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We present a complex case of a neonate, delivered urgently for hydrops fetalis, with a large vascular mass of the extremity, diagnosed postnatally as a congenital hemangioma. The patient suffered immediate cardiac compromise and severe coagulopathy atypical for the diagnosis and subsequently died from these complications. Treatment was imperative but challenging due to a lack of a standardized treatment approach and few historical reports of equally critically ill patients. In this report, we review potential medical and surgical interventions and discuss treatment considerations in similar, life-threatening cases of congenital hemangiomas.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematologic malignancy that can present with multiorgan involvement including the skin. While this entity accounts for 0.1% of all cutaneous lymphomas in adults, fewer than 90 pediatric cases have been described in the literature. We present three cases of pediatric BPDCN, highlighting the variable cutaneous and clinical presentation.

An infant girl developed a hemangioma affecting her left iris concurrently with diffuse cutaneous infantile hemangiomas from day 2 of life. Intraocular hemangiomas are rarely reported and are usually associated with neonatal hemangiomatosis, the presence of which indicates a high risk for visceral lesions. This striking case highlights the unusual clinical presentation of iris hemangioma and demonstrates the importance of conducting visceral screening when faced with these lesions. Oral propranolol was commenced and resulted in rapid improvement of all lesions without complication.

We present a 14-year-old male with chronic, recurrent pyoderma gangrenosum that resolved after treatment of latent tuberculosis. As pyoderma gangrenosum often occurs secondary to underlying diseases, we describe latent tuberculosis as a possible trigger for pyoderma gangrenosum.

Infantile hemangiomas are the most common benign vascular tumors in childhood. Propranolol is the first-line treatment for infantile hemangiomas, but failures may occur. Sirolimus, an mTOR inhibitor, is a promising drug for the treatment of vascular malformations and vascular tumors. We present the case of a child with multiple infantile hemangiomasthat was successfully treated with sirolimus and propranolol after failure of combined propranolol and prednisolone treatment.

Common polygenic skin disorders, such as atopic dermatitis, may rarely present in a segmental or linear distribution due to cutaneous mosaicism. Only seven cases of superimposed linear atopic dermatitis have been reported to date. Here, we present a child with severe superimposed linear atopic dermatitis and highlight the first successful use of dupilumab in its treatment.

Bluish nodular mammary lesions in prepubertal girls are a challenging diagnosis. Retroareolar cysts are rare in this population, but relatively common among adolescent women. This diagnosis can be suspected clinically and ultimately confirmed by cutaneous ultrasonography, avoiding unnecessary biopsies or complex radiologic studies.

Hidradenitis suppurativa (HS) is a chronic inflammatory disease that disproportionately affects women of childbearing age. Pregnancy influences HS severity for many women, thus diligent continued management throughout pregnancy and postpartum may be required. Herein, we provide an updated review of pregnancy and lactation safety data for HS medications, including topical antiseptic washes, topical and systemic antibiotics, biologic and nonbiologic immunomodulators, immunosuppressants, adjunct medical therapies, and pain medications, to help guide risk-benefit discussions between providers and HS patients.

Mycobacterium caprae, a member of the Mycobacterium tuberculosis complex, causes tuberculosis in humans and animals, but the incidence in humans is very low worldwide. Direct contact with animals and with unpasteurized dairy products is the most frequent source of contagion. Cutaneous tuberculosis supposes a diagnostic challenge due to its variety of clinical manifestations and the difficulty in demonstrating resistant acid-alcohol bacilli in histological samples. In cases of high suspicion, the initiation of antituberculosis treatment should be considered empirically. Combined therapeutic regimens are necessary to prevent long-term resistance and complications, although the duration of treatment has not been fully established.

Aquagenic pruritus is a rare debilitating condition, which can be idiopathic, iatrogenic, or associated with systemic disease. In idiopathic cases, treatment can be challenging as options are limited and of variable efficacy. Here, we report the case of a teenage boy with refractory idiopathic aquagenic pruritus effectively managed with administration of β-alanine supplementation, a treatment gaining traction in lay media but not yet reported in the medical literature. This report adds to the limited options published for treatment of idiopathic aquagenic pruritus in pediatric patients.

