Immunogenesis and blood reactivity in early childhood Prof. Nasso (Klin. Woch. No. 7, 1932)

Prof. Nasso (Klin. Woch. No. 7, 1932) considers it possible to assume, on the basis of his own experiments and the observation of a number of other authors, that many febrile diseases of early childhood, with an unknown etiology, can be measles, scarlet fever, and rubella etc., in which the absence of a skin symptom, a rash, must be attributed to insufficient reactivity of the skin.

Randomized phase 3 ALCANZA study of brentuximab vedotin vs physician's choice in cutaneous T-cell lymphoma: final data

The primary analysis of the phase 3 ALCANZA trial showed significantly-improved objective responses lasting ≥4 months (ORR4; primary endpoint) and progression-free survival (PFS) with brentuximab vedotin vs physician's choice (methotrexate or bexarotene) in CD30-expressing mycosis fungoides (MF) or primary cutaneous anaplastic large-cell lymphoma (C-ALCL). Cutaneous T-cell lymphomas often cause pruritus and pain; brentuximab vedotin improved skin symptom burden with no negative effects on quality of life. We report final data from ALCANZA (median follow-up 45.9 months). Adults with previously treated CD30-expressing MF/C-ALCL were randomized to brentuximab vedotin (n = 64) or physician's choice (n = 64). Final data demonstrated improved responses per independent review facility with brentuximab vedotin vs physician's choice: ORR4, 54.7% vs 12.5% (P < .001); complete response, 17.2% vs 1.6% (P = .002). Median PFS with brentuximab vedotin vs physician's choice was 16.7 months vs 3.5 months (P< .001). Median time to next treatment was significantly longer with brentuximab vedotin than with physician's choice (14.2 vs 5.6 months; hazard ratio, 0.27; 95% CI, 0.17-0.42; P < .001). Of 44 patients in the brentuximab vedotin arm who experienced any-grade peripheral neuropathy (PN), (grade 3, n = 6; grade 4, n = 0), 86% (38/44) had complete resolution (26/44) or improvement to grade 1-2 (12/44). PN was ongoing in 18 patients (all grade 1-2). These final analyses confirm improved, clinically meaningful, durable responses and longer PFS with brentuximab vedotin vs physician's choice in CD30-expressing MF or C-ALCL. This trial was registered at https://www.clinicaltrials.gov/ct2/show/NCT01578499 as #NCT01578499.

Three Years Retrospective Study of Melanocytic Lesion in Tertiary Hospital: Comparing Benign & Malignant Data

