Time to codify a challenge in communication.

Infophobia, a term not being introduced in the medical literature, is one of many factors that may hamper a Patient-Health Care Provider (HCP) encounter. This phobia creates resistance to accepting medical knowledge, potentially becoming a significant barrier in medical practice, explained by patients' fear of information that may negatively impact medical assessments, therapies, and immunization. Since complications of this phobia are well beyond information, it should be recognized, and herein by presenting a dermatological case, we aim to establish this concept to identify this phenomenon.

Chronological association between alopecia areata and autoimmune thyroid diseases: A systematic review and meta-analysis.

The association between alopecia areata (AA) and autoimmune thyroid diseases (AITD) has been suggested; however, the chronological relationship between AA and AITD remains elusive. A systematic review and meta-analysis were conducted to assess the association between AA and AITD focusing on the prevalence of thyroid antibodies, thyroid diseases and serological thyroid dysfunctions, respectively. Data collection was performed in October 2018 by searching for articles in two electronic databases: Medline and Embase. Case-control, cohort and cross-sectional studies were included. Meta-analysis of studies eligible for quantitative synthesis was performed to estimate pooled odds ratios of thyroid antibodies; thyroid peroxidase antibody (TPO-Ab) and thyroglobulin antibody (TG-Ab), diagnosed thyroid diseases and serological thyroid dysfunctions. Four hundred and eighty nine research papers were identified and 17 studies with 262 581 patients and 1 302 655 control subjects were included for quantitative synthesis. AA was significantly associated with both TPO-Ab and TG-Ab. In comparison, there was no significant association between AA and diagnosed hypothyroidism or hyperthyroidism and serological hypothyroidism or hyperthyroidism. In conclusion, AA is significantly associated with the existence of thyroid antibodies rather than with clinical or laboratory thyroid abnormality. Lack of long-term follow-up data is a limitation of the existing published work. Our findings do not support routine screening of thyroid diseases for asymptomatic AA patients but highlight the potential future risk of AITD particularly in severe and refractory AA.

Drug Reaction with Eosinophilia and Systemic Symptoms (DReSS): How Far Have We Come?

Drug reaction with eosinophilia and systemic symptoms (DReSS), also known as drug-induced hypersensitivity syndrome (DiHS), is an uncommon severe adverse reaction to medications. It is important to recognize it as it is potentially fatal and can cause significant morbidity. From the first reports of drug reactions related to certain anticonvulsants characterized by fever, liver enzyme elevation, and skin changes, our continuously growing understanding of this entity has allowed us to describe its physiopathology and clinical features even further. The relationship of genetic factors, viral activation, and specific drug exposure is now known to play a role in this disease. There is still not a widely accepted marker for DReSS/DiHS, but the spectrum of clinical and laboratory features has now been better outlined. The mainstay of treatment is the use of systemic corticosteroids, but other options such as intravenous immunoglobulin, cyclosporine, mycophenolate mofetil, rituximab, and cyclophosphamide have been described. We present a comprehensive review of the literature on DReSS/DiHS, focusing on its history, etiopathogenesis, diagnosis, therapeutic approach, and outcome.

An Update on Calciphylaxis.

Calciphylaxis, also known as calcific uremic arteriolopathy and uremic small artery disease with medial wall calcification and intimal hyperplasia, is a multifactorial cutaneous vascular disease characterized by chronic, painful, non-healing wounds that occur frequently in patients with chronic kidney disease, predominantly in those with end-stage renal disease. The pathogenesis remains unclear, and the development of calciphylaxis lesions depends on medial calcification, intimal fibrosis of arterioles and thrombotic occlusion. Despite an increase in reports of calciphylaxis in the literature and clinical recognition of demographic characteristics and risk factors associated with calciphylaxis, it remains a poorly understood disease with high morbidity and mortality. In this review, we analyze and summarize the clinical manifestations, pathogenesis and pathophysiology, histopathology, differential diagnosis, diagnostic workup and treatment modalities for calciphylaxis. Because of the lack of consensus regarding the optimal approach to and treatment of this disorder, a high degree of clinical suspicion, early diagnosis, and multimodal and multidisciplinary treatment in collaboration with dermatology, nephrology, wound care, nutrition and pain management specialties may improve survival in patients with calciphylaxis.

Case for diagnosis. A bluish nodule on the scalp.

Acrospiroma, also known as hidradenoma, is a rare cutaneous tumor that has several histological characteristics. As a consequence, a high index of suspicion is necessary for its diagnosis. Here we report a case that illustrates the importance of a good clinical-pathologic correlation in order to recognize this disease.

Case for diagnosis. A bluish nodule on the scalp

Abstract: Acrospiroma, also known as hidradenoma, is a rare cutaneous tumor that has several histological characteristics. As a consequence, a high index of suspicion is necessary for its diagnosis. Here we report a case that illustrates the importance of a good clinical-pathologic correlation in order to recognize this disease.

Cold subcutaneous abscesses as the first manifestation of disseminated coccidioidomycosis in an immunocompromised host.

