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Psoriasis is a common multisystem inflammatory disease, and arthritis is an essential component of the disorder, requiring early diagnosis and prompt treatment for successful management. In this study, we aimed to investigate the relationship between nail and scalp involvement and other covariates with psoriatic arthritis (PsA). This cross-sectional study, conducted from June 2021 through December 2021, included 763 patients from 11 different centers in Turkey. The severity of involvement was evaluated using psoriasis area severity index (PASI), nail psoriasis severity index (NAPSI), and psoriasis scalp severity index (PSSI) scores. Predictors for PsA were evaluated using univariate and multivariate logistic regression models. PsA (nâ =â 155, 21.5%) was significantly more common in patients having a family history of psoriasis (43.2% vs 30.9%, Pâ =â .004), nail involvement (68.4% vs 52.3%, Pâ <â .001), and coexistence of nail and scalp involvement (53.7% vs 39.6%, Pâ =â .002). Furthermore, patients with PsA had considerably higher PASI (7 vs 5.6, Pâ =â .006), NAPSI (5 vs 2, Pâ <â .001), and PSSI scores (7 vs 4, Pâ =â .002) and longer disease duration (months) (126 vs 108, Pâ =â .009). In multivariate analysis, female gender [OR: 3.01, 95% CI (1.861-4.880), Pâ <â .001], nail involvement [OR: 2.06, 95% CI (1.293-3.302), Pâ =â .002)], and body mass index (BMI) [OR: 1.06, 95% CI (1.017-1.100), Pâ =â .005] were identified as independent predictors for PsA. Female gender, nail involvement, and high BMI are significant predictors for PsA and warrant detailed rheumatological assessment. Notably, being female is the strongest predictor of increased risk of PsA in our survey. Scalp involvement appears not to be associated with PsA. Also, the presence of PsA seems related to a more severe skin involvement phenotype.
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Artrite Psoriásica , Doenças da Unha , Couro Cabeludo , Índice de Gravidade de Doença , Humanos , Artrite Psoriásica/epidemiologia , Artrite Psoriásica/complicações , Estudos Transversais , Feminino , Masculino , Turquia/epidemiologia , Pessoa de Meia-Idade , Adulto , Doenças da Unha/etiologia , Doenças da Unha/epidemiologia , Couro Cabeludo/patologia , Psoríase/complicações , Psoríase/epidemiologia , Dermatoses do Couro Cabeludo/epidemiologia , IdosoRESUMO
INTRODUCTION: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening acute mucocutaneous disorders usually triggered by drugs. In this study, we aimed to evaluate the factors affecting mortality in patients with SJS-TEN. METHODS: Our study is a retrospective cohort study, analyzing data collected from a total of 12 tertiary care centers between April 2012 and April 2022. RESULTS: The study included 59 males and 107 females, a total of 166 patients, with an average age of 50.91 ± 21.25 years. Disease classification was TEN in 50% of cases, SJS in 33.1%, and SJS-TEN overlap in 16.9%. The average SCORTEN within the first 24 h was 2.44 ± 1.42. Supportive care was provided to 99.4% of patients. The most commonly used systemic immunomodulatory treatments were systemic steroids (84.3%), IVIG (intravenous immunoglobulin) (49.3%), and cyclosporine (38.6%). Plasmapheresis was administered to five patients. While 66.3% of patients were discharged, 24.1% resulted in exitus. Our comparative analysis of survivors and deceased patients found no effect of systemic steroids, IVIG, and cyclosporine treatments on mortality. Univariate analysis revealed that the SCORTEN scores on days 1 and 3 as well as the rates of detachment at the onset and during follow-up were significantly higher in deceased patients compared to survivors. The rates of fever, positive blood cultures, and systemic antibiotic use were higher in deceased patients compared to survivors. The presence of comorbidities, diabetes, and malignancy were significantly more common in deceased patients. Multivariate regression analysis indicated that over SCORTEN 2, the mortality risk exponentially rose with each SCORTEN increment, culminating in an 84-fold increase in mortality at SCORTEN 5-6 (odds ratio [95% confidence interval]: 13.902-507.537, p < 0.001) compared to SCORTEN 0-1. Additionally, the utilization of plasmapheresis was associated with a 22-fold increase in mortality (odds ratio [95% confidence interval]: 1.96-247.2, p = 0.012). CONCLUSION: Our study found that a high SCORTEN score within the first 24 h and the use of plasmapheresis were related to increased mortality, while systemic steroids, IVIG, and cyclosporine treatments had no impact on mortality. We believe that data gathered from one of the most comprehensive studies which we conducted on SJS-TEN will enrich the literature, although additional research is warranted.
