Sirolimus (Rapamune), a mammalian target of Rapamycin (mTOR) inhibitor, which has been used extensively in children following solid organ transplantation, has been demonstrated to have anti-angiogenic activity in pre-clinical models. Limited experience suggests that it may have application to the treatment of vascular lesions. We describe our experience with a 1-year-old female with a kaposiform hemangioendothelioma and Kasabach-Merritt phenomenon who had rapid and dramatic response to sirolimus (0.1 mg/kg/day). This case provides further rationale for clinical trials of sirolimus in the treatment of vascular lesions.

Harlequin ichthyosis (HI) is a rare and severe form of congenital ichthyosis. Linked to deletion and truncation mutations of a keratinocyte lipid transporter, HI is characterized by diffuse epidermal hyperkeratinization and defective desquamation. At birth, the HI phenotype is striking with thick hyperkeratotic plate-like scales with deep dermal fissures, severe ectropion and eclabium, among other findings. Over the first months of life, the hyperkeratotic covering is shed, revealing a diffusely erythematous, scaly epidermis, which persists for the remainder of the patient's life. Although HI infants have historically succumbed in the perinatal period related to their profound epidermal compromise, the prognosis of HI infants has vastly improved over the past 20 years. Here, we report a case of HI treated with acitretin, focusing on the multi-faceted management of the disease in the inpatient setting. A review of the literature of the management of HI during the perinatal period is also presented.

The dosing regimen used in the present study is considerably higher than that used in previous studies of isotretinoin. At 1 year after completion of isotretinoin treatment, we found that patients receiving 220 mg/kg or more had a significantly decreased risk of relapse. Rash was the only adverse effect that was significantly more common in the high-dose group during treatment. This study suggests that significantly higher doses of isotretinoin are effective for treating acne and decreasing relapse rates without increasing adverse effects.

Atopic dermatitis is a common chronic skin condition in children. Treatment strategies often require stringent adherence to skin care regimens for symptom resolution. As many factors influence the course of the condition, we investigated the role of a designated "atopic dermatitis educator" in a pediatric dermatology clinic. We planned to determine whether the individual interaction with an atopic dermatitis educator affects the course of disease severity, resolution, and quality of life in atopic children. New and return pediatric atopic dermatitis patients from English-speaking families were recruited from a pediatric dermatology clinic with a single pediatric dermatologist. The 151 subjects were randomized to either the control or the intervention group. A total of 106 subjects completed the study. Those in the intervention group received the atopic dermatitis educator's individual counseling/education session. Subjects' severity was determined by the Scoring Atopic Dermatitis severity index and quality of life by either the Children's Dermatology Life Quality Index or the Infants' Dermatitis Quality of Life index depending on the patient's age. Analysis of covariance was measured. No significant difference was found in the percentage change of severity or quality of life between the groups.

Atopic dermatitis is a chronic inflammatory skin condition with drastic impacts on pediatric health. The pathogenesis of this common disease is not well understood, and the complex role of the skin microbiome in the pathogenesis and progression of atopic dermatitis is being elucidated. Skin commensal organisms promote normal immune system functions and prevent the colonization of pathogens. Alterations in the skin microbiome may lead to increased Staphylococcus aureus colonization and atopic dermatitis progression. Despite the evidence for their important role, probiotics have not been deemed efficacious for the treatment of atopic dermatitis, although studies suggest that probiotics may be effective at preventing the development of atopic dermatitis when given to young infants. This review will cover the most recent published work on the microbiome and pediatric atopic dermatitis.