Faculty Bibliography

BACKGROUND:Cutaneous (or "Metastatic") Crohn disease (CCD) is a rare and underrecognized disease characterized by cutaneous granulomatous inflammation. We describe patient demographics, clinical characteristics, histology, and treatment of 89 pediatric cases of CCD, including 78 previously reported and 11 new cases seen at four academic institutions. We emphasize the efficacy of biologic mono- and dual therapy. METHODS:PubMed identified cases using keywords including "metastatic Crohn disease" and "cutaneous Crohn disease". Patients were identified by retrospective review of the electronic health record including histopathologic diagnosis consistent with CCD. Chart review collected demographic, clinical, and histologic data. RESULTS:Most pediatric patients with CCD are male 55% (49/89), present with edema (73/89, 82%) and erythema (47/89, 53%) of the genitals (33/49, 67%), and have intestinal Crohn disease (69/89, 78%). Oral corticosteroids (53/75, 71%) and metronidazole (29/75, 39%) are the most frequently prescribed medications. Of the 17 patients treated with tumor necrosis factor (TNF)-blockade, 94% (16/17) had partial or total clearance. Ustekinumab resulted in clearance of cutaneous disease in two patients (2/3, 67%) and partial clearance in one patient (1/3, 33%). Two cases achieved total clearance with the use of dual biologic therapy defined as the use of two biologic therapies with differing mechanisms of action or the use of a biologic therapy and small molecule inhibitor. CONCLUSIONS:TNF blockade is an effective treatment for pediatric CCD, and interleukin-12/23 inhibitors may be similarly effective. Consideration of dual biologic therapy may be useful in pediatric patients requiring discordant therapies for their intestinal and cutaneous CD.

During 2016-2022, Medicare part D beneficiaries filled 8,674,460 clotrimazole-betamethasone dipropionate prescriptions. Annual rates were stable (30.9 prescriptions/1,000 beneficiary-years in 2022, enough for one in every 33 beneficiaries). Diagnostic testing was infrequent, particularly among internal medicine, family medicine, and general practitioners, suggesting potential opportunities to improve diagnostic and prescribing practices.

BACKGROUND:Necrobiosis lipoidica (NL) is a rare, idiopathic, and recalcitrant disease of collagen degeneration for which treatment options have been poorly studied. Due to its recurring nature, risk for ulceration, and high morbidity, there is a need to understand existing treatment modalities to better inform clinical care. OBJECTIVE:This review aims to describe the therapeutic modalities reported in the literature for the treatment of NL. METHODS:A literature search of treatments was performed by searching for publications between January 2016 and May 2022 on PubMed and Scopus. Given the limited high-quality evidence, case reports and series were included. Only publications presenting information on both attempted treatments and outcomes were included. RESULTS:A total of 60 novel articles were identified (54 case reports, two case series, and four retrospective cohort studies). These studies cumulatively reported on 274 patients and covered treatments including phototherapy, topical corticosteroids, topical calcineurin inhibitors, biologics, immunosuppressants, JAK inhibitors, combination therapies, and several others. The greatest amount of evidence was found for photodynamic therapy (improvement in 72 of 80 patients), UVA-based phototherapy (12 of 33), topical corticosteroids (21 of 46), compression therapy (15 of 20), and topical calcineurin inhibitors (11 of 17). Several newer treatments were also described, including ustekinumab and JAK inhibitors. CONCLUSIONS:This systematic review provides a comprehensive summary of recently published treatments for NL. As the existing data comes predominantly from case reports and series, statistical conclusions are not assessed. A greater number of randomized controlled trials with standardized endpoints are necessary to compare treatment efficacy.

Sarcoidosis is a chronic, multisystem, inflammatory disorder that is characterized by noncaseating granulomas that cause organ dysfunction with various clinical subphenotypes. The incidence and prevalence of sarcoidosis varies greatly by ethnic background. There are significant racial disparities in prevalence, severity, and outcomes; however, there is a dearth of studies investigating the impact of structural racism. The skin is often the presenting and second most frequently involved organ with significant implications on diagnosis and management in patients with darkly pigmented skin. Workup should be comprehensive given the multisystem involvement. There are many therapies for sarcoidosis, although none is universally effective.

Purpose of Review: Dermatomyositis can present with a range of manifestations and severity that may necessitate hospital admission. Dermatologists are frequently consulted for patients with dermatomyositis inpatient. Herein we describe clinical features and management of multisystem complications of dermatomyositis with a focus on the inpatient setting. Recent Findings: Patients with dermatomyositis are at risk for hospitalization due to disease flares, infections, and systemic complications. Furthermore, patients may seek care for symptoms including shortness of breath, fever, or cutaneous eruptions which can lead to a new diagnosis of dermatomyositis. Patients with dermatomyositis have increased healthcare utilization and necessitate multidisciplinary and collaborative care. Cutaneous findings may be subtle yet provide important prognostic information. Symptoms arising from skin disease may also be chronic and refractory. Summary: Dermatologists are essential in both diagnosing and managing dermatomyositis and must be attuned to the multiple systemic manifestations and complications that impact inpatient care.