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BACKGROUND: Chronic Spontaneous Urticaria (CSU) is characterized by recurrent wheals and/or angioedema for longer than 6-weeks. Guidelines recommend Omalizumab (Oma) as first-line and Cyclosporine-A (Cs-A) as second-line treatment in antihistamine resistant CSU. This step-wise algorithm might be time-consuming and costly. OBJECTIVE: To determine indicators of response to Oma or Cs-A in CSU patients. METHODS: We retrospectively analyzed data from seven centers in Turkey; the inclusion criteria for patients were to receive both Oma and Cs-A treatment (not concurrently) at some point in time during their follow-up. Clinical and laboratory features were compared between groups. RESULTS: Among 110 CSU patients; 47 (42.7%) were Oma-responders, 15 (13.6%) were Cs-A-responders, and 24 (21.8%) were both Oma and Cs-A responders and 24 (21.8%) were non-responders to either drug. High CRP levels were more frequent in Cs-A-responders (72.7% vs. 40.3%; p = 0.055). Oma-responders had higher baseline UCT (Urticaria Control Test) scores (6 vs. 4.5; p = 0.045). Responders to both drugs had less angioedema and higher baseline UCT scores compared to other groups (33.3% vs. 62.8%; p = 0.01 and 8 vs. 5; p = 0.017). Non-responders to both drugs had an increased frequency in the female gender and lower baseline UCT scores compared to other groups (87.5% vs. 61.6%; p = 0.017 and 5 vs. 7; p = 0.06). STUDY LIMITATIONS: Retrospective nature, limited number of patients, no control group, the lack of the basophil activation (BAT) or BHRA (basophil histamine release assay) tests. CONCLUSIONS: Baseline disease activity assessment, which considers the presence of angioedema and disease activity scores, gender, and CRP levels might be helpful to predict treatment outcomes in CSU patients and to choose the right treatment for each patient. Categorizing patients into particular endotypes could provide treatment optimization and increase treatment success.
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Angioedema , Antialérgicos , Urticária Crônica , Urticária , Doença Crônica , Ciclosporina , Feminino , Humanos , Omalizumab , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Birt-Hogg-Dube syndrome (BHDS) is a rare disorder characterized by the triad of cutaneous lesions, renal tumors, lung cysts and inactivation of the gene folliculin (FLCN). Here, we present three female patients diagnosed with BHDS. First case a 55-year-old female had flesh moles histopathology compatible with angiofibroma, multiple cysts in the lung and kidneys, FLCN gene mutations ('c.1285dupC [p.His429Profs*]' 11th exon and 'c.653G>A [p.Arg258His]' 7th exon). The second case a 76-year-old female had trichodiscoma on her skin, multiple cysts in the lung, spontaneous pneumothorax, FLCN gene mutation 'c.1285dupC (p.His429Profs*27) 11th exon' and, her son had renal carcinoma history under 50 years of age. Our third case, also the daughter of case 2, had dermal papules histopathology compatible with trichodiscoma, spontaneous pneumothorax, FLCN gene mutation 'c.1285dupC (p.His429Profs*27) 11th exon' and, parotid oncocytoma. Through our cases, we document the first case of two mutations ('c.1285dupC [p.His429Profs*]' 11th exon and 'c.653G>A [p.Arg258His]' 7th exon) in the same FLCN gene and the 11th known case of parotid oncocytoma associated with BHDS in the light of the literature.