Melanocytic lesion is defined as skin symptom due to proliferation of melanocytes. It may be considered benign, commonly diagnosed as melanocytic nevus (MN), or may also be malignant as malignant melanoma (MM). Publication of epidemiologic data about melanocytic lesion in Indonesia is limited. The aim of this study was to evaluate the epidemiologic findings of melanocytic lesion based on histopathology and clinical data. This descriptive study was done by collecting retrospective pigmented lesion from histopathology database at Department of Anatomical Pathology dr. Cipto Mangunkusumo National Hospital from 2014 - 2017. Retrieved data were analyzed descriptively for MN and MM include clinical diagnosis, age, sex, location, size and clinical working diagnosis. There were 121 cases of melanocytic lesions consisting of 87.6% MN. Females were more frequent for benign lesions. MN cases were mostly seen at the age below the fourth decade, while from 12 cases of MM found above fourth decade. MN was located mostly on the head and neck, while MM mostly in the lower extremities and soles. There were 75 lesions size and 4 lesions location were unknown data missing. MM is still considered rare. Completing clinical finding in the histopathology request form by surgeons will aid in defining better characteristic of melanocytic lesion in our population. Keyword: epidemiology, melanocytic lesion, nevus melanocytic, malignant melanoma. Studi Retrospektif Lesi Melanositik dalam Tiga Tahun di RS Tersier: Perbandingan Data Jinak dan Ganas Abstrak Lesi melanositik didefinisikan sebagai lesi kulit akibat proliferasi melanosit. Lesi melanositik dapat dianggap jinak, umumnya didiagnosis sebagai nevus melanositik (NM), atau merupakan ganas yaitu melanoma maligna (MM). Publikasi data epidemiologi tentang lesi melanositik di Indonesia masih terbatas. Tujuan dari penelitian ini adalah untuk melakukan evaluasi gambaran epidemiologis lesi melanositik berdasarkan data histopatologi dan klinis. Penelitian deskriptif ini dilakukan dengan mengumpulkan data retrospektif lesi berpigmen dari basis data histopatologi di Departemen Patologi Anatomi Rumah Sakit Nasional dr. Cipto Mangunkusumo dari tahun 2014 - 2017. Data dianalisis secara deskriptif untuk NM dan MM termasuk diagnosis kerja, usia, jenis kelamin, lokasi, ukuran, dan diagnosis klinis. Terdapat 121 kasus lesi melanositik yang terdiri atas 87,6% NM. Lesi jinak lebih banyak ditemukan pada wanita. Terdapat 75 dan 4 kasus dengan data ukuran lesi dan lokasi tidak tercantum. MM masih dianggap jarang. Kasus NM sebagian besar terlihat pada usia di bawah dekade keempat, sedangkan dari 12 kasus MM di atas dekade keempat. NM sebagian besar terletak di kepala dan leher, sedangkan MM ditemukan di ekstremitas bawah dan telapak kaki. Melengkapi temuan klinis dalam formulir permintaan histopatologi oleh dokter bedah akan membantu dalam menentukan karakteristik lesi melanositik lebih baik pada populasi ini. Kata kunci: epidemiologi, lesi melanositik, nevus melanositik, melanoma maligna

Relationship of acanthosis nigricans with metabolic syndrome in obese children

ObjectivesAcanthosis nigricans is a skin symptom in obesity that helps to identify patients at high risk for dyslipidemia, hypertension, insulin resistance, and diabetes. It is the most important complication of obesity in metabolic syndrome. Studies investigating the relationship between acanthosis nigricans and metabolic syndrome in obese children are insufficient. In our study, the relationship of acanthosis nigricans and metabolic syndrome was evaluated in children.MethodsObese children aged between 6 and 18 years old, who were examined in the pediatric endocrinology outpatient clinic, were included. The patients’ anthropometric measurements and laboratory results were recorded. Modified IDF (International Diabetes Federation) criteria for children were used in metabolic syndrome classification.ResultsA hundred and forty-eight obese children were evaluated. The mean age of the cases was 11.91 ± 2.94 years old. Of the cases, 56.1% were female (n=83) 43.9% (n=65) were male. In 39.9% (n=59) of cases, acanthosis nigricans was determined. Acanthosis nigricans was mostly located in the axillary area (27.1%) and the neck (16.9%). In 55.9% of the cases, it was located in more than one area. The relation of regionally detected acanthosis nigricans and metabolic syndrome was not significant (p=0.291). Metabolic syndrome was detected in 14% of 136 patients according to IDF criteria. Acanthosis nigricans and metabolic syndrome combination was present in 27.7%; however, 6.7% of the metabolic syndrome patients did not have acanthosis nigricans. There was a strong relation between metabolic syndrome and the presence of acanthosis nigricans (p=0.003).ConclusionsIn our study, a correlation between acanthosis nigricans and metabolic syndrome was detected. Acanthosis nigricans is a skin sign that can be easily detected by clinician. It is an important and easy-to-detect dermatosis that helps determine patients at risk of metabolic syndrome in obese children.

Allantoin induces pruritus by activating MrgprD in chronic kidney disease

Chronic kidney disease is a disease with decreased, irreversible renal function. Pruritus is the most common skin symptom in patients with chronic kidney disease, especially in end-stage renal disease (AKA chronic kidney disease-associated pruritus [CKD-aP]); however, the underlying molecular and neural mechanism of the CKD-aP in patients remains obscure. Our data show that the level of allantoin increases in the serum of CKD-aP and CKD model mice. Allantoin could induce scratching behavior in mice and active DRG neurons; the calcium influx and the action potential were significantly reduced in DRG neurons of MrgprD KO or TRPV1 KO mice. U73122, an antagonist of PLC, could also block calcium influx in DRG neurons induced by allantoin. Thus, our results concluded that allantoin plays an important role in CKD-aP, mediated by MrgprD and TrpV1, in CKD patients.

