CARMIL2-related immunodeficiency manifesting with photosensitivity

We report a case of a newly recognized primary immunodeficiency due to biallelic mutations in CARMIL2 manifesting as an actinic prurigo-like photodermatitis, allergic diathesis and recurrent infections in a child. We present this case to highlight a rare phenotype seen in this T-cell immunodeficiency and provide an overview of other dermatologic manifestations among published reports of this condition.     

Solitary congenital Langerhans cell histiocytoma: A pattern of benign,spontaneous regression in patients with single lesion disease

Langerhans cell histiocytosis (LCH) is the neoplastic proliferation of dendritic langerin-positive histiocytes manifesting as either single system unifocal, single system multifocal, or multisystem disease. The designation Hashimoto-Pritzker, or self-healing LCH, has fallen out of favor since it is impossible to predict at time of diagnosis whether the disease is truly self-remitting or capable of spreading to other organ systems. We review the English literature on solitary congenital Langerhans cell histiocytoma, draw novel conclusions from the data provided by 81 cases in the literature, and illustrate a typical presentation of the diagnosis with a previously unreported patient. Each of the patients diagnosed with solitary congenital histiocytoma experienced spontaneous resolution and had no signs of systemic disease at latest follow-up. Furthermore, we offer an analysis of the histopathological findings available from the 81 cases and our patient. Based on our study observations, we propose solitary congenital Langerhans cell histiocytoma may portend a good prognosis and represent a distinct entity. However, until further confirmation with prospective studies, we recommend clinicians continue conducting appropriate workup to rule out systemic involvement.     

Hospital Readmission Penalties: Coming Soon to a Nursing Home Near You!

The Protecting Access to Medicare Act of 2014 includes provisions for hospital readmission penalties for skilled nursing facilities (SNFs) starting in 2018. This presents an opportunity for care improvement but also raises several concerns regarding quality of care. The readmission measure for SNFs is similar to the current readmission measure for hospitals mandated under the Affordable Care Act, with the exception of adjustments made for sex. Because these measures for hospitals are similar, lessons can be learned from implementation of the existing hospital readmission penalties. In addition, there are three specific concerns that the authors relate to implementing the proposed measure in SNFs. There is poor communication and care coordination between care settings, including posthospitalization and post‐SNF care in the current healthcare system. Adding readmission penalties to SNF regulations may create perverse incentives for prolonged SNF stays. The evidence base for the best means of caring for individuals after a brief stay in a SNF needs enrichment. These challenges need to be addressed as part of implementation of these new hospital readmission penalties for SNFs to improve care and prevent new unintended consequences.