Background: A 25-year-old female with a history of SLE on HCQ, MMF, belimumab and prednisone presented with an acute onset of scattered skin-colored asymptomatic papules with central umbilication and crusting involving the face, trunk, extremities and the groin. Biopsy showed extensive acantholysis, multi-nucleated keratinocytes with peripheral margination of chromatin, and single cell necrosis. These findings raised concern for herpesvirus infection and possible disseminated zoster and the patient was admitted for IV acyclovir. Notably the patient denied a history of chickenpox infection and had received one varicella vaccine as a child. During hospitalization, lesional VZV PCR was positive, and the patient had fever, cough, transaminitis with a chest x-ray showing left sided patchy opacity that were all favored to reflect disseminated zoster. After the lesions had crusted, the patient transitioned to a 6-month course of oral valacyclovir. Varicella zoster virus commonly causes two distinct dermatologic presentations: (1) "chickenpox" and (2) herpes zoster (HZ) or "shingles."1 Chickenpox occurs following primary VZV infection in non-immune hosts whereas HZ occurs following reactivation of dormant VZV in the dorsal root ganglia.1 It may be difficult to distinguish disseminated HZ from primary varicella infection, particularly if patients do not recall a history of chickenpox and have an incomplete or unknown vaccination history. Both conditions can be complicated by disseminated disease and life threatening sequelae, more commonly in immunocompromised patients. 2, 3 Assessing immunity to VZV may be beneficial in immunocompromised patients, as primary VZV and disseminated HZ can be associated with significant morbidity.

A 70-year-old woman with newly diagnosed AML awaiting induction chemotherapy presented with pruritic erythematous papulovesicles on her back, abdomen, and extremities. Vesicle fluid HSV and VZV PCR tests were negative, but the patient was diagnosed with presumed disseminated zoster and started on IV acyclovir. However, after 8 days of treatment the rash was not improved. Biopsy at that time revealed hyperkeratosis, focal intraepidermal split with necrotic epidermis, acantholysis, and features consistent with herpetic cytopathic changes including multinucleated keratinocytes and nuclear molding. Immunohistochemical stains for HSV and VZV were negative, and repeat lesional fluid PCR for HSV and VZV were also negative. The patient was diagnosed with pseudoherpetic transient acantholytic dermatosis (TAD) or Grover disease. The rash improved with use of triamcinolone 0.1% cream and ammonium lactate lotion. TAD classically presents with pruritic erythematous papules across the trunk and is thought to be brought on by UV radiation, sweat, heat, or persistent fevers. Histology shows focal acantholysis, dyskeratosis, and intraepidermal clefting. Variants that resemble other acantholytic diseases such as Darier disease, pemphigus vulgaris and Hailey-Hailey disease have been described. A rare variant, pseudoherpetic TAD (P-TAD), has been reported to mimic HSV/VZV infection with multinucleated and hypereosinophilic keratinocytes but lacks true cytopathic effect despite multinucleation; HSV/VZV immunohistochemistry is negative. Clinically, PHD features ungrouped vesicles distributed regionally rather than the grouped vesicles classically seen in herpetic infection. Clinicians should consider a diagnosis of P-TAD in patients with suspected herpetic infection with negative confirmatory PCR testing and lack of response to antivirals.

BACKGROUND:Atopic dermatitis (AD) is a common, chronic type 2 inflammatory skin disease, typically starting in infancy, with increased risk for subsequent extracutaneous atopic morbidities. Dupilumab is the first biologic agent targeting type 2 inflammation approved by the U.S. Food and Drug Administration (USFDA); it was licensed in 2017 for adults with moderate to severe AD and 2 years later for adolescents. Systemic treatment for pediatric AD remains a significant unmet medical need. OBJECTIVE:To analyze off-label use of dupilumab in children with AD. METHODS:Multicenter retrospective review that evaluated children who were prescribed dupilumab for moderate to severe AD. RESULTS:One hundred eleven of 124 patients (89.5%) gained access to dupilumab after a mean of 9 weeks. The dosing range was 4 to 15.5 mg/kg for the loading dose and 2.0 to 15.3 mg/kg every other week for maintenance. The range was widest for 6- to 11-year-olds and was related to use of either full or half of adult dosing. Associated morbidities, treatment response, and adverse events were comparable to those in previous adolescent and adult trials. LIMITATIONS:The retrospective design of the study limited uniform data collection. CONCLUSION:Access to dupilumab was achievable for the majority of children after a mean 9-week delay because of insurance payment denial. This review supports dupilumab response and tolerability in children. Optimal dosing for patients younger than 12 years has not been defined. Availability of the drug in 2 different concentrations is an important safety issue.

Attitudes of patients as well as infectious disease, gastroenterology, and primary care providers need to be addressed to improve surveillance rates for high-risk patients with chronic hepatitis B infections.