Treatment patterns and outcomes in patients with chronic urticaria during pregnancy: Results of PREG-CU, a UCARE study.

BACKGROUND: Although chronic urticaria (CU) is a common and primarily affects females, there is little data on how pregnancy interacts with the disease. OBJECTIVE: To analyse the treatment use by CU patients before, during and after pregnancy as well as outcomes of pregnancy. METHODS: PREG-CU is an international, multicentre study of the Urticaria Centers of Reference and Excellence network. Data were collected via a 47-item-questionnaire completed by CU patients who became pregnant during their disease course. RESULTS: Questionnaires from 288 CU patients from 13 countries were analysed. During pregnancy, most patients (60%) used urticaria medication including standard-dose second generation H1-antihistamines (35.1%), first generation H1-antihistamines (7.6%), high-dose second-generation H1-antihistamines (5.6%) and omalizumab (5.6%). The preterm birth rate was 10.2%; rates were similar between patients who did and did not receive treatment during pregnancy (11.6% vs. 8.7%, respectively). Emergency referrals for CU and twin birth were risk factors for preterm birth. The caesarean delivery rate was 51.3%. More than 90% of new-borns were healthy at birth. There was no link between any patient or disease characteristics or treatments and medical problems at birth. CONCLUSION: Most CU patients used treatment during pregnancy especially second-generation antihistamines which seem to be safe during pregnancy regardless of the trimester. The rates of preterm births and medical problems of new-borns in CU patients were similar to population norms and not linked to treatment used during pregnancy. Emergency referrals for CU increased the risk of preterm birth and emphasize the importance of sufficient treatment to keep urticaria under control during pregnancy.

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.

Effects of pregnancy on chronic urticaria: Results of the PREG-CU UCARE study.

BACKGROUND: Chronic urticaria (CU) predominantly affects women, and sex hormones can modulate disease activity in female CU patients. As of now, the impact of pregnancy on CU is largely unknown. AIM: To analyze the course and features of CU during and after pregnancy. PATIENTS AND METHODS: PREG-CU is an international, multicenter study of the Urticaria Centers of Reference and Excellence (UCARE) network. Data were collected via a 47-item questionnaire completed by CU patients, who became pregnant within the last 3 years. RESULTS: A total of 288 pregnancies of 288 CU patients from 13 countries were analyzed (mean age at pregnancy: 32.1 ± 6.1 years, duration of CU: 84.9 ± 74.5 months; CSU 66.9%, CSU + CIndU 20.3%, CIndU 12.8%).During pregnancy, 51.1% of patients rated their CU as improved, 28.9% as worse, and 20.0% as unchanged.CU exacerbations most commonly occurred exclusively during the third trimester (in 34 of 124 patients; 27.6%) or the first (28 of 124; 22.8%). The risk factors for worsening of CU during pregnancy were having mild disease and no angioedema before pregnancy, not taking treatment before pregnancy, CIndU, CU worsening during a previous pregnancy, treatment during pregnancy, and stress as a driver of exacerbations. After giving birth, urticaria disease activity remained unchanged in 43.8% of CU patients, whereas 37.4% and 18.1% experienced worsening and improvement, respectively. CONCLUSIONS: These results demonstrate the complex impact of pregnancy on the course of CU and help to better counsel patients who want to become pregnant and to manage CU during pregnancy.

Patch Test Results to European Baseline Series in Turkey: A Prospective and Multicenter Study.

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.

Chronic Nodular Prurigo: A European Cross-sectional Study of Patient Perspectives on Therapeutic Goals and Satisfaction.

Chronic nodular prurigo is characterized by recalcitrant itch. Patient perspectives on therapeutic goals, satisfaction with therapy and efficacy of therapeutic regimens for this condition are unknown. This questionnaire study examined these issues in 406 patients with chronic nodular prurigo from 15 European dermatological centres. Improvements in itch, skin lesions and sleep were the most important goals. Emollients, topical corticosteroids and antihistamines were the most frequently used treatments, while a minority of patients were prescribed potent medications, such as systemic immunosuppressants and gabapentinoids. Most patients were not satisfied with their previous therapy (56.8%), while 9.8% did not receive any therapy despite having active disease. A substantial number of respondents (28.7%) considered none of the therapeutic options effective. Although chronic nodular prurigo is a severe disease, most patients were not treated with potent systemic drugs, which may contribute to the high levels of dissatisfaction and disbelief in available therapies. Specific guidelines for chronic nodular prurigo and the development of novel therapies are necessary to improve care.

European S2k Guideline on Chronic Pruritus.