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BACKGROUND: Patch testing with the European Baseline Series (EBS) is an essential diagnostic tool for the assessment of allergic contact dermatitis. OBJECTIVE: The aims of the study were to describe the most common contact allergens identified with patch testing in Turkey and to establish the distribution of contact allergens among different regions in Turkey. PATIENTS AND METHODS: Twelve centers consisting of tertiary health care institutions were included in the study. A total of 1169 patients were patch tested with the EBS from 2015 to 2017. RESULTS: A total of 596 patients (51.0%) had 1 or more positive patch test reactions to the EBS. A total of 30.2% (n = 353) of those tested had clinical relevance. Contact sensitivity was more common in young patients. The most common 10 allergens were nickel sulfate (20.4%), followed by textile dye mix (8.6%), cobalt chloride (8.3%), potassium dichromate (8.0%), p-phenylenediamine base (4.5%), balsam of Peru (4.3%), methylchloroisothiazolinone/methylisothiazolinone (3.8%), thiuram mix (3.6%), fragrance mix I (3.0%), and methylisothiazolinone (2.9%). CONCLUSIONS: This study provides a comprehensive profile of patch test results with the EBS in Turkey. An ongoing decrease in contact allergy to methylchloroisothiazolinone/methylisothiazolinone and methylisothiazolinone was observed. Textile dye mix and p-phenylenediamine are beginning to pose a new risk in Turkey.
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Alérgenos/administração & dosagem , Dermatite Alérgica de Contato/diagnóstico , Dermatite Alérgica de Contato/epidemiologia , Testes do Emplastro/normas , Alérgenos/efeitos adversos , Humanos , Prevalência , Estudos Prospectivos , TurquiaRESUMO
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.
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Pênfigo/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Pênfigo/diagnóstico , Pênfigo/imunologia , Estudos Prospectivos , Turquia/epidemiologia , Adulto JovemRESUMO
BACKGROUND/AIM: Narrowband UVB (Nb UVB) treatment is commonly used for the management of psoriasis and atopic dermatitis, and is less often used for vitiligo in children. The aim of this study was to evaluate the efficacy and short-term safety of Nb UVB phototherapy in children diagnosed with vitiligo retrospectively. MATERIALS AND METHODS: A total of 26 patients younger than 18 years with the diagnosis of vitiligo and managed with Nb UVB phototherapy as documented in archive records were evaluated. Clinical response was assessed according to repigmentation of the lesions: good response when there was more than 75% repigmentation, moderate response when there was 25%-74% repigmentation, poor response when repigmentation was less than 24%, and unresponsive when there was no pigmentation and new lesions occurred. RESULTS: A total of 26 patients received Nb UVB treatment; 14 were girls and 12 were boys. The age at onset of the disease varied between 2 and 18 years, with a mean age of onset of 10.07 ± 4.53 years. Repigmentation rate of >75% was detected in 45.4% of cases. CONCLUSION: Nb UVB phototherapy seems to be a well-tolerated effective and safe treatment option in children, especially those unresponsive to topical treatment and those with widespread lesions. However, long-term risks such as photocarcinogenesis and photoaging should kept in mind.
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Terapia Ultravioleta , Vitiligo/radioterapia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Terapia Ultravioleta/métodos , Vitiligo/patologiaRESUMO
Levetiracetam is a relatively new antiepileptic drug that has previously not been associated with severe dermatological side effects. We report the case of a 31-year-old male treated with levetiracetam for seizures who subsequently developed a mycosis fungoides-like drug reaction that resolved upon dosage reduction.
