No abstract

An effective epidermal barrier requires structural and functional integration of adherens junctions, tight junctions, gap junctions (GJ), and desmosomes. Desmosomes govern epidermal integrity while GJs facilitate small molecule transfer across cell membranes. Some patients with severe dermatitis, multiple allergies, and metabolic wasting (SAM) syndrome, caused by biallelic desmoglein 1 (DSG1) mutations, exhibit skin lesions reminiscent of erythrokeratodermia variabilis, caused by mutations in connexin (Cx) genes. We, therefore, examined whether SAM syndrome-causing DSG1 mutations interfere with Cx expression and GJ function. Lesional skin biopsies from SAM syndrome patients (n ¼ 7) revealed decreased Dsg1 and Cx43 plasma membrane localization compared with control and nonlesional skin. Cultured keratinocytes and organotypic skin equivalents depleted of Dsg1 exhibited reduced Cx43 expression, rescued upon re-introduction of wild-type Dsg1, but not Dsg1 constructs modeling SAM syndrome-causing mutations. Ectopic Dsg1 expression increased cell-cell dye transfer, which Cx43 silencing inhibited, suggesting that Dsg1 promotes GJ function through Cx43. As GJA1 gene expression was not decreased upon Dsg1 loss, we hypothesized that Cx43 reduction was due to enhanced protein degradation. Supporting this, PKC-dependent Cx43 S368 phosphorylation, which signals Cx43 turnover, increased after Dsg1 depletion, while lysosomal inhibition restored Cx43 levels. These data reveal a role for Dsg1 in regulating epidermal Cx43 turnover.

Kerion is a severe hypersensitivity reaction to fungal infection that is rarely seen in the groin. Frequent shaving of pubic hair and religious conservatism surrounding genital hygiene are common among Bedouin women in the Negev Desert, and may predispose to kerion. This case highlights the clinical course of a 20-year-old Bedouin woman who presented with severe kerion celsi of the pubis and vulva with secondary bacterial infection. The patient was successfully treated with intravenous antibiotics, oral antifungal medication and wet topical dressings. The case outlines the risk factors and treatment for severe kerion celsi of the groin, as well as possible preventive measures that may reduce its incidence.

Scabies is a pruritic disorder caused by Sarcoptes scabiei var. hominis infestation of the skin. Transferred by close body contact, scabies is endemic within nursing homes and among poor and overcrowded populations. Crusted scabies is a severe form of disease, characterised by a large, thick, crusted eruption with significant mite infestation. We report a patient hospitalised with crusted scabies that had massive nail involvement. A 79-year-old female patient with multiple comorbidities and several recent prior scabies diagnoses presented with agitation and dystrophic fingernails; scabies mites were found embedded in and below the nail keratin. Aggressive treatment resulted in complete resolution, with notable improvements in mental status. Crusted scabies with nail involvement is extremely rare and may be more likely to develop from initially subclinical infestation sites. It is important to consider this potential presentation, as standard topical treatments may prove ineffective when there is deep nail involvement.

Gram‐negative bacterial toe web infection (GNBTWI) caused by Pseudomonas Aeruginosa combined with fungal infection has variety of treatments. However, these treatments have been poorly described in the literature. Our retrospective study describes patients that had been treated in our medical center with acetic acid combined with local antifungal treatment, to evaluate evidences for the appropriateness of this treatment. Ten patients with evidence of GNBTWI caused by Pseudomonas Aeruginosa combined with local fungal infection which have been treated with acetic acid in Emek Medical Center were identified. Eight patients (80%) had a complete response while two patients (20%) showed only a partial response. Side effects were minimal and included temporary stinging sensation. Acetic acid is a relatively cheap ingredient with minimal side effect profile and highly effective outcomes as a treatment for GNBTWI caused by Pseudomonas Aeruginosa and should be considered as an adjuvant treatment.