Acne and rosacea are two well-known chronic skin diseases in dermatology. There are many known therapeutic options of both diseases, but new treatment agents and therapeutic advances come to the agenda day by day. We would like to summarize new treatment advances for acne and rosacea diseases.

Background : Systemic JIA (sJIA) is the most severe sub type of JIA with limited treatment option and responsible for most of the disability and morbidity in JIA. Objective: To assess the efficacy of Tocilizumab (TCZ) in refractory sJIA patients according to disease activity score in 28 joint criteria. Methodology: This interventional study was carried in the department of Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU). A total number of 22 sJIA patients who were refractory to traditional DMARDs were offered TCZ. Among them 15 patients agreed to take TCZ (cases) and 7 patients did not (controls). All the cases and controls were assessed according to DAS 28 criteria at enrollment, 12 weeks and 24 weeks of TCZ therapy. DAS 28 scores during assessment were compared. Results: After TCZ therapy systemic features subsided and laboratory features improved in 100% cases. Among cases 93.3% and 92.3% had moderate response at 12 and 24 weeks respectively according to DAS 28 criteria. Among controls 71.5% and 85.7% had moderate response at 12 and 24 weeks respectively (p0.001). Among cases 40% and 60% patients achieved remission at 12 and 24 weeks, where as among controls none achieved remission. Dose of steroid was significantly reduced among cases but was increased significantly among controls. Few side effects like pharyngitis, Varicella, septicemia, anaphylactoid reaction and raised ALT were observed. Conclusion: Tocilizumab therapy was effective according to DAS 28 criteria refractory sJIA. Bangladesh J Child Health 2015; VOL 39 (3) :129-134

Patienten mit systemischem Lupus erythematodes (SLE) unterscheiden sich heute im Wunsch nach Absetzen der immunsuppressiven und immunmodulatorischen Therapie nicht von Patienten mit anderen rheumatischen Erkrankungen. Um sie gut zu beraten, mussen einerseits die moglichen Risiken einer Dauertherapie mit dem jeweiligen Medikament und andererseits das angenommene Risiko besonders von schweren Schuben betrachtet werden. Die Risiken einer Langzeittherapie sind fur einzelne Substanzen sehr unterschiedlich. Sie reichen von hohen, inakzeptablen Risiken bei Cyclophosphamid und hoher dosierten Glukokortikoiden bis zu niedrigem, normalerweise durch Langzeitvorteile ausgeglichenem Risiko bei Hydroxychloroquin. Das individuelle Risiko eines SLE-Schubs ist oft schwer abzuschatzen, aber klinische Remission und zumindest 3 Jahre Immunsuppression werden fur die Lupusnephritis empfohlen. Die Dauer der Remission kann vielleicht auch kurzer sein, wenn eine mildere Erkrankung vorliegt. Diese Ubersichtsarbeit versucht, die vorhandene Evidenz in die klinische Perspektive zu setzen und daraus konkrete Empfehlungen abzuleiten.

Disseminated congenital pyogenic granuloma (DCPG) is an uncommon condition. Individual lesions of DCPG share clinical and histologic similarities with infantile hemangioma (IH); endothelial glucose transporter 1 (GLUT-1), which is highly expressed in IH but generally not in pyogenic granulomas (PG), is an important diagnostic tool. Treatment for DCPG remains difficult. We describe a case of DCPG effectively treated with propranolol.

Epidermolysis bullosa is a group of rare genetic disorders with multiple organ system involvement. In one severe form, recessive dystrophic epidermolysis bullosa, chronic anemia is common. This report outlines the multifactorial nature of anemia in recessive dystrophic epidermolysis bullosa and presents a practical clinical algorithm based on expert consensus for the diagnosis and treatment of anemia in recessive dystrophic epidermolysis bullosa.

