ABSTRACT
Patients with Atopic Dermatitis (AD) suffer from inflamed skin and skin barrier defects. Proper formation of the outermost part of the skin, the stratum corneum (SC), is crucial for the skin barrier function. In this study we analyzed the localization and activity of lipid enzymes ß-glucocerebrosidase (GBA) and acid sphingomyelinase (ASM) in the skin of AD patients and controls. Localization of both the expression and activity of GBA and ASM in the epidermis of AD patients was altered, particularly at lesional skin sites. These changes aligned with the altered SC lipid composition. More specifically, abnormal localization of GBA and ASM related to an increase in specific ceramide subclasses [AS] and [NS]. Moreover we related the localization of the enzymes to the amounts of SC ceramide subclasses and free fatty acids (FFAs). We report a correlation between altered localization of active GBA and ASM and a disturbed SC lipid composition. Localization of antimicrobial peptide beta-defensin-3 (HBD-3) and AD biomarker Thymus and Activation Regulated Chemokine (TARC) also appeared to be diverging in AD skin compared to control. This research highlights the relation between correct localization of expressed and active lipid enzymes and a normal SC lipid composition for a proper skin barrier.
Subject(s)
Dermatitis, Atopic/immunology , Epidermis/pathology , Glucosylceramidase/metabolism , Lipid Metabolism/immunology , Sphingomyelin Phosphodiesterase/metabolism , Adolescent , Adult , Biopsy , Case-Control Studies , Ceramides/analysis , Ceramides/metabolism , Chemokine CCL17/metabolism , Dermatitis, Atopic/pathology , Epidermis/chemistry , Epidermis/enzymology , Fatty Acids, Nonesterified/analysis , Fatty Acids, Nonesterified/metabolism , Female , Healthy Volunteers , Humans , Male , Water Loss, Insensible/immunology , Young Adult , beta-Defensins/metabolismABSTRACT
We present a 38-year-old woman who suddenly developed erythema with follicular papules and pustules on her face during the second trimester of pregnancy. The diagnosis 'rosacea fulminans' was made. This is an uncommon skin condition that predominantly affects younger women. Treatment with erythromycin was successful.
Subject(s)
Rosacea/diagnosis , Adult , Diagnosis, Differential , Erythema/diagnosis , Erythema/drug therapy , Erythromycin/therapeutic use , Female , Humans , Pregnancy , Pregnancy Trimester, Second , Rosacea/drug therapy , Treatment OutcomeABSTRACT
Pyoderma gangrenosum is a rare, destructive ulcerative condition of the skin. The painful ulcers may occur spontaneously, or result from a minor injury or surgery. The clinical presentation can mimic a necrotizing bacterial infection; however, no micro-organisms can be cultured from the skin lesions and the ulcers fail to respond to antibiotic therapy. Surgical interventions can aggravate the disease process. We describe 2 patients, a 22-year-old woman and a 45-year-old woman, with ulcerative wound abnormalities after breast surgery. After failure of antibiotic therapy and standard wound care, tissue biopsy of the wounds confirmed the clinical diagnosis 'pyoderma gangrenosum'. Wound healing began after systemic steroid treatment. Delays in diagnosis and treatment of pyoderma gangrenosum may result in extensive ulceration and scarring. It is, therefore, important to recognise the characteristic clinical features at an early stage and to start appropriate treatment immediately.