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Warty dyskeratoma is a rare tumor that presents mainly as an isolated papule or nodule on the scalp, face or neck in the adults. Warty dyskeratoma frequently arises as a single lesion with a central keratotic plug on the skin of middle-aged or elderly people. Multiple warty dyskeratomas are very rare cutaneous lesions which are mostly seen on the scalp. We herein report a multiple warty dyskeratoma in a female patient.
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Cutaneous bronchogenic anomalies are very rare lesions, and most of them present as cysts and/or sinus tracts in the subcutaneous layer. We report a 15-year-old boy who presented with a crusted and papillomatous plaque over the right scapular region. Histopathological examination of this plaque revealed pseudostratified ciliated columnar epithelium with papillary invaginations that included mucinous material. The lesion had no cyst or sinus formation and was bordered with smooth muscle bundles in stroma.
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Cisto Broncogênico/diagnóstico , Coristoma/diagnóstico , Escápula , Dermatopatias/diagnóstico , Adolescente , Cisto Broncogênico/patologia , Coristoma/patologia , Diagnóstico Diferencial , Humanos , Masculino , Dermatopatias/patologiaRESUMO
Sweet syndrome or acute febrile neutrophilic dermatosis is a condition characterized by painful erythematous papules, nodules, and plaques, which demonstrate mature neutrophilic infiltration of the upper dermis histopathologically, and is usually associated with systemic symptoms such as fever and neutrophilia. Generalized Sweet syndrome lesions are usually noted in association with malignancies. Sweet syndrome lesions are rarely seen in patients with Behçet disease and, if present, are usually fewer in number. In this report, we present two female patients who developed generalized Sweet syndrome lesions during follow-up after the diagnosis of Behçet disease. The clinical and histopathologic findings in our patients together with the data in the literature suggest that generalized Sweet syndrome lesions in a patient with clinically inactive Behçet disease should remind the clinician that a strict differential diagnosis needs to be made between Sweet syndrome associated with Behçet disease and the rare cutaneous manifestation of Behçet disease that resembles the lesions of Sweet syndrome.
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Síndrome de Behçet/complicações , Síndrome de Sweet/etiologia , Adulto , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/fisiopatologia , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Síndrome de Sweet/diagnóstico , Síndrome de Sweet/fisiopatologiaAssuntos
Aminoquinolinas/administração & dosagem , Antineoplásicos/administração & dosagem , Carcinoma Basocelular/tratamento farmacológico , Interferon-alfa/administração & dosagem , Neoplasias Cutâneas/tratamento farmacológico , Administração Tópica , Quimioterapia Combinada , Feminino , Humanos , Imiquimode , Interferon alfa-2 , Pessoa de Meia-Idade , Neoplasias Nasais/tratamento farmacológico , Proteínas RecombinantesRESUMO
Multiple miliary osteoma cutis of the face represents primary extra-skeletal bone formation that arises within the skin of the face. Multiple miliary osteoma cutis of the face is a rare complication of chronic inflammatory acne vulgaris and has invasive and non-invasive treatment alternatives different from acne vulgaris. Invasive techniques should be simple, easy, and inexpensive, with minimal risk of scarring and pigmentation. We used a needle microincision-extirpation technique in a patient with multiple miliary osteoma cutis unresponsive to non-invasive treatment modalities. Skin overlying the papules was incised with a needle and then the calcificated papules were extirpated by using a small curettage device. Lesions were left to secondary healing. Results were quite good and cosmetically acceptable.
