Proper treatment is crucial since it can result in devastating consequences. First-line treatments include systemic corticosteroids or cyclosporine. However, no standardized treatment regimens for refractory cases exist and treatment outcomes are affected by underlying conditions. PG after cesarean section, which is believed to occur in association with underlying pregnancy- and parturition-related immune changes, is extremely rare, and all reported cases in the literature have been successfully treated with systemic or topical corticosteroids. We report a case of a 32-year-old patient with severe PG occurring on her cesarean scar 3 days after the cesarean delivery. Treatment with systemic corticosteroids and first-line immunomodulatory agents resulted in insufficient response and serious complications. Intravenous immunoglobulin (IVIG) was then initiated, and a rapid clinical response was seen. Corticosteroid dose was gradually decreased and ceased. IVIG infusion was continued for 3 months until complete recovery. Reactivation was not observed in a 1-year follow-up period. Due to its cost, IVIG infusion is less suitable as a first-line agent. However, IVIG may be an important therapeutic option in resistant postpartum PG, in which first-line agents have failed or led to complications.

Internalized Stigma in Pediatric Psoriasis: A Comparative Multicenter Study.

BACKGROUND: Internalized stigma, adoption of negative attitudes and stereotypes of the society regarding persons' illness, has not been studied previously in pediatric psoriasis patients. OBJECTIVE: We aimed to investigate the internalized stigma in pediatric psoriasis patients and to determine differences according to factors affecting internalized stigma compared to adult psoriasis patients. METHODS: This multicenter, cross-sectional, comparative study included 125 pediatric (55 female, 70 male; mean age±standard deviation [SD], 14.59±2.87 years) and 1,235 adult psoriasis patients (577 female, 658 male; mean age±SD, 43.3±13.7 years). Psoriasis Internalized Stigma Scale (PISS), Dermatology Life Quality Index (DLQI), Perceived Health Status (PHS), and the General Health Questionnaire (GHQ)-12 were the scales used in the study. RESULTS: The mean PISS was 58.48±14.9 in pediatric group. When PISS subscales of groups were compared, the pediatric group had significantly higher stigma resistance (p=0.01) whereas adult group had higher scores of alienation (p=0.01) and stereotype endorsement (p=0.04). There was a strong correlation between mean values of PISS and DLQI (r=0.423, p=0.001). High internalized stigma scores had no relation to either the severity or localization of disease in pediatric group. However, poor PHS (p=0.007) and low-income levels (p=0.03) in both groups, and body mass index (r=0.181, p=0.04) in the pediatric group were related to high PISS scores. CONCLUSION: Internalized stigma in pediatric patients is as high as adults and is related to poor quality of life, general health, and psychological illnesses. Unlike adults, internalized stigma was mainly determined by psoriasis per se, rather than disease severity or involvement of visible body parts, genitalia or folds.

Systemic comorbidities associated with rosacea: a multicentric retrospective observational study.

BACKGROUND: Once considered a disorder limited to the skin, rosacea is now known to be associated with systemic disorders. The aim of this study was to determine what systemic comorbidities accompany rosacea and to determine the relationship between the type, severity, and duration of rosacea, and the presence of and type of systemic comorbidities. METHODS: This retrospective multicenter study was conducted by the Turkish Society of Dermatology Acne Study Group. Thirteen dermatology clinics throughout Turkey participated in the study. A structured physician-administered questionnaire was used to collect patient demographics, clinical findings, and lifestyle data. The principal rosacea subtype, physician global assessment of severity, and duration of rosacea were recorded. Physicians recorded each participant's medical history, including current and past comorbidities, duration of any such comorbidity, and the use of medications to treat any comorbidities. RESULTS: The study included 1,195 rosacea patients and 621 controls without rosacea aged 18-85 years. As compared to the controls, more of the rosacea patients had respiratory tract, gastrointestinal system, and metabolic and hepatobiliary system disorders in a rosacea's severity- and duration-dependent manner. CONCLUSION: Clinicians must be aware of the potential for systemic comorbidities in rosacea patients, which becomes more likely as disease duration and severity increase.

