Approach to Mycosis Fungoides in children: Consensus-based recommendations.

BACKGROUND: Pediatric Mycosis fungoides (MF) management extrapolates from adult guidelines, despite differing clinical aspects. Recommendations are essential to address unique challenges in this distinct patient group. OBJECTIVE: This project aims to derive consensus recommendations for pediatric MF management. METHODS: Experts from pediatric dermatology, general dermatology, dermatopathology, and pediatric hematology-oncology (N = 83) were invited to contribute to consensus recommendations. The process involved 3 electronic Delphi rounds, concluding with a final consensus meeting using a modified Nominal Group Technique for unresolved items. RESULTS: Consensus included more clinical severity measures than tumor-node-metastasis-blood staging: pruritus, functional or esthetic impairment (eg, palms, soles, genitalia), quality of life impact, and psychological aspects (eg, embarrassment, anxiety, depression), plus parental anxiety. Ten recommendations were made for managing early and advanced pediatric MF. Disagreement emerged in choosing therapies beyond stage I of the disease. DISCUSSION: This multinational initiative aimed to standardize optimal pediatric MF management and successfully generated consensus recommendations. Additional work is needed for structured, prospective protocols in advanced-stage pediatric MF. LIMITATIONS: Lack of pediatric hematologists-oncologists and patients' representatives. CONCLUSION: Documentation of extended clinical severity and outcome measures is recommended. Addressing the need for structured protocols in advanced-stage pediatric MF and implementing systematic, prospective data collection is crucial.

Intravascular Lobular Capillary Hemangioma in the Corpus Spongiosum.

Lobular capillary hemangioma (LCH) or pyogenic granuloma is a very common benign vascular tumor. However, its etiology still remains unknown. This tumor classically arises from epithelium-lined tissue, such as skin and mucosa, but subcutaneous and intravenous variants have also been described. Intravenous LCH usually arises within the lumen of large caliber veins, but other intravascular examples have been reported in association with vascular malformations, namely port-wine stains and arteriovenous fistulas. A 54-year-old man presented after the sudden appearance of a subcutaneous nodule on his penile coronal sulcus. A partial biopsy disclosed a lesion with typical features of LCH but with the particularity of being located within the dilated vascular spaces of the corpus spongiosum. After the biopsy, the lesion completely regressed. Although no causal factor could be elicited, trauma was a possible trigger considering the site. Additionally, we speculate that perhaps the normal anatomy of the corpus spongiosum may mimic a similar "slow blood flow environment" found in some vascular malformations, thus providing a possible explanation for the unusual location of the lesion in our patient. This case represents, to our knowledge, the first intravascular LCH described in the corpus spongiosum. Awareness of this unusual intravascular variant of LCH is of paramount importance to avoid misdiagnosis and unnecessary treatment, because the lesion may resolve even if incompletely excised, like in this case.

Histopathology of the Exanthema in DRESS Is Not Specific but May Indicate Severity of Systemic Involvement.

OBJECTIVE: Exanthema in drug reaction with eosinophilia and systemic symptoms (DRESS) has no specific clinical diagnostic hallmark and there are few histopathologic studies. The aim of this study was to describe dermal-epidermal histopathologic features in DRESS and correlate them with the culprit drug, viral reactivation, or systemic organ involvement. METHODS: Skin biopsies were independently evaluated by 2 dermatopathologists who characterized the main histological patterns and scored dermal and epidermal changes, which were further correlated with clinical and laboratorial data. RESULTS: In 15 DRESS patients (9 male/6 female patients, mean age 53.3 years), the main observation was lymphocyte exocytosis (1.87 ± 1.25), spongiosis (0.93 ± 0.94), scattered keratinocyte necrosis (1.70 ± 1.44), basal cell vacuolization (2.13 ± 1.42), lymphocyte infiltration around dermal vessels (2.93 ± 0.92) or at the dermal-epidermal junction (2.07 ± 1.12), often with eosinophils and extravasated erythrocytes, swollen endothelial cells, and intravascular neutrophils but no vasculitis. Histopathologic patterns were classified mainly as spongiotic (5), erythema multiforme-like (3), or lichenoid (2). There was a significant positive correlation between the intensity of lymphocyte infiltration and the severity of hepatic cytolysis (r = 0.51; P < 0.05) and eosinophilia (r = 0.51; P < 0.05). No correlation was observed between the intensity and type of dermal inflammation and the degree of epidermal damage or the culprit drug. Human herpes virus type 6-positive patients had a pseudolymphomatous reaction or a perifollicular localization of the infiltrate. CONCLUSIONS: Histopathology in DRESS is variable with no specific diagnostic aspect, but there is a possible correlation between the intensity of the lymphocyte infiltrate and DRESS severity, namely, liver cytolysis.

