Viral infections, including SARS-CoV-2, the virus that causes COVID-19 infection, have been implicated in the development of pustular dermatoses, including generalized pustular psoriasis (GPP) and acute generalized exanthematous pustulosis (AGEP). We performed a literature review of existing cases of GPP and AGEP associated with COVID-19 infection and/or treatment reported over a period of 12 months. We summarize the clinical characteristics of these cases and report an additional six new cases of GPP and AGEP. Seven patients with COVID-19 infection were diagnosed with new-onset or exacerbated GPP, and 33 patients were diagnosed with AGEP. In 55% of the cases, no concomitant potential culprit drug trigger was identified. We present this review of cases of COVID-associated acute pustular dermatoses to further contribute to the spectrum of cutaneous eruption associated with SARS-CoV-2 infection.
DGUOK deficiency is an autosomal recessive mitochondrial disorder characterized by hepatic and neurological manifestations. In patients with liver failure, the decision to perform LT can be difficult due to the likelihood of progressive neurological disease. We present a case of a 9-month-old boy who had DGUOK deficiency (E227K/ R118H genotype) intact neurological status and liver failure. His MRI indicated extensive white matter changes, which created hesitation to perform LT. After a multidisciplinary evaluation, he underwent LT from a living donor at 11 months of age. Six years post-transplant, he has had no significant complications and no progression of neurological symptoms. Our case supports that even in the presence of neurological MRI findings, but in the absence of significant neurological symptoms, LT represents a viable option in DGUOK-deficient patients who have the E227K/R118H mutation combination along with liver failure.
K E Y W O R D SDGUOK, liver failure, living donor, pediatric liver transplantation
SDRIFE), palmoplantar erythrodysesthesia, toxic epidermal necrolysis, and bullous dermatosis, mainly pemphigus-like lesions. 2 BP and EV-induced bullae can be clinically indistinguishable.However, histology may allow the distinction. A lichenoid pattern with basal vacuolization and interface dermatitis is observed in EV-induced bullous reactions. IFI studies in EV dermatosis are negative for BP180, BP230, Dsg1, and Dsg3 autoantibodies.EV-induced bullous dermatosis is thought to be caused by the disruption of nectin-4 at the suprabasal layers of the epidermis, where it acts as a cell-to-cell adhesion protein. 2,3 Differential diagnosis included bullosis diabeticorum and pseudoporphyria, among others. The former is a specific bullous disease occurring in patients with diabetes mellitus usually associated with peripheral neuropathy. It is usually manifested by acral asymptomatic blisters which heal slowly after 2-6 weeks. Although histology was initially described as epidermal blisters, recent publications have also reported subepidermal bullae. DIF and IFI studies are negative. 4 Pseudoporphyria is a photodistributed bullous disorder with clinical and histological features that resemble porphyria cutanea tarda. It was originally described in patients using nonsteroidal anti-inflammatory drugs, antibiotics, diuretics, and suffering from chronic renal failure among others. Clinically, it may present as vesicles, bullae, skin fragility, milia, and scarring occurring on sun-exposed skin. Histology shows subepidermal bullae with or without festooning of dermal papillae and scant to mild lymphocytic perivascular infiltrate. DIF demonstrates a granular deposit of C3 and IgG at the dermoepidermal junction. IFI studies are negative. 5 We would like to highlight the importance of considering EV in the differential diagnosis of drug-induced bullous dermatosis.EV is a novel medication recently introduced for urothelial cancer treatment, which will gain importance in the near future; hence, it is important to be aware of possible new side effects.
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