The diagnosis of atopic dermatitis (AD) is made using evaluated clinical criteria. Management of AD must consider the symptomatic variability of the disease. It is based on hydrating topical treatment, and avoidance of specific and unspecific provocation factors. Anti-inflammatory treatment is used for exacerbation management. Topical corticosteroids remain the first choice. Systemic anti-inflammatory treatment should be kept to a minimum, but may be necessary in rare refractory cases. The new topical calcineurin inhibitors (tacrolimus and pimecrolimus) expand the available choices of topical anti-inflammatory treatment. Microbial colonization and superinfection (e.g. with Staphylococcus aureus, Malassezia furfur) can have a role in disease exacerbation and can justify the use of antimicrobials in addition to the anti-inflammatory treatment. Evidence for the efficacy of systemic antihistamines in relieving pruritus is still insufficient, but some patients seem to benefit. Adjuvant therapy includes ultraviolet (UV) irradiation preferably of UVA wavelength; UVB 311 nm has also been used successfully. Dietary recommendations should be specific and only given in diagnosed individual food allergy. Stress-induced exacerbations may make psychosomatic counselling recommendable. 'Eczema school' educational programmes have proved to be helpful.

Diagnosis of food allergy in children with atopic dermatitis (AD) relies on a good knowledge of the prevalence of the disease and of the foods most frequently involved. Our objective was to define these characteristics in a population-of Swiss children with AD. Patients referred to a pediatric allergist or a dermatologist for AD were routinely tested by skin-prick test (SPT) to seven common food allergens (milk, egg, peanut, wheat, soy, fish, and nuts), and to all other foods suspected by history. Patients with positive SPTs were further evaluated for specific serum immunoglobulin E (IgE) antibodies (by using the CAP System FEIA ). CAP values were interpreted following previously published predictive values for clinical reactivity. Patients with inconclusive results (between the 95% negative predictive value [NPV] and the 95% positive predictive value [PPV]) were challenged with the suspected food. A total of 74 children with AD were screened for food allergies. Negative SPTs excluded the diagnosis in 30 subjects. Nineteen patients were diagnosed by histories suggestive of recent anaphylactic reactions to foods and/or CAP values above the 95% PPV. Forty-three food challenges (35 open challenges and eight double-blind, placebo-controlled in children with persistent lesions of AD despite aggressive topical skin treatment) were performed in patients with positive SPTs but with inconclusive CAP values. Six patients were diagnosed as positive to 15 foods. Challenges were not performed to high-allergenic foods in young children (under 12 months of age for egg and fish, and under 3 years of age for peanuts and nuts). Altogether, 33.8% (25 of 74) of the AD patients were diagnosed with food allergy. The prevalence of food allergy was 27% (seven of 25) in the group referred to the dermatologist for primary care of AD. The foods most frequently incriminated were egg, milk, and peanuts. The prevalence of food allergy in our population was comparable to that in other westernized countries, suggesting an incidence of food allergy in approximately one-third of children with persistent lesions of AD. Together with milk and eggs, peanuts were most frequently involved in allergic reactions.

In a retrospective study including 84 patients, we assessed precipitating factors of granuloma annulare (GA) and asssociated pathologies. Fifteen per cent of the patients reported stress as an important trigger of GA, and in 10 patients (12%) we found an association between GA and diabetes mellitus: 3 latent, 4 type I and 3 type II. Eight of the diabetic patients presented multiple and 5 generalized GA. They suffered significantly more often from chronic relapsing GA than nondiabetic patients.

Bullous pemphigoid (BP) is an autoimmune blistering disorder that may very rarely occur in childhood. We describe a 9-month-old child who developed bullous pemphigoid while she was being treated for presumptive atopic eczema with a homeopathic regimen comprising sulfur, mercury, cantharides, and Rhus (Toxicodendron). She had generalized bullae and a progressive worsening of her general condition with asthenia, dehydration, malnutrition. While the role of homeopathy in triggering the disease remains unclear, our observation attests to the potential life-threatening course of childhood BP in instances where appropriate treatment is withheld.

IFAP syndrome is a rare genetic disorder characterized by ichthyosis follicularis, atrichia, and photophobia. Previous research found that mutations in MBTPS2, encoding site-2-protease (S2P), underlie X-linked IFAP syndrome. The present report describes the identification via whole-exome sequencing of three heterozygous mutations in SREBF1 in 11 unrelated, ethnically diverse individuals with autosomaldominant IFAP syndrome. SREBF1 encodes sterol regulatory element-binding protein 1 (SREBP1), which promotes the transcription of lipogenes involved in the biosynthesis of fatty acids and cholesterols. This process requires cleavage of SREBP1 by site-1-protease (S1P) and S2P and subsequent translocation into the nucleus where it binds to sterol regulatory elements (SRE). The three detected SREBF1 mutations caused substitution or deletion of residues 527, 528, and 530, which are crucial for S1P cleavage. In vitro investigation of SREBP1 variants demonstrated impaired S1P cleavage, which prohibited nuclear translocation of the transcriptionally active form of SREBP1. As a result, SREBP1 variants exhibited significantly lower transcriptional activity compared to the wild-type, as demonstrated via luciferase reporter assay. RNA sequencing of the scalp skin from IFAP-affected individuals revealed a dramatic reduction in transcript levels of low-density lipoprotein receptor (LDLR) and of keratin genes known to be expressed in the outer root sheath of hair follicles. An increased rate of in situ keratinocyte apoptosis, which might contribute to skin hyperkeratosis and hypotrichosis, was also detected in scalp samples from affected individuals. Together with previous research, the present findings suggest that SREBP signaling plays an essential role in epidermal differentiation, skin barrier formation, hair growth, and eye function.