Generalized pustular psoriasis (GPP) is a rare heterogeneous cutaneous disease characterized by multiple flares of painful pustules with widespread inflammation. 1 Chronic systemic inflammation in GPP may result in anaemia by mechanisms such as iron sequestration in macrophages. 2 Lee et al. 3 demonstrated an increased risk of psoriasis in chronic kidney disease patients with low haemoglobin levels. Though some level of associations between anaemia and GPP has been previously considered, we sought to evaluate whether the presence of anaemia was associated with adverse inpatient outcomes in patients hospitalized with GPP.We reviewed the National Inpatient Sample, a publicly available, de-identified database of US hospital encounters produced by the Agency for Healthcare Research and Quality (AHRQ), from the years 2016 to 2020. GPP diagnosis was identified by searching for International Classifications of Disease, 10th Edition (ICD-10-CM) code L40.1 (generalized pustular psoriasis) as a primary diagnosis.We utilized the AHRQ Elixhauser Comorbidity Software Refined (CMR) to identify deficiency anaemia comorbidity. A full list of ICD-10-CM codes categorized under deficiency anaemia can be viewed in Table S1. The CMR was used to search for the presence of leukaemia, lymphoma, metastatic cancer, solid tumor without metastasis and in situ/malignant. Demographic characteristics including age, sex, race, income, insurance status and outcome measures of length of hospital stay and cost of inpatient visit were collected. Cost was standardized between hospitals using cost-to-charge ratio files.Patients under the age of 18 were excluded. The sample was separated into cohorts by the presence or absence of a deficiency anaemia comorbidity. Univariate analysis was conducted between groups
Summary
Intravascular papillary endothelial hyperplasia (IPEH) is an uncommon benign malformation of the skin and subcutaneous tissue. In this retrospective multicentre study, we aimed to investigate the clinical and pathological features of 261 patients with IPEH. IPEH is classified into three categories; in our study, the proportions were pure (50%), mixed (46%) and extravascular (4%). IPEH frequently stained positive for immunohistochemical markers such as CD31, CD34, smooth muscle actin and erythroblast transformation‐specific‐related gene. Clinicians' initial impression of the lesion often included ambiguous terms such as ‘soft tissue mass’. There is an opportunity for increased awareness of this lesion and its consideration within a differential diagnosis.
In conclusion, this report emphasizes that variant analysis should always be performed in the parents of patients with sporadic EBS in order to distinguish between true de novo variants and mosaicism in parents, which has important implications for the determination of recurrence risk and thus genetic counselling.
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