Screening electrocardiography (ECG) before initiation of propranolol for treatment of infantile hemangiomas (IH) is controversial. A retrospective chart review was conducted to assess the utility of pretreatment ECG in infants with IH starting propranolol. Although nearly half of the ECGs were abnormal, no contraindications to treatment were identified from screening ECG, and no association was found between any of the reported side effects and abnormal ECG. These results support previously published data, and in a larger cohort, providing further confirmation that pretreatment ECG is not necessary in most infants with IH.

Pyoderma gangrenosum is a neutrophilic dermatosis that is rare in infancy, with only 20 cases reported in the literature. We present a case of infantile pyoderma gangrenosum refractory to topical steroids, tacrolimus, and dapsone as well as systemic steroids and infliximab that is currently well controlled with the addition of oral tacrolimus. To our knowledge, this is the first report of the effective, safe use of oral tacrolimus in combination with infliximab for infantile pyoderma gangrenosum. We review all current cases of infantile pyoderma gangrenosum, as well as tacrolimus and its role in the treatment of this condition.

Prurigo pigmentosa (PP) is a rare inflammatory skin disease. Neutrophil-mediated inflammation is considered to be responsible for the etiopathogenesis of PP. We consider that colchicine may be an effective agent in the treatment of PP since it exerts an antiinflammatory effect by inhibiting neutrophil chemotaxis. Further studies are required to verify whether colchicine is an effective treatment option for PP.

Autosomal recessive congenital ichthyosis is a genetically and phenotypically heterogenous group of scaling skin disorders. We describe a patient with ARCI caused by homozygous variants in NIPAL4, in whom the dermatologic phenotype and an associated arthropathy, markedly improved with ustekinumab.

Livedoid vasculopathy (LV) is a thrombo occlusive disorder presenting with recurrent painful ulcers of lower extremities. Association of LV with increased level of lipoprotein (a) (LP(a)), a risk factor for cardiovascular disease, has been reported. Danazol has been used with success in the management of LV, but none of the previous studies looked at the correlation between response to the treatment and level of LP(a). The aim of this study was to demonstrate the efficacy of low-dose danazol in the treatment of LV and its effects on LP(a). We present four cases with LV who were successfully treated with low-dose danazol, assessing the clinical characteristics and laboratory tests including the level of LP(a). The average age of the patients was 45 years and the mean duration of the disease was 19 years. The treatment regime of danazol 200 mg daily led to complete healing of ulcers and reduction in pain and a 70% (ranging from 52 to 87%) reduction in the level of LP(a). The limitation of this study is "small sample size." In our patients with LV, low-dose danazol led to clinical improvement along with significant reduction in the level of LP(a).

Eosinophilic annular erythema is a rare, benign, recurrent condition characterized by annular skin lesions, tissue eosinophilia, and resistance to a variety of treatments. There are fewer than 30 cases reported in the English literature, 7 of which are in children. We present a case of recurrent eosinophilic annular erythema in an adolescent that was successfully treated with dupilumab, an interleukin-4 receptor alpha antagonist.

A categoria docente é exposta a ambientes conflituosos, alta exigência e estresse, repercutindo na saúde física, mental e no desempenho destes profissionais. Em levantamento realizado na Secretaria Municipal de Educação de Uberaba-MG (SEMED), os problemas de saúde mental e osteomusculares são responsáveis por quase metade de todos os afastamentos. Estes fatores guardam relação intrínseca ao sistema de trabalho, stress, sobrecarga e baixa qualidade de vida. Este estudo procura compreender as principais causas de sofrimento dos professores, segundo a voz, expressão e opinião dos mesmos. Foram realizadas dez sessões de Terapia Comunitária Sistêmica e Integrativa (TCSI) em dez escolas com a participação de 296 profissionais. Olhando para a rede de atendimento e suporte à saúde do professor é possível perceber que o acolhimento ofertado aos professores da rede municipal é eficiente no que diz respeito ao tratamento do indivíduo, porém deixa a desejar em relação ao acolhimento coletivo. Nesse sentido, surge a indagação “se o sujeito adoece socialmente por que tratá-lo individualmente?”. Os resultados apontam que a TCSI pode fazer parte de projetos mais amplos voltados à compreensão dos sofrimentos coletivos, à saúde do trabalhador e para futuramente contribuir na elaboração de políticas públicas de instituições de ensino.