Pruritus is a frequent symptom in medicine. Population-based studies show that every 5th person in the general population has suffered from chronic pruritus at least once in the lifetime with a 12-month incidence of 7%. In patient populations its frequency is much higher depending on the underlying cause, ranging from around 25% in haemodialysis patients to 100% in skin diseases such as urticaria and atopic dermatitis (AD). Pruritus may be the result of a dermatological or non-dermatological disease. Especially in non-diseased skin it may be caused by systemic, neurological or psychiatric diseases, as well as being a side effect of medications. In a number of cases chronic pruritus may be of multifactorial origin. Pruritus needs a precise diagnostic work-up. Management of chronic pruritus comprises treatment of the underlying disease and topical treatment modalities, including symptomatic antipruritic treatment, ultraviolet phototherapy and systemic treatment. Treating chronic pruritus needs to be targeted, multimodal and performed in a step-wise procedure requiring an interdisciplinary approach. We present the updated and consensus based (S2k) European guideline on chronic pruritus by a team of European pruritus experts from different disciplines. This version is an updated version of the guideline that was published in 2012 and updated in 2014 (www.euroderm.org).

Humanistic burden of chronic pruritus in patients with inflammatory dermatoses: Results of the European Academy of Dermatology and Venereology Network on Assessment of Severity and Burden of Pruritus (PruNet) cross-sectional trial.

BACKGROUND: Chronic pruritus is a multifactorial, challenging symptom of global relevance. OBJECTIVE: The European Academy of Dermatology and Venereology Network on Assessment of Severity and Burden of Pruritus (PruNet) investigation aimed to analyze the severity and humanistic burden of chronic pruritus in patients suffering from inflammatory dermatoses across Europe. METHODS: Prospectively collected routine data on 552 patients (with atopic dermatitis, contact dermatitis, prurigo nodularis, psoriasis vulgaris, lichen planus, or mycosis fungoides [pruritus numeric rating scale score ≥3]) from 9 European centers (in Austria, France, Germany, Italy, Poland, Russia, Spain, Switzerland, and Turkey) were analyzed by univariate and multivariate variance analyses of various itch characteristics and quality of life (as measured by the Dermatology Life Quality Index and the ItchyQoL). RESULTS: Duration, frequency, and intensity of pruritus (according to a numeric rating scale and visual analog scale) and related impairment of quality of life differed between European centers and dermatologic diagnoses (P < .05). The country in which the center was located had a greater impact on how patients evaluated pruritus intensity and quality of life than diagnosis did (P < .001). LIMITATIONS: One center per country was included. CONCLUSION: The humanistic burden of chronic pruritus in patients with inflammatory dermatoses is high. European cross-cultural factors may have a stronger influence than a specific dermatologic diagnosis on how patients rate intensity of pruritus and quality of life.

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.

Macroglossia as a presenting feature of multiple myeloma.

Macroglossia has been very rarely reported as a first clinical sign of multiple myeloma.

18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Imaging in a Patient with HIV (-) Kaposi Sarcoma.

Kaposi sarcoma (KS) is a vascular neoplasm that often manifests with multiple vascular nodules on the skin and other organs. Various imaging modalities can be used to display disease extent. Herein we present a 65-year-old female patient with human immunodeficiency virus negative KS along with her whole-body positron emission tomography/computed tomography imaging findings.

Neurologic Itch Management.

Neurologic itch is defined as pruritus resulting from any dysfunction of the nervous system. Itch arising due to a neuroanatomic pathology is seen to be neuropathic. Causes of neuropathic itch range from localized entrapment of a peripheral nerve to generalized degeneration of small nerve fibers. Antipruritic medications commonly used for other types of itch such as antihistamines and corticosteroids lack efficacy in neuropathic itch. Currently there are no therapeutic options that offer relief in all types of neuropathic pruritus, and treatment strategies vary according to etiology. It is best to decide on the appropriate tests and procedures in collaboration with a neurologist during the initial work-up. Treatment of neuropathic itch includes general antipruritic measures, local or systemic pharmacotherapy, various physical modalities, and surgery. Surgical intervention is the obvious choice of therapy in cases of spinal or cerebral mass, abscess, or hemorrhagic stroke, and may provide decompression in entrapment neuropathies. Symptomatic treatment is needed in the vast majority of patients. General antipruritic measures should be encouraged. Local treatment agents with at least some antipruritic effect include capsaicin, local anesthetics, doxepin, tacrolimus, and botulinum toxin A. Current systemic therapy relies on anticonvulsants such as gabapentin and pregabalin. Phototherapy, transcutaneous electrical nerve stimulation, and physical therapy have also been of value in selected cases. Among the avenues to be explored are transcranial magnetic stimulation of the brain, new topical cannabinoid receptor agonists, various modes of acupuncture, a holistic approach with healing touch, and cell transplantation to the spinal cord.

Widespread miliaria crystallina in a newborn with hypernatremic dehydration.

Miliaria, which is extravasation of sweat into the epidermis and dermis due to sweat duct obstruction, is a commonly seen dermatologic disorder in newborns. We present a case of extensive, widespread miliaria crystallina that developed in a newborn during treatment of severe hypernatremic dehydration. A possible mechanism could be the destruction of sweat ducts with excretion of sweat with high levels of sodium.

Rosacea treatment with intermediate-dose isotretinoin: follow-up with erythema and sebum measurements.