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Dermatoses Faciais/cirurgia , Ossificação Heterotópica/cirurgia , Acne Vulgar/complicações , Face/patologia , Face/cirurgia , Dermatoses Faciais/etiologia , Dermatoses Faciais/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Procedimentos Cirúrgicos Menores/métodos , Agulhas , Ossificação Heterotópica/etiologia , Ossificação Heterotópica/patologiaAssuntos
Paclitaxel/uso terapêutico , Sarcoma de Kaposi/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Idoso , Antineoplásicos Fitogênicos/administração & dosagem , Antineoplásicos Fitogênicos/uso terapêutico , Relação Dose-Resposta a Droga , Esquema de Medicação , Feminino , Humanos , Imuno-Histoquímica , Paclitaxel/administração & dosagem , Proteínas Proto-Oncogênicas c-bcl-2/análise , Sarcoma de Kaposi/metabolismo , Sarcoma de Kaposi/patologia , Neoplasias Cutâneas/metabolismo , Neoplasias Cutâneas/patologia , Resultado do TratamentoRESUMO
Parry-Romberg syndrome (PRS) or progressive hemifacial atrophy is a rare entity characterized by unilateral atrophy of the skin, subcutaneous tissue and sometimes bone and cartilage. Although this syndrome has overlapping features of scleroderma 'en coup de sabre', it shows little or no sclerosis and may affect the entire distribution of the trigeminal nerve including the eye and tongue. As the pathogenesis is unknown, no effective therapy exists. We present here the third case of PRS associated with borreliosis and more interestingly two cases whose progressive course have been stabilized with gel PUVA therapy.
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Infecções por Borrelia/diagnóstico , Hemiatrofia Facial/diagnóstico , Adulto , Infecções por Borrelia/complicações , Infecções por Borrelia/tratamento farmacológico , Infecções por Borrelia/patologia , Criança , Diagnóstico Diferencial , Hemiatrofia Facial/complicações , Hemiatrofia Facial/tratamento farmacológico , Hemiatrofia Facial/patologia , Feminino , Humanos , Masculino , Terapia PUVA , Couro Cabeludo/patologia , Língua/patologiaRESUMO
Epidermodysplasia verruciformis (EV) is a very rare and chronic disease characterized by a susceptibility to cutaneous infections by a group of phylogenetically related human papillomavirus (HPV) types. We present here a 52-year-old man previously diagnosed and treated as squamous cell carcinoma but also found to have epidermodysplasia verruciformis lesions in association with Bowen's disease. The patient was effectively treated with a combination of interferon therapy (6 million units, twice a week) and topical imiquimod for Bowen's disease (five times a week). We conclude that topical imiquimod is very effective in the treatment of Bowen's disease and may be combined with systemic interferon therapy in rare cases with multiple vivid presentations of HPV infections.
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Doença de Bowen/diagnóstico , Carcinoma de Células Escamosas/diagnóstico , Epidermodisplasia Verruciforme/diagnóstico , Neoplasias Cutâneas/diagnóstico , Administração Cutânea , Aminoquinolinas/administração & dosagem , Aminoquinolinas/uso terapêutico , Antineoplásicos/administração & dosagem , Antineoplásicos/uso terapêutico , Doença de Bowen/complicações , Doença de Bowen/tratamento farmacológico , Doença de Bowen/patologia , Carcinoma de Células Escamosas/complicações , Carcinoma de Células Escamosas/tratamento farmacológico , Carcinoma de Células Escamosas/patologia , Diagnóstico Diferencial , Quimioterapia Combinada , Epidermodisplasia Verruciforme/complicações , Epidermodisplasia Verruciforme/tratamento farmacológico , Epidermodisplasia Verruciforme/patologia , Humanos , Imiquimode , Interferons/administração & dosagem , Interferons/uso terapêutico , Masculino , Pessoa de Meia-Idade , Couro Cabeludo/patologia , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/patologiaRESUMO
Localized unilateral hyperhidrosis (LUH) is an uncommon but well-defined special form of localized hyperhidrosis with diverse causes. It often occurs within a sharply demarcated area on the face or upper extremities. Here, we report a case of LUH in a 16-year-old male with neurofibromatosis type 1 (NF1) and review related data in the literature. The association of this rare entity and NF1 has not yet been described. Subcutaneous botulinum toxin A injection provided a good clinical response with symptomatic relief.