Background Incidence of inpatient (inpt) events, comorbidities and age of death for Irish vasculitis patients is currently unknown Objectives Establish mortalitity & comorbidities in Irish vasculitis inpts Methods The HIPE system was evaluated from 57 Irish hospitals between 2005–2014 for vasculitis inpt episodes (not individual patients) based on ICD10. Age, length of stay (LOS), gender and admitting indication and mortality were recorded. Results are shown as totals, % & median (IQR). The Mann–Whitney U test and χ2 were employed Results 5,841 vasculitis inpt records were reviewed with median annual admissions of 601 (601–622). 2,229 inpt admissions had large vessel vasculitis (LVV – Takayasu9s & GCA) whose median age was 75yrs (66–81), LOS 6 days (3–13) and 68% were female. 3612 inpt admissions had small & medium vessel vasculitis (SMVV –GPA, eGPA, MPA, PAN, Behcet9s, Goodpastures, Necrotizing & Juvenile Vasculitis) with a median age 58yrs (42–70), LOS 6 days (2–13) and 48% were female. MPA, eGPA and GPA admissions were more common for men whereas GCA, Takayasu9s and Behcets admissions were more common for women (table 1). 58% LVV inpts & 50% SMVV inpts were admitted for systemic inflammation (p Conclusions Around 600 vasculitis admissions occurred each year across the 57 hospitals directing the need for a concentrated number of centres of excellence to facilitate expert care, research and education. Small vessel vasculitis admissions were more common for men and large vessel and Behcet9s for women. Despite advances in therapy systemic autoimmune disease and infection prevailed as causes for admission and death in vasculitis inpts, with greater mortality in SMVV from disease activity and renal failure and from vascular infarction in LVV. There is a need for improved diagnostic tests. Disclosure of Interest None declared

In current cutaneous retinoid therapy systemic exposure is low and the risk of teratogenesis appears to be limited. However, new indications, altered posologies and the introduction of new synthetic retinoids demand continuous assessment of the teratogenic risk. Teratogenicity testing of new substances in animals is only of value if accompanied by detailed pharmacokinetic analysis to establish the relationships between the levels of parent compound and metabolite in both maternal plasma and fetal tissues and teratogenic events. This information should be compared to the maximum of relevant pharmacokinetic data which can be ethically obtained in man or from human tissues. The presence or absence of teratogenic effects following cutaneous administration of retinoids in animals has, as such, little direct bearing on the risk in man. Two special cases exist where teratogenic risk can be evaluated directly in man without reference to animal studies. The first concerns substances whose teratogenic potential has been established in man by other routes of administration permitting a comparison with the cutaneous route on a pharmacokinetic basis. The second concerns the cutaneous administration of endoge-nously occurring substances and their eventual disturbance of systemic retinoid equilibrium.

This paper briefly reviews the historical evolution of paradigms that have been purported to characterise the clinical behaviour of breast cancer, with the intention of guiding treatment approaches. Results from randomised clinical trials and the explosion of knowledge in the area of cancer biology have discredited the monolithic paradigms that had dominated thinking about breast cancer in the past. Contemporary notions of breast cancer biology recognise that, although some cancers disseminate well before becoming clinically detectable, acquisition of a metastatic phenotype can occur at any point (or not at all) in the local evolution of the tumour. As a consequence, both systemic and timely local–regional therapies can be expected to influence disease dissemination and patient survival. This is consistent with results observed in clinical trials, overviews of which indicate that prevention of four local recurrences will, on the average, prevent one death from breast cancer. Optimisation of local–regional treatment is an important goal in breast cancer management.

Se describe la estructura de la entrevista que nos permita conocer el mapa relacional actual y pasado del individuo, así como su contexto significativo y contrato terapéutico. The structure of the interview is described, that the option to know contribute us the present relational map and past of the individual and the significant context and therapeutic contract.