Stevens-Johnson syndrome associated with Hodgkin's lymphoma.

Hodgkin's lymphoma accounts for approximately 40% of all lymphomas presenting in childhood and can be associated with a variety of dermatologic manifestations. Here, we describe a case of Hodgkin's lymphoma presenting with Stevens-Johnson syndrome. Clinicians should be aware of this association, especially if no alterative trigger for Stevens-Johnson syndrome can be identified.

Assessment of neonaticide in the setting of concealed and denied pregnancies.

It is known that concealed and denied pregnancy are both associated with increased health risks to the mother and infant. Whilst there is literature surrounding management and safeguarding in these instances, we are not aware of a case review of post-mortem findings in infants with a history of concealed or denied pregnancy. We performed a retrospective review of all coronial post-mortems performed between 2003 and 2018 on infants and fetuses with a history of concealment or denial of pregnancy. Maternal demographics, delivery information, post-mortem findings and results of ancillary investigations were analyzed. Twenty cases (1.8% of total coronial workload in the period of the study) were included. Four women admitted to concealing their pregnancy, eleven denied their pregnancy and in the remaining five cases the bodies of the infants had been abandoned and the mother was not traceable. The bodies of these infants were found in waste disposal sites, wooded areas and in a drainpipe. Only six infants in total were judged to have survived delivery, all others were stillborn or unascertained. Perinatal hypoxia, large subdural hemorrhage and congenital pneumonia were the reported causes of death in those infants that were liveborn. In one case there was suspicion of neonaticide. Concealment and denial of pregnancy occur in a wider demographic than perhaps anticipated and is not limited to teenage primigravids. Mothers with concealed and denied pregnancy hid the body of their deceased infant out of fear of prosecution. In many circumstances, viability at birth cannot be ascertained.

Geroderma osteodysplasticum: Histological features and the role of panel-based exome sequencing in diagnosis.

Geroderma osteodysplasticum (GO) has clinical and histological features that overlap with other causes of wrinkly skin. Here we present the case of a child diagnosed with GO following exome sequencing of a panel of genes covering the wide differential diagnosis. The histological features of the overlapping conditions are presented, highlighting the utility of panel testing for conditions of this type. This is relevant to many genetic conditions and can influence ongoing management as exemplified by this case.

SARS-COV2 placentitis and pregnancy outcome: A multicentre experience during the Alpha and early Delta waves of coronavirus pandemic in England.

Background: Pregnant women with SARS-CoV-2 infection experience higher rates of stillbirth and preterm birth. A unique pattern of chronic histiocytic intervillositis (CHI) and/or massive perivillous fibrin deposition (MPFD) has emerged, coined as SARS-CoV-2 placentitis. Methods: The aim of this study was to describe a cohort of placentas diagnosed with SARS-CoV-2 placentitis during October 2020-March 2021. Cases with a histological diagnosis of SARS-CoV-2 placentitis and confirmatory immunohistochemistry were reported. Maternal demographic data, pregnancy outcomes and placental findings were collected. Findings: 59 mothers delivered 61 infants with SARS-CoV-2 placentitis. The gestational age ranged from 19 to 41 weeks with most cases (78.6%) being third trimester. 30 infants (49.1%) were stillborn or late miscarriages. Obese mothers had higher rates of pregnancy loss when compared with those with a BMI <30 [67% (10/15) versus 41% (14/34)]. 47/59 (79.7%) mothers had a positive SARS-CoV-2 PCR test either at the time of labour or in the months before, of which 12 (25.5%) were reported to be asymptomatic. Ten reported only CHI, two cases showed MPFD only and in 48 placentas both CHI and MPFD was described. Interpretation: SARS-CoV2 placentitis is a distinct entity associated with increased risk of pregnancy loss, particularly in the third trimester. Women can be completely asymptomatic and still experience severe placentitis. Unlike 'classical' MPFD, placentas with SARS-CoV-2 are generally normal in size with adequate fetoplacental weight ratios. Further work should establish the significance of the timing of maternal SARS-CoV-2 infection and placentitis, the significance of SARS-CoV2 variants, and rates of vertical transmission associated with this pattern of placental inflammation. Funding: There was not funding associated with this study.

Metachronous Diffuse Large B-Cell Lymphoma and Kaposi Sarcoma of the Right Eyelid and Lacrimal Gland in a Patient with Granulomatous Common Variable Immunodeficiency.

PURPOSE: To describe the ophthalmic and histopathological features of a female with granulomatous common variable immunodeficiency (CVID) who presented with upper-lid swelling. PROCEDURES: The patient underwent a biopsy of the right upper lid/palpebral lacrimal gland with imaging showing a left-sided nasopharyngeal mass, multiple lymph nodes within the mediastinum, bilateral lung nodules and a peritoneal nodule in the right iliac fossa. The right upper-lid swelling progressed and was subject to a second biopsy. RESULTS: The first right upper-lid biopsy revealed a diffuse large B-cell lymphoma (DLBCL), confirmed with clonal IgH gene rearrangement with PCR. The nasopharyngeal mass and lymph nodes were suspected clinically to be DLBCL. However, a biopsy of the nasopharyngeal mass showed Kaposi sarcoma (KS). The second biopsy of the right upper lid/palpebral lacrimal gland revealed KS with no evidence of DLBCL. CONCLUSION: This is the first documentation of periocular/orbital metachronous DLBCL and KS in a patient with granulomatous CVID. We discuss the role of fluctuating immunity in CVID to explain the spontaneous regression of the DLBCL and the varying clinical picture.