Superficial Epidermolytic Ichthyosis-Hypertrichosis as a Clue to Diagnosis.

Superficial epidermolytic ichthyosis (SEI) is an autosomal dominant disorder caused by a mutation in the keratin 2 gene and clinically characterized by mild hyperkeratosis, superficial blisters and shedding, referred to as the moulting phenomenon. We report a case of SEI in an 18-month-old girl presenting with marked hypertrichosis. Although not invariably present, we believe that hypertrichosis can be an important clue for diagnosis.

Vancomycin-induced linear IgA bullous dermatosis: associations.

UNLABELLED: Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease. LABD is considered mostly idiopathic, butsome cases have been reported to be drug-induced, mainly associated with vancomycin (VCM).We present two cases of LABD possibly associated with VCM used for cardiac surgery prophylaxis; in the presented cases, the eruptions occurred only after VCM withdrawal, therefore leaving a question about the relationship between VCM and LABD in these cases.We reviewed previous reports of VCM-induced LABD and analyzed the following parameters: gender, age, recent medical history, concurrent medication, latency period, progression after withdrawal, time to resolution, treatment, and rechallenge. RESULTS: The causal relationship between VCM and LABD was often unclear; patients frequently had concurrent medication and symptoms frequently began and/or progressed after VCM withdrawal. Among the 46 reviewed patients in addition to our two cases (n=48), 20 (42%) had recent history of cardiac procedure, cardiac infection, congestive heart failure, or aortic aneurism. CONCLUSION: Further investigation is needed to ascertain the association between LABD, VCM, and heart disease.

Digital work platform: Understanding platforms, workers, clients in a service relation.

The rapid growth of digital economic activity had led to considerable scholarly interest in the phenomenon of platforms. Evidence shows how digital work platforms constitute one of the most relevant changes that have occurred in recent years and assume the condition of actors with an important presence in national and global work markets. However, these changes cannot be understood by focusing only on the work sphere, as the sphere of consumption is also central to this debate. In fact, the new ways of organizing, dividing and coordinating work on digital platforms are interconnected with specific modalities of consumption of the services made available by them. This article argues that a service relation approach allows an understanding of what is happening on digital work platforms, both in terms of the structural and conjunctural configurations of the interrelationships between platforms, workers and clients, as well as their social and economic consequences. This approach allows the analysis of the web of interdependencies between distinctive types of platforms, workers and clients, and to discuss how changes longitudinally within it are conditioned by the very transformations inherent to the platforms market. Thus, future research needs to explore the network of the voices of platforms, workers and clients in order to produce a robust analysis of these triangular relations as well as of the challenges regarding the differences and interconnections between algorithmic and human management.

Preparation of Binary and Ternary Deep Eutectic Systems.

The preparation of deep eutectic systems (DES) is a priori a simple procedure. By definition, two or more components are mixed together at a given molar ratio to form a DES. However, from our experience in the laboratory, there is a need to standardize the procedure to prepare, characterize and report the methodologies followed by different researchers, so that the results published can be reproduced. In this work, we test different approaches reported in the literature to prepare eutectic systems and evaluated the importance of water in the successful preparation of liquid systems at room temperature. These published eutectic systems were composed of citric acid, glucose, sucrose, malic acid, ß-alanine, L-tartaric acid and betaine and not all of preparation methods described could be reproduced. However, in some cases, it was possible to reproduce the systems described, with the inclusion of water as a third component of the eutectic mixture.

Extrapulmonary Tuberculosis Presenting as a Suspected Case of Metastatic Breast Cancer.

Extrapulmonary tuberculosis (TB) represents a diagnostic challenge. Bone TB is an uncommon and important presentation of extrapulmonary TB, which can lead to bone destruction, deformity and even paraplegia. Breast TB is rare and often confused with neoplasia, since the clinical and imaging presentations are not specific. Any of these extrapulmonary TB presentations, in the absence of cultural isolation of mycobacteria, oblige the exclusion of other diseases (secondary or infectious diseases). The authors report a case of multifocal extrapulmonary TB, as an example of the problem with the differential diagnosis of the disease. LEARNING POINTS: Extrapulmonary TB remains a diagnostic challenge.Consider breast TB as a differential diagnosis of mammary nodules.A high level of suspicion is necessary to make an early diagnosis and start appropriate treatment before irreversible damage takes place.