Isotretinoin is one of the therapeutic options for rosacea. However, the response of erythema to treatment with isotretinoin is usually slow and incomplete with common (0.5-1 mg/kg/day) or low (10 mg/day) doses. This study investigated the efficacy of, and relapse on, 20 mg/day isotretinoin treatment in rosacea, with the aid of instrumental measurement of facial erythema and sebum levels. A 20 mg/day dose of isotretinoin was given for 4 months, and then the dose was tapered off within the following 6 months. A total of 25 patients were included in the study. Papule and pustule counts, erythema index, sebum level, dermatologist's and patient's erythema scores, and dermatologist's sebum scores were significantly lower in the first month of therapy compared with pre-treatment values (p < 0.05). Within a median follow-up of 11 months (95% confidence interval: 8.4-13.5 months) 45% of patients had a relapse. In conclusion, 20 mg/day isotretinoin was rapidly efficient for reducing both inflammatory lesions and erythema in rosacea.

Plasmacytoid dendritic cell tumor: A case report.

A 62-year-old man presented with a painless eruption on his arms and trunk. Physical examination showed 2 well-demarcated erythematous plaques on the anterior trunk and 6 purple-red papules on the back and upper extremities. Blood chemistry and computed tomography results were normal. Herein we describe a patient with plasmacytoid dendritic cell neoplasm in the absence of systemic symptoms.

Green staining of clothing: a signal for pseudomonal infection.

Anticonvulsant hypersensitivity syndrome (AHS) is a nondose-related idiosyncratic reaction to aromatic antiepileptic drugs and is a cause of drug discontinuation. Pseudomonas aeruginosa is a gram-negative bacillus that can produce infections in many different organs, including the skin and soft tissue. We report a patient with erythroderma and AHS who developed a pseudomonal infection. Green staining of the underwear served as a diagnostic clue for severe P aeruginosa infection that had developed because of a local flexural skin infection that spread due to a damaged skin barrier. Inspection of the patient's clothes may give information about any exudate from the skin and should be done routinely as part of the physical examination.

Investigation of the inflammatory mechanisms in alopecia areata.

We aimed to investigate the profile of the inflammatory infiltrate in lesional and nonlesional tissue in alopecia areata (AA) and look for possible associations between inflammatory mechanisms, neuropeptide expressions, and various clinical features. Twenty-four patch-type AA patients were included. Forty-eight lesional and nonlesional skin samples were stained immunohistochemically with antibodies for CD1a, CD3, CD4, CD8, CD20, CD57 (for natural killer cells), mast cell tryptase, nerve growth factor receptor (NGFR), and substance P (SP). Various clinical findings were recorded. Psychological distress levels and stress-related hormones were measured. Lesional skin showed statistically more CD3(+), CD8(+), and CD57(+) lymphocytes, mast cells, Langerhans cells, and more prominent immunoreactivities of NGFR and SP (P < 0.003). Most nonlesional skin showed CD3(+) and CD57(+) cells, mast cells, and NGFR(+) nerve fibers. NGFR and SP, and SP and perivascular mast cell infiltrates were correlated, whereas peribulbar mast cells and anagen follicle counts were inversely correlated in nonlesional skin (P < 0.05). Near half of the patients' distress levels were high. No relationship among biochemical, psychological, and clinical parameters could be shown. AA may involve the entire skin in which lesions occur as a result of local T cell-mediated cytotoxic inflammatory response initiated by Langerhans cells and mast cells activated via neuropeptides.

Cutaneous metastatic adenocarcinoma arising from a malignant sacrococcygeal teratoma in an adult.

We present a patient with cutaneous metastasis caused by an adenocarcinoma arising from a malignant teratoma. A 37-year-old woman seen for the complaint of swelling in the genital region also complained of a draining mass in her gluteal region present since birth. Physical examination showed marked edema in the labia majora, multiple hyperkeratotic papules in the left labium majus, and erythema, induration, and swelling in the left femoral and inguinal regions. A soft tumor that exhibited sinus tracts was palpated in the left gluteus. Excision of the gluteal tumor revealed a teratoma. Vulvar skin biopsy confirmed a mucinous adenocarcinoma which had derived from this teratoma. A tumor that arises from pluripotent germ cells, teratoma rarely shows malignant transformation. The patient presented is a rare example of a cutaneous metastasis originating from a congenital sacrococcygeal teratoma in an adult.

A rare cause of neonatal seizure: incontinentia pigmenti.

Incontinentia pigmenti (IP) is a rare genetic multisystem disorder that may affect many organs including the skin, bone, eyes and the central nervous system. Central nervous system manifestations are seen in 30% of cases with seizures and mental retardation. Seizures occurring as the presenting sign of IP are rarely reported. We report a case of a female newborn with IP who had seizures on day 4 of life, which were followed in her second month by the development of the characteristic cutaneous changes for IP. With this case report, we would like to emphasize the need for inclusion of IP in the differential diagnosis of neonatal seizures.