Demodex spp. (Acari: Demodicidae) infection in healthy young adults in Poland – occurrence and risk factors

Introduction: Demodex mites are associated with various symptoms pertaining to facial and eyelid skin in humans. Demodicosis is often reported in elderly people, above 50 years of age. In young adults, the Demodex spp. infection is less common and is asymptomatic or the symptoms are mild. Aim: The aim was to evaluate the occurrence of Demodex spp. in healthy young adults in Poland and analyze the associations between the presence of mites, participants gender and skin type, with regards to hygienic practices and symptoms. Material and methods: The content of facial sebaceous glands and eyelash follicles was examined in a group of 94 people, aged 18–32 years. Metric data and information about face and eyelid skin symptom occurrence, skin type and hygienic practices were collected by diagnostic survey. Results and discussion: Demodex spp. was identified in 21.3% of subjects. In 45% and 30% of carriers Demodex folliculorum and Demodex brevis occurred, respectively. A mixed infestation in 25% of subjects was detected. Demodex infestation was slightly higher in people with oily and mixed skin (23.5%), compared to those with dry and normal skin (18.6%). No significant influence of shared hygienic accessories on the level of Demodex spp. infection were observed. The skin symptoms of the face and eyelids were reported at 75% for subjects positive for Demodex. Conclusions: Demodex mites are often prevalent in healthy young adults in Poland in both the sebaceous glands of the face and hair follicles. While diagnosing face and eyelid skin diseases in young people, Demodex spp. infection should be considered.

Experimental models of dermatological diseases

This review presents analysis of experimental models of atopic dermatitis, psoriasis, skin symptoms of autoimmune systemic connective tissue diseases, and blistering skin diseases. Presented in the review are experimental models of atopic dermatitis which reproduce various stages and types of disease that allows the investigation of disease pathogenesis. Atopic dermatitis can develop spontaneously in Nc/Nga mice. There are atopic dermatitis models initiated by monoclonal IgE injection or epicutant sensitization under dermal barrier disfunction imitation. Genetically modified atopic dermatitis models - transgenic and knockout mice – are convenient for investigation of disease stages, cytokines, antigen-presenting cells and T-cells influence. We show that the psoriasis models created by genetic engineering methods are the most convenient for investigation of the role of particular cell types and specific factors in the disease development. Up-regulation of adhesion molecules, cytokines, transcription factors, inflammation mediators in both keratinocytes and immune cells of transgenic mice reveals their influence on psoriasis pathogenesis. There are descriptions of skin symptom models of autoimmune systemic connective tissue diseases and blistering skin disease models with and without genetic modifications. Each model demonstrates some peculiarities of pathogenesis and disease symptoms, whereas combined use of the models will allow to study the mechanisms of development of atopic dermatitis, psoriasis, blistering skin diseases and skin lesions under autoimmune systemic connective tissue diseases, that will contribute to the development of modern effective methods of treatment.

Blueberry muffin baby syndrome – praca poglądowa

Blueberry muffin baby syndrome is a nonspecific skin symptom characterized by the presence of blue-red, purple, small skin lesions with a coherent consistency, size from 1-5 mm, which have historically been described for the first time in newborns with congenital rubella virus infection. Originally, these changes were associated with the presence of persistent fetal dermal erythropoiesis during the fetal period. We now know that the described changes may be related not only to infectious disorders, but also to other disease entities ranging from hematological, vascular and metabolic disorders to neoplastic diseases including neonatal period. The image of skin lesions depending on the underlying disease may be significantly different. To our knowledge, at present there is no generally accepted algorithm for diagnostic and therapeutic procedures in patients with blueberry muffin skin changes. Knowledge about the most common causes underlying the symptom of bluberry muffin baby syndrome is the key to conducting an efficient diagnostic process, establishing a diagnosis and implementing a quick and effective treatment appropriate for the underlying blueberry muffin disease.