Xanthoma striatum palmare and biliary tract atresia: An unusual association.

Cutaneous xanthomas develop as a result of lipid deposition in the dermis and may be a manifestation of various systemic diseases. The morphology and anatomic location of xanthomas are often a clue to the underlying cause. Xanthoma striatum palmare (XSP) is classically associated with dysbetalipoproteinemia and rarely observed in hepatic disorders. We present a case of a 2-year-old child diagnosed with XSP and biliary tract atresia.

Epidemiology and Predisposing Factors for Erythema Toxicum Neonatorum and Transient Neonatal Pustular: A Multicenter Study.

BACKGROUND/OBJECTIVES: Erythema toxicum neonatorum (ETN) and transient neonatal pustular melanosis (TNPM) are benign pustular skin conditions that are relatively common in newborns, but the predisposing factors for these conditions are unknown. Our goal was to verify the prevalence of ETN and TNPM and their predisposing factors in a large sample of neonates. METHODS: In this prospective 1-year multicenter study, 2,831 neonates (age ≤72 hours) born in southern Brazil were randomized to undergo skin examination by a dermatologist. RESULTS: Most were Caucasian (of European descent). There were 602 cases of ETN (21.3% prevalence) and 97 cases of TNPM (3.4% prevalence). ETN was more prevalent in Caucasian neonates, male neonates, those with 1-minute Apgar scores from 8 to 10, those who had no gestational risk factors, and those not admitted to a neonatal intensive care unit (NICU). Positive correlations were observed between ETN and gestational age and birthweight. ETN was least frequent in the winter. No NICU admission or birth during the spring was associated with TNPM. CONCLUSIONS: At least in the region where this study was conducted, seasonality affects these benign pustular skin conditions. The health of the child at birth, gestational age, and birthweight influenced the development of ETN. This finding may indicate a role of immune system maturity in ETN, but additional studies are needed to confirm this.

Role of gut microbiota in infectious and inflammatory diseases.

Thousands of microorganisms compose the human gut microbiota, fighting pathogens in infectious diseases and inhibiting or inducing inflammation in different immunological contexts. The gut microbiome is a dynamic and complex ecosystem that helps in the proliferation, growth, and differentiation of epithelial and immune cells to maintain intestinal homeostasis. Disorders that cause alteration of this microbiota lead to an imbalance in the host's immune regulation. Growing evidence supports that the gut microbial community is associated with the development and progression of different infectious and inflammatory diseases. Therefore, understanding the interaction between intestinal microbiota and the modulation of the host's immune system is fundamental to understanding the mechanisms involved in different pathologies, as well as for the search of new treatments. Here we review the main gut bacteria capable of impacting the immune response in different pathologies and we discuss the mechanisms by which this interaction between the immune system and the microbiota can alter disease outcomes.

Milium cysts on hands; hypertrichosis on face.

The patient's dermatologic symptoms and his history of a particular chronic condition pointed toward the diagnosis.

Vohwinkel syndrome: ichthyosiform variant in a family.

Vohwinkel syndrome belongs to the group of hereditary palmoplantar keratoderma, having an autosomal dominant inheritance. In this report, the authors present a case of a four-year-old boy with diffuse scaling over his entire body and transgredient palmoplantar hyperkeratosis with some fissured areas. Family evaluation revealed that his mother and other family members were affected. Based on his clinical findings and on family history, the diagnosis of the ichthyotic Vohwinkel syndrome subtype, characterized by generalized ichthyosis and palmoplantar hyperkeratosis, was established.

Skin symptoms as diagnostic clue for autoinflammatory diseases.

Autoinflammatory disorders are immune-mediated diseases with increased production of inflammatory cytokines and absence of detectable autoantibodies. They course with recurrent episodes of systemic inflammation and fever is the most common symptom. Cutaneous manifestations are prevalent and important to diagnosis and early treatment of the syndromes. The purpose of this review is to emphasize to dermatologists the skin symptoms present in these syndromes in order to provide their early diagnosis.

Nail psoriasis treated with pulsed dye laser.

Nail changes are present in about 50% of psoriasis patients and tend to be refractory to conventional treatments. Pulsed dye laser has emerged as an alternative therapy. Our aim is to evaluate the efficacy of pulsed dye laser in nail psoriasis and the impact of treatment on quality of life. Fourteen patients were treated in monthly sessions for three months. The outcome assesment was made by the Nail Psoriasis Severity Index (NAPSI). The median improvement in the scores of the overall NAPSI, nail bed NAPSI, and nail matrix NAPSI were 44.2% (P = 0.002), 50% (P = 0.033) and 65.1% (P = 0.024), respectively.

Prevalence and characterization of neonatal skin disorders in the first 72h of life.

OBJECTIVE: To determine the prevalence of neonatal dermatological findings and analyze whether there is an association between these findings and neonatal and pregnancy characteristics and seasonality. METHODS: Newborns from three maternity hospitals in a Brazilian capital city were randomly selected to undergo dermatological assessment by dermatologists. RESULTS: 2938 neonates aged up to three days of life were randomly selected, of whom 309 were excluded due to Intensive Care Unit admission. Of the 2530 assessed neonates, 49.6% were Caucasians, 50.5% were males, 57.6% were born by vaginal delivery, and 92.5% of the mothers received prenatal care. Some dermatological finding was observed in 95.8% of neonates; of these, 88.6% had transient neonatal skin conditions, 42.6% had congenital birthmarks, 26.8% had some benign neonatal pustulosis, 2% had lesions secondary to trauma (including scratches), 0.5% had skin malformations, and 0.1% had an infectious disease. The most prevalent dermatological findings were: lanugo, which was observed in 38.9% of the newborns, sebaceous hyperplasia (35%), dermal melanocytosis (24.61%), skin desquamation (23.3%), erythema toxicum neonatorum (23%), salmon patch (20.4%), skin erythema (19%), genital hyperpigmentation (18.4%), eyelid edema (17.4%), milia (17.3%), genital hypertrophy (12%), and skin xerosis (10.9%). CONCLUSIONS: Dermatological findings are frequent during the first days of life and some of them characterize the newborn's skin. Mixed-race newborns and those whose mothers had some gestational risk factor had more dermatological findings. The gestational age, newborn's ethnicity, gender, Apgar at the first and fifth minutes of life, type of delivery, and seasonality influenced the presence of specific neonatal dermatological findings.

Syndrome in question. A case of Cowden´s syndrome.

The authors describe a case of Cowden's syndrome in a female patient with classic cutaneous lesions, plus papillomatous lesions in the gastrointestinal tract and a previous history of thyroid carcinoma. Mucocutaneous lesions occur in 90% of Cowden's syndrome cases and are characterized by facial trichilemmomas, oral mucosal papillomas and benign acral keratoses. Sites of extracutaneous involvement include: the thyroid, gastrointestinal tract, breast and endometrial tissue. There is risk of malignancies in these organs and they need to be monitored with imaging tests. The early diagnosis of the syndrome by a dermatologist through mucocutaneous lesions enables the investigation and diagnosis of extracutaneous involvement.

Syndrome in Question.

Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness.

Epidermodysplasia Verruciformis-like Skin Eruption in an HIV-positive Patient.

Epidermodysplasia Verruciformis-like skin eruption is a rare syndrome that usually occurs in patients with immunodeficiency, predisposing them to infections with human papilloma ß virus (HPV). We report here an HIV patient presenting with this syndrome.