Coccidioidomycosis is an endemic fungal infection in the southwestern USA and northern Mexico. It is caused by Coccidioides immitis and C. posadasii. This infection occurs due to the inhalation of airborne arthroconidia, causing a mild pulmonary infection, but most cases are asymptomatic. Disseminated coccidioidomycosis (DC) is a rare entity occurring in less than 1% of all cases, usually in immunocompromised patients, and it carries high risks of morbidity and mortality. The skin is one of the most frequently affected organs and in some cases cutaneous lesions may be the first or only sign of infection. A wide spectrum of clinical lesions may develop, including cold abscess. In immunocompromised hosts, DC represents a diagnostic and therapeutic challenge. Treatment is based on antifungal drugs, such as amphotericin B and azoles, administered for long periods of time and under close follow up to monitor the treatment response and to detect relapse. In the following case report, we present a 35-year-old male patient with systemic lupus erythematosus under immunosuppressive therapy who presented with cold subcutaneous abscesses as the first sign of DC.

[News in severe clinical adverse drug reactions: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)]. / Actualidades en farmacodermias severas: síndrome de Stevens-Johnson (SSJ) y necrólisis epidérmica tóxica (NET).

Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening conditions associated with significant morbidity and mortality. They are considered to be part of a spectrum of cutaneous drug reactions, differing only by their extent of skin detachment due to keratinocyte apoptosis. Drugs are assumed as the main cause of Stevens-Johnson syndrome and toxic epidermal necrolysis in most cases. The pathophysiology is incompletely understood; however, current pathogenic models involve Fas ligand, granulysin, and cytokines. Diagnosis relies mainly on clinical signs together with the histological analysis, and treatment requires early cessation of the causative drug and supportive care. Of these conditions, herein we will review the advances in clinical, pathogenesis, and management.

Disseminated brown macules in an infant.

Urticaria pigmentosa is the most common form of mastocytosis. Mastocytosis usually presents at birth or early childhood, and may involve only the skin or, less commonly, other internal organs. Diagnosis is clinical, but a skin biopsy may be useful. Prognosis is usually good, and treatment focuses on the avoidance of certain triggers and administration of topical and systemic medications. Appropriate counselling of parents regarding the benign nature of this disease is important as most cases resolve by adolescence.

Higher doses of CD34+ progenitors are associated with improved overall survival without increasing GVHD in reduced intensity conditioning allogeneic transplant recipients with clinically advanced disease.

OBJECTIVE: The influence of CD34+ cell dose on the outcome of allogeneic peripheral blood stem cell (PBSC) transplantation after reduced intensity conditioning (RIC) remains controversial. The impact of the number of CD34+ hematoprogenitors infused on transplant outcome and on the incidence of graft versus host disease (GVHD) was analyzed. MATERIALS AND METHODS: Data of 138 patients with advanced hematological diseases who received an allogeneic PBSC transplant after RIC were analyzed. Donors were mobilized with granulocyte colony-stimulating factor and underwent one to three apheresis procedures. Incidence of acute and chronic GVHD and overall and event-free survival (OS and EFS) was determined. RESULTS: The median number of CD34+ cells infused was 5.57 × 10(6) kg(-1) (range: 1.1-15.6). There was no relationship between CD34+ cell dose and neutrophil or platelet engraftment. Patients receiving ≥5 × 10(6) kg(-1) CD34+ cells had a 63.1% 5-year OS when compared with 48.2% for those receiving a lower number (P = 0.024). At 5-year follow-up, there was no significant difference in EFS between the groups (44% vs. 42.8%, P = 0.426). No relationship between CD34+ cell dose and acute GVHD was found (P = 0.1). Relapse rate was the same in patients with and without acute GVHD (P = 0.117). A nonsignificant improvement on OS and EFS in patients who developed chronic GVHD was found (P = 0.57 and 0.41). CONCLUSION: A CD34+ cell dose ≥5 × 10(6) kg(-1) was associated with a significantly higher OS, but no improved EFS in high-risk patients. The number of CD34+ progenitors infused had no influence on the incidence of acute or chronic GVHD.

Long-term results of placental blood allografting using reduced-intensity conditioning: multicenter experience in a developing country.

BACKGROUND: Placental blood (PLB) hematopoietic stem cell transplantation has recently been explored in an increasing number of patients; the best conditioning regimen has not been established. MATERIAL AND METHODS: In an eight-year period, 66 consecutive patients, both children and adults (40 males and 26 females), were grafted with allogeneic placental blood cells using a reduced-intensity conditioning regimen: 23 patients were grafted because of a non-malignant condition and 43 patients for a malignant disease. The median age was 7 years (range 5 months to 72 years). RESULTS: Median time to recover >0.5×10(9)/l granulocytes was 19 days, whereas median time to recover >20×10(9)/l platelets was 23 days. Thirty-eight individuals failed to engraft and they either recovered endogenous hematopoiesis or died. Patients have been followed for periods ranging from 0.5 to 66 months, median 9 months. The median overall post-transplant survival (OS) was 22 months and the 36-month OS was 32%; it was significantly better for individuals grafted with 6/6 matched cords (45%). The cumulative incidences of grade II-IV acute graft-versus-host disease (GVHD) and grade III-IV acute GVHD for the patients who engrafted were 33 and 10%, respectively. DISCUSSION: The low engraftment rate should be improved by selecting better cord blood units; additional studies are needed to define if non-myeloablative conditioning is preferable over conventional conditioning.