Estresse, depressão e ansiedade em mães de autistas: Revisão nacional / Stress, depression and anxiety in mothers of autism: National review

A proposta deste estudo foi identificar, na literatura brasileira, a presença e a intensidade de estresse, depressão e ansiedade em mães de indivíduos com Transtorno do Espectro Autista (TEA). Trata-se de uma pesquisa de revisão sistemática, desenvolvida a partir de produções publicadas nas bases de dados bibliográficas BVS (Biblioteca Virtual em Saúde), Portal de Periódicos CAPES e Pepsic (Periódicos Eletrônicos de Psicologia), sem restrição de tempo. Do procedimento de busca, resultaram nove artigos para o banco final de análise. Os resultados evidenciam que as mães são afetadas significativamente pelo estresse, sendo constatada a presença do constructo na maioria do público-alvo, com predominância total de sintomas psicológicos e a prevalência de fases alarmantes de estresse. Em relação à depressão e ansiedade, os estudos demonstraram divergências quanto aos resultados. Tendo em vista os inúmeros aspectos que influenciam no desenvolvimento de determinados constructos, assim como nos resultados das pesquisas, embora a presença de depressão e ansiedade, além de altos níveis de estresse, tenham sido constatados nas pesquisas nacionais, não é possível afirmar que o transtorno seja a principal causa. A atual pesquisa permitiu demonstrar, ainda, a escassez de estudos brasileiros de determinado cunho. Assim, evidenciou a necessidade de investigações nacionais que se dediquem a esse público, de forma a embasar, fidedignamente, intervenções que visem a promoção de saúde mental e maior qualidade de vida.

The proposal of this study it was to identify, in the Brazilian literature, the presence and intensity of stress, depression and anxiety in mothers of individuals with Autism Spectrum Disorder (ASD). It is about a research of systematic review, developed on productions published in the bibliographic databases BVS (Biblioteca Virtual em Saúde), Portal de Periódicos CAPES and Pepsic (Periódicos Eletrônicos em Psicologia), without time restriction. The search procedure resulted in nine articles for the final analysis database. The results demonstrate that mothers are significantly affected by stress, with the presence of the construct in most of the target audience, with a total predominance of psychological symptoms and the prevalence of alarming phases of stress. In relation to depression and anxiety, the studies demonstrated divergences regarding the results. Considering the numerous aspects that influence the development of certain constructs, as well as the results of research, although the presence of depression and anxiety, in addition to high levels of stress, have been found in national research, it is not possible to say that the disorder be the main cause. The present research also made it possible to demonstrate the scarcity of Brazilian studies of a certain nature. Thus, it highlighted the need for national investigations dedicated to this public, in order to reliably support interventions aimed at promoting mental health and better quality of life.

UVA1 for diffuse cutaneous systemic sclerosis in a Fitzpatrick skin type VI patient: outcomes in the modified Rodnan skin score.

INTRODUCTION: Cutaneous sclerosis can lead to important mobility impairment. Ultraviolet (UV) A1 phototherapy may improve skin sclerosis, although most of the studies have been with Caucasian patients. MATERIAL AND METHODS: A 44-year-old patient, Fitzpatrick skin type VI, was being followed up with the diagnosis of diffuse cutaneous systemic sclerosis. He had significant mobility impairment, especially of the right hand and arm. In 2015 he started UVA1 phototherapy daily, Monday until Friday (Waldmann® 7001 UVA cabin equipped with 40 Philips TL/10R lamps - spectral irradiation between 340 and 400 nm). The initial dose was 10 J/cm2, rapidly increased up to a steady dose of 35 J/cm2. RESULTS: After 40 sessions of UVA1, active fingers flexion and abduction of the right arm significantly improved and the modified Rodnan skin score changed from 26 to 11. CONCLUSION: The modified Rodnan skin score is a practical and useful tool during the follow-up of patients with systemic sclerosis. UVA1 phototherapy improves cutaneous sclerosis, and the related mobility impairment, and a dose of 35 J/cm2 is effective, even in higher phototypes, having a good safety profile.

[Anogenital Warts in a Major Venereology Clinic: Centro de Saúde da Lapa - Lisbon, 2008 to 2014]. / Condilomas Anogenitais numa Consulta de Doenças Sexualmente Transmissíveis: Centro de Saúde da Lapa - Lisboa, 2008 a 2014.