Evaluation of sequential effect of isotretinoin on the haematological parameters in patients with acne vulgaris.

PURPOSE: Isotretinoin is the most effective drug for acne with some side effects. Few studies exist regarding the effects of isotretinoin on haematological parameters with different results. Mostly, baseline values with a single value during or at the end of the treatment were compared. In this study, we aimed to determine the differences in haematological parameters during isotretinoin treatment until reaching the cumulative dose of 120 mg/kg. MATERIALS AND METHODS: The study included 118 patients with moderate-to-severe acne vulgaris. Patients with preexisting liver disease, anaemia, iron deficiency, abnormal liver function tests, thrombocytopenia/thrombocytosis or hyperlipidaemia were excluded. Laboratory monitoring for haematological parameters was performed at baseline and monthly during treatment. Parameters at the baseline, at the first and second months and at the end of the therapy were taken into account. RESULTS: According to general linear model analysis, platelets and plateletcrit increased at the first month of the treatment and then decreased to baseline. White blood cells and neutrophils decreased at the first month, then increased to baseline value at the second month, and were found to be decreased again at the end of the treatment. Mean corpuscle volume was found to be increased at the end of the treatment. Other parameters in CBC did not show statistically significant differences. CONCLUSIONS: Although some changes occur in haematological parameters during isotretinoin therapy, all of these changes remain within the normal range. Evaluating the spot values at any time during treatment may cause misinterpretations.

Internalized stigma in psoriasis: A multicenter study.

Internalized stigma is the adoption of negative attitudes and stereotypes of the society regarding a person's illness. It causes decreased self-esteem and life-satisfaction, increased depression and suicidality, and difficulty in coping with the illness. The primary aim of this study was to investigate the internalized stigma state of psoriatic patients and to identify the factors influencing internalized stigma. The secondary aim was to identify the correlation of internalized stigma with quality of life and perceived health status. This multicentre, cross-sectional study comprised 1485 patients. There was a significant positive correlation between mean values of Psoriasis Internalized Stigma Scale (PISS) and Psoriasis Area and Severity Index, Body Surface Area, Dermatological Life Quality Index and General Health Questionnaire-12 (P < 0.001 in all). Lower percieved health score (P = 0.001), early onset psoriasis (P = 0.016), family history of psoriasis (P = 0.0034), being illiterate (P < 0.001) and lower income level (P < 0.001) were determinants of high PISS scores. Mean PISS values were higher in erythrodermic and generalized pustular psoriasis. Involvement of scalp, face, hand, genitalia and finger nails as well as arthropathic and inverse psoriasis were also related to significantly higher PISS scores (P = 0.001). Our findings imply that psoriatic patients experience high levels of internalized stigma which are associated with psoriasis severity, involvement of visible body parts, genital area, folds or joints, poorer quality of life, negative perceptions of general health and psychological illnesses. Therefore, internalized stigma may be one of the major factors responsible from psychosocial burden of the disease.

Malignant acanthosis nigricans associated with ileocecal adenocarcinoma.

Malignant acanthosis nigricans (MAN) is a cutaneous sign of internal malignancy, usually an adenocarcinoma. With this report, we aim to present a rare case of concomitant colon cancer and acanthosis nigricans (AN). A 62-year-old woman with hyperpigmentation, velvety cutaneous thickening and verrucous excrescences involving the axillary, inframammar, and inguinal regions with a history of 10 years is presented. Hyperpigmentation and thickening existed also on the nape, umbilicus, and periorbital regions. Histopathological findings of skin lesions were consistent with AN. Computed tomography of the abdomen revealed cecal wall thickening and multiple enlarged lymph nodes in pericolic fat tissue. The tumor was resected surgically and the diagnosis of poor differentiated adenocarcinoma of the colon with lymphatic metastasis was established. Although MAN is mostly reported in the course of abdominal malignancies, this is a very rare case report of MAN associated with adenocarcinoma of the colon.

