Athena Speciality Certificate Examination case for formulation and systemic therapy.

This Athena case describes possible differential diagnoses in a patient with multiple blisters. Histopathology showed subepidermal clefting and superficial dermal inflammation, while immunohistochemistry showed linear deposition of IgG and C3 along the basement membrane zone.

Monkeypox: key pointers for dermatologists.

The current monkeypox epidemic is a major public health concern as the global outbreak continues to grow. We present a case report to highlight the salient points for dermatologists. Human monkeypox is a zoonosis caused by monkeypox virus, belonging to the Orthopoxvirus genus and is a close relative of the variola virus. Pleomorphic skin lesions appear ranging from macules, papules, vesicles, pustules, some of which may appear umbilicated before crusting over. Dermatologists play a key role in early recognition of new cases, aiding diagnoses leading to prevention of disease spread through identification and isolation, contact tracing and education.

Panton-Valentine leucocidin-producing Staphylococcus aureus: a clinical review.

Panton-Valentine leucocidin (PVL) is a virulence factor produced by certain strains of Staphylococcus aureus (SA). Through its cytolytic action on the cell membranes of human polymorphonuclear neutrophils, PVL causes a range of pathologies collectively known as PVL-SA disease. The hallmark clinical signs of PVL-SA are recurrent boils and necrotizing skin and soft tissue infections (SSTIs) in otherwise healthy patients; however, it can lead to more severe and invasive presentations, including necrotizing haemorrhagic pneumonia, necrotizing fasciitis and purpura fulminans. Young adults with minimal previous exposure to healthcare settings tend to be at highest risk for acquiring PVL-SA disease, with close physical contact playing a central role in disease transmission. The prevalence of PVL-SA varies globally; however, this is often underestimated owing to a lack of routine PVL testing. In the UK, PVL-positive SA isolates have been rising over the past decade alongside an increasing prevalence of multidrug resistance in larger cities. This review article aims to raise awareness of the PVL toxin, to aid clinicians with diagnostic pointers and to provide guidance with treatment, with an emphasis on the need for further population-based studies.

Influenza Vaccination as a Novel Trigger of Wells Syndrome in a Child.

Wells syndrome is a rare disorder of unknown etiology. Precipitants include insect bites, infections, medications, malignancies, and vaccinations. Possible mechanisms include hypersensitivity reactions to antigens. There are four reports in the literature of Wells syndrome precipitated by vaccinations (hepatitis B vaccine, tetanus vaccine, tetanus-diptheria vaccine and triple antigen vaccine). We present a further case of Wells syndrome in a 22-month-old child after influenza vaccine as a novel trigger not previously reported.

Epithelial inflammation resulting from an inherited loss-of-function mutation in EGFR.

Epidermal growth factor receptor (EGFR) signaling is fundamentally important for tissue homeostasis through EGFR/ligand interactions that stimulate numerous signal transduction pathways. Aberrant EGFR signaling has been reported in inflammatory and malignant diseases, but thus far no primary inherited defects in EGFR have been recorded. Using whole-exome sequencing, we identified a homozygous loss-of-function missense mutation in EGFR (c.1283 G>A; p.Gly428Asp) in a male infant with lifelong inflammation affecting the skin, bowel, and lungs. During the first year of life, his skin showed erosions, dry scale, and alopecia. Subsequently, there were numerous papules and pustules--similar to the rash seen in patients receiving EGFR inhibitor drugs. Skin biopsy demonstrated an altered cellular distribution of EGFR in the epidermis with reduced cell membrane labeling, and in vitro analysis of the mutant receptor revealed abrogated EGFR phosphorylation and EGF-stimulated downstream signaling. Microarray analysis on the patient's skin highlighted disturbed differentiation/premature terminal differentiation of keratinocytes and upregulation of several inflammatory/innate immune response networks. The boy died at the age of 2.5 years from extensive skin and chest infections as well as electrolyte imbalance. This case highlights the major mechanism of epithelial dysfunction following EGFR signaling ablation and illustrates the broader impact of EGFR inhibition on other tissues.

Subcutaneous granuloma annulare of the penis in 2 adolescents.

Granuloma annulare (GA) is a benign inflammatory disorder of unknown etiology characterized histologically by dermal palisading granulomas with central degeneration of collagen (necrobiosis). There is a rare subcutaneous clinical variant, this occurring more frequently in children than in adults and very rarely involves the penis. We describe 2 cases of penile subcutaneous GA developing in adolescent boys who to our knowledge has not previously been described in literature. Both were initially treated with surgical excision. Circumcision was performed on one of the boys, with subsequent improvement with the resolution of most of the nodules. Granuloma annulare of the penis is very rare, with only 7 cases reported to date [Narouz N, Allan PS, Wade AH. Penile granuloma annulare. Sex Transm Infect 1999;75(3):186-7; Trap R, Wiebe B. Granuloma annulare localised to the shaft of the penis. Scand J Urol Nephrol 1993;27(4):549-51; Laird SM. Granuloma annulare of the penis. Genitourin Med 1992;68(4):277; Hillman RJ, Waldron S, Walker MM, et al. Granuloma annulare of the penis. Genitourin Med 1992;68(1):47-9; Kossard S, Collins AG, Wegman A, et al. Necrobiotic granulomas localised to the penis: a possible variant of subcutaneous granuloma annulare. J Cutan Pathol 1990;17(2):101-4] and no previous reports in children or adolescents to our knowledge. All except one of the cases reported so far were of the subcutaneous (nodular) form of GA.

Neonatal hemangiomatosis and atrial septal defect: a rare cause of right heart failure in infancy.

Neonatal hemangiomatosis is a well-recognized cause of left ventricular failure. We describe an infant with neonatal hemangiomatosis and an ostium secundum atrial septal defect who developed severe right heart failure. This was due to the combination of increased flow through the right heart as a result of the atrial septal defect, and the background high cardiac output from the massive arteriovenous shunting and multiple hemangiomas. In addition, the multiple hepatic hemangiomas may exert a vasoactive influence on the pulmonary vasculature.