INTRODUCTION: Human papillomavirus infection is the most common sexual transmitted infection in the world, being associated with different diseases, namely anogenital warts, recurrent respiratory papillomatosis and anal, cervical, and oropharyngeal cancers. Among sexually active people, approximately 1% has anogenital warts, 90% of cases resulting from genotypes 6 and 11. MATERIAL AND METHODS: Patients diagnosed with first episode of anogenital warts from 2008 to 2014 in Lisbon's major venereology clinic were identified, and characterized according to sex, sexual orientation, age, warts location, and number of sexual partners. RESULTS: We observed a statistically significant decrease in first anogenital warts diagnosis among < 19 year old females (r = -0.848;p = 0.016) and a non-statistically significant decrease among < 19 ' year-old males and among 20 ' 24 year old females. DISCUSSION: In October 2008, the quadrivalent vaccine (genotypes 6, 11, 16, 18) was introduced in the Portuguese national vaccination program, targeting 13-year-old females, with a catch-up for 17 year old females. In the women'´s group, decrease in first anogenital warts diagnosis is probably related to human papillomavirus vaccination before onset of sexual activity. CONCLUSION: This study reinforces the importance of national human papillomavirus vaccination program.

Introdução: O vírus do papiloma humano é responsável pela infeção sexualmente transmissível mais comum, podendo manifestar--se por um conjunto amplo de doenças, nomeadamente condilomas anogenitais, papilomatose laríngea recorrente, e neoplasias da região anogenital, colo do útero e orofaringe. Estima-se que os condilomas anogenitais afetem 1% da população sexualmente ativa, causados em cerca de 90% pelos genótipos 6 e 11. Material e Métodos: Identificámos os doentes com primeiro diagnóstico de condilomas anogenitais da consulta de doenças sexualmente transmissíveis do Centro de Saúde da Lapa, entre janeiro de 2008 e dezembro de 2014, e caracterizámos os doentes por sexo, orientação sexual, idade, localização das lesões, e número de parceiros nos últimos seis meses. Resultados: Foram identificados 902 indivíduos com primeiro diagnóstico de condilomas anogenitais. Observámos uma diminuição significativa de novos casos nas mulheres com < 19 anos (r = -0,848; p = 0,016), e uma diminuição sem significado estatístico nos homens com < 19 anos e nas mulheres entre os 20 - 24 anos. Discussão: Em outubro de 2008, a vacina quadrivalente (genótipos 6, 11, 16, 18) foi introduzida no plano nacional de vacinação de Portugal, abrangendo as adolescentes com 13 anos, com um catch-up para as de 17 anos. A diminuição de primeiros diagnósticos de condilomas anogenitais nas mulheres, deve-se provavelmente à sua vacinação antes de iniciarem a vida sexual. Conclusão: Este estudo reforça a import'ncia do programa nacional de vacinação para o vírus do papiloma humano.

Histological variability and the importance of clinicopathological correlation in cutaneous Rosai-Dorfman disease.

Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown etiology. The disease mainly affects lymph node tissue, although it is rarely confined to the skin. Here, we describe a 53-year-old woman with purely cutaneous Rosai-Dorfman disease. The patient presented with a large pigmented plaque on her left leg, and sparse erythematous papules on her face and arms. A complete clinical response was achieved with thalidomide, followed by recurrence at the initial site one year later. The histological examination displayed the typical features of Rosai-Dorfman disease in the recent lesions but not in the older lesions. In the setting of no lymphadenopathy, the histopathological features of Rosai-Dorfman disease are commonly misinterpreted. Therefore, awareness of the histological aspects present at different stages, not always featuring the hallmark microscopic signs of Rosai-Dorfman disease, is particularly important for a correct diagnosis of this rare disorder.

Fixed drug eruption by etoricoxib confirmed by patch test.

Non-steroidal, anti-inflammatory drugs, followed by antibiotics, are the main causes of fixed drug eruption. They provoke one or several round erythematous or bullous lesions that recur in the same place after taking the causative medication. A positive patch test on residual, lesional skin can replace satisfactorily oral reintroduction. We describe the case of a 74-year-old woman with numerous, rounded, erythematous lesions on the trunk and recurrent blistering on the fifth right-hand finger, which developed a few hours after taking etoricoxib. Lesional patch testing with etoricoxib was positive and reproduced the typical pattern of a fixed drug eruption upon histopathology. We emphasize the specific reactivity of the etoricoxib patch test, and the capacity to reproduce the histologic pattern of the reaction.

Lower lip reconstruction with nasolabial flap--going back to basics.

Squamous cell carcinoma of the lower lip is frequent, and radical excision sometimes leads to complex defects. Many lip repair techniques are aggressive requiring general anesthesia and a prolonged post-operative period. The nasolabial flap, while a common flap for the repair of other facial defects, is an under-recognized option for the reconstruction of the lower lip. We describe the use of nasolabial flap for the repair of a large defect of the lower lip in a ninety year-old male, with good functional results and acceptable cosmetic outcome. We believe the nasolabial flap is a good alternative for intermediate-to-large lower lip defects in patients with impaired general condition.