Coexistence of psoriasis and bullous pemphigoid: remission with low-dose methotrexate.

The coexistence of psoriasis vulgaris and bullous pemphigoid is rare. Here, we report a 90-year-old patient with a 20-year history of psoriasis. She developed vesicles and bullae suddenly and was diagnosed as bullous pemphigoid. Low-dose methotrexate therapy quickly cleared both psoriatic and bullous lesions.

Expression of nuclear factor kappa B and survivin in psoriasis.

Background and Objective. Suppression of apoptosis has been proposed as a mechanism responsible for epidermal thickness in psoriasis. Survivin is a member of the inhibitor of apoptosis family. Nuclear factor kappa B (NF-κB) is one of the transcriptional factors that regulate many genes affecting apoptosis. The aim of this study was to determine survivin and NF-κB expressions in psoriasis in comparison with normal epidermis. Patients and Methods. Immunohistochemical expressions of survivin and NF-κB were investigated in 41 psoriatic and 21 normal skin samples. Results. Diffuse nuclear survivin expression in all epidermal layers was seen in all of the psoriatic samples. NF-κB expression in different epidermal locations was seen in all of the psoriatic samples. Nuclear staining was positive in 40 psoriasis samples. Similar survivin and NF-κB expressions were observed in normal skin samples. Conclusion. Since similar expressions are seen in both normal and psoriatic epidermis, no important roles for survivin and NF-κB can be attributed in epidermal proliferation and thickness seen in psoriasis.

Serum homocysteine, vitamin B12, folic acid levels and methylenetetrahydrofolate reductase (MTHFR) gene polymorphism in vitiligo.

The aim of this study was to determine serum vitamin B12, folic acid and homocysteine (Hcy) levels as well as MTHFR (C677, A1298C) gene polymorphisms in patients with vitiligo, and to compare the results with healthy controls. Forty patients with vitiligo and 40 age and sex matched healthy subjects were studied. Serum vitamin B12 and folate levels were determined by enzyme-linked immunosorbent assay. Plasma Hcy levels and MTHFR polymorphisms were determined by chemiluminescence and real time PCR methods, respectively. Mean serum vitamin B12 and Hcy levels were not significantly different while folic acid levels were significantly lower in the control group. There was no significant relationship between disease activity and vitamin B12, folic acid and homocystein levels. No significant difference in C677T gene polymorphism was detected. Heterozygote A1298C gene polymorphism in the patient group was statistically higher than the control group. There was no significant relationship between MTHFR gene polymorphisms and vitamin B12, folic acid and homocysteine levels. In conclusion, vitamin B12, folate and Hcy levels are not altered in vitiligo and MTHFR gene mutations (C677T and A1298C) do not seem to create susceptibility for vitiligo.

Efficacy of 5% imiquimod cream in a patient with classic Kaposi sarcoma.

BACKGROUND: The classic Kaposi sarcoma is most common in the Mediterranean population over 50 years of age and presents with reddish-brown papules and nodules particularly on the lower limbs. Treatment depends on the clinical presentation and extension of lesions. Imiquimod is as an immune response modifier with antiangiogenic activity. MAIN OBSERVATIONS: We present a 74-year-old man with classic Kaposi sarcoma who had multiple, small, violaceous papules and nodules on the trunk and extremities with a history of 14 years. He complained particularly from plantar hyperkeratotic painful nodules. Treatment with imiquimod 5% cream under occlusion resulted with almost complete regression within 12 weeks. No local or systemic side effects were observed. CONCLUSIONS: Topical imiquimod was a safe and effective therapy in our patient with classic Kaposi sarcoma.

Oxidative stress impairs endothelial nitric oxide levels in Behçets' disease.

BACKGROUND: Behçet's disease (BD) is an inflammatory vasculitis. Endogenous nitric oxide (NO), produced by endothelial cells, has pleiotropic effects such as vasodilatator, antiplatelet, antiproliferative. Reactive oxygen species (ROS) are produced at sites of endothelial inflammation. ROS target polyunsaturated lipids, which results in malondialdehyde (MDA) production. OBJECTIVE: The aim was to investigate the oxidative stress in BD patients by measuring MDA and total antioxidant status (TAS) levels and to establish a possible relationship with respect to NO levels regarding disease activity. MATERIALS AND METHODS: 55 BD patients (30 active/25 inactive) and 20 healthy volunteers were included in the study. Blood samples were drawn following an overnight fasting. TAS and MDA levels were determined spectrophotometrically. Serum nitrite (NO(2-)) and nitrate (NO(3-)) levels were measured to estimate NO production. Data were expressed as mean ± SD. RESULTS: TAS levels were significantly lower in BD patients than the controls (1.19 ± 0.34 vs. 3.29 ± 0.89 mmol/L). In the active BD group, MDA levels (0.36 ± 0.19 nmol/mL) were significantly higher than both the inactive BD group (0.25 ± 0.18 nmol/mL) and controls (0.18 ± 0.41 nmol/mL). NO levels were significantly lower in the active group compared to the inactive group (18.0 ± 2.80 vs. 19.40 ± 2.70 µmol/L). MDA levels correlated negatively with NO levels in the active group. CONCLUSION: Decreased NO levels mediated by increased oxidative stress significantly contribute to endothelial dysfunction observed in BD.

Imiquimod 5% cream for isolated lichen planus of the lip.

Lichen planus (LP) of the lips is a rare condition that is generally associated with other parts of the oral mucosa. Lip localization has an increased risk, since external trauma, smoking and ultraviolet light trigger malignant transformation. Only a few cases of isolated LP of the lips have been reported up to now, but results of larger series on oral LP suggest that it might be underestimated. Treatment of oral LP is usually difficult and lesions are generally resistant or recur, so that novel therapy alternatives are necessary. Here we report four cases of isolated LP of the lip successfully treated with imiquimod 5% cream. It was applied twice daily, 5 days a week, for 2 weeks. Two weeks after therapy, complete clinical and histopathological resolution was observed. No recurrence was observed during the 5, 10 and 18 months' follow-up period in cases 4, 3 and 1, respectively. Clinical and histopathological cure was also observed in case 2, but the patient showed clinical activation after 6 months. We suggest that imiquimod 5% cream is a safe and effective therapeutic treatment for oral LP.

Primary cutaneous anaplastic CD30 + large-cell lymphoma that completely regressed after incisional skin biopsy.

We describe a 48-year-old woman with three erythematous nodules localized on the left forearm, with 2 months evolution. Histological and immunohistochemical examination revealed a CD30(+) large-cell lymphoma. Systemic involvement was not detected. The tumor regressed spontaneously within a week, after the incisional skin biopsy. In control skin biopsy, there was not any histological feature of lymphoma. No reactivation or any symptom of systemic disease was observed during the 10-month follow-up period.

Therapeutic hotline: Treatment of prurigo nodularis and lichen simplex chronicus with gabapentin.

Psychocutaneous conditions are frequently encountered in dermatology practice. Prurigo nodularis and lichen simplex chronicus are two frustrating conditions that are classified in this category. They are often refractory to classical treatment with topical corticosteroids and antihistamines. Severe, generalized exacerbations require systemic therapy. Phototherapy, erythromycine, retinoids, cyclosporine, azathiopurine, naltrexone, and psychopharmacologic agents (pimozide, selective serotonin reuptake inhibitor antidepressants) were tried with some success. Here five cases with lichen simplex chronicus and four cases with prurigo nodularis, who responded well to gabapentin, are presented.