Juvenile xanthogranulomas: Examining single, multiple, and extracutaneous presentations.

BACKGROUND: Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis characterized by yellowish papules in the skin. JXGs most often occur in infancy or early childhood and are typically solitary and asymptomatic, often regressing after several years. While JXGs predominantly occur on the skin, extracutaneous JXGs also exist. AIMS: In this paper, we review the literature on single, multiple, and visceral JXGs and provide recommendations on monitoring and work-up. MATERIALS & METHODS: A literature review was conducted with the PubMed database using selective search terms for single, multiple, ocular, and visceral lesions as well as NF1/JMML. RESULTS / DISCUSSION: JXG is typically a self-limited disorder if lesions are cutaneous and singular. While rare, JXGs may manifest as multiple and extracutaneous lesions. Further screening and referral to specialists may be warranted in these cases based on age and extent of involvement. CONCLUSION: Our review demonstrates common presentations of single, multiple, and extracutaneous lesions in addition to those that occur with NF1 and JMML. We suggest patients be evaluated on a case-by-case basis by a dermatologist and referred to specialists as appropriate.

Professionalism of clinical photography in the pediatric setting.

Photography has a wide range of use in the medical field, ranging from clinical care to publication and teaching purposes. While photography is more common in certain specialties such as dermatology and plastic surgery, the accessibility to cameras and ease of documentation provides the opportunity for all fields to utilize this technology. In pediatric dermatology, photographs are taken to determine if conditions such as rashes and moles have changed or remained stable. Photos are also taken prior to biopsies to ensure the correct site is biopsied and used for monitoring for post-excisional changes. In surgery, photography is used to capture pre- and post-operative changes. Photography is also used to document trauma and physical abuse, which may be used as legal evidence. Clinical photography has also demonstrated its usefulness in this age of team-based healthcare, where different providers see the same patients over subsequent visits, allowing for new providers to see photographical documentation of conditions at a prior state.

The use of etanercept for treatment of toxic epidermal necrolysis when toxic shock syndrome is in the differential.

Toxic shock syndrome (TSS) can sometimes mimic Steven Johnsons syndrome/toxic epidermal necrolysis (SJS/TEN). Tumor necrosis factor (TNF) alpha is thought to play a role in the pathogenesis of both TSS and SJS/TEN. Etanercept, a TNF-alpha inhibitor has been recently shown to treat and decrease mortality of SJS/TEN. We report a 51-year-old female with history of SJS presenting with painful skin and bullae 2 days following cystoscopy with botulinum toxin injection into the bladder. Due to initial concern for SJS/TEN, the patient was treated with 50 mg of subcutaneous etanercept. Punch biopsies were not consistent with SJS, and the patient fulfilled five out of five criteria for a confirmed case of TSS. The patient ultimately had a favorable outcome despite etanercept treatment. Ultimately, TNF-alpha antagonists are an emerging therapy to treat SJS/TEN, and are unlikely to worsen TSS prognosis. Given that etanercept can be used to successfully treat SJS/TEN and TNF-alpha levels are elevated in TSS, if a dermatologist chooses to treat TEN with etanercept, consideration of TSS on the differential should not necessarily exclude etanercept as a reasonable treatment option.

Case Report: Patient with Hepatitis C, p-ANCA, and Cryoglobulin Antibodies Presenting with Necrotizing Crescentic p-ANCA Glomerulonephritis.

Hepatitis C (HCV) infection has a prevalence of 3 million infected individuals in the United States, according to recent Center for Disease Control reports, and can have various renal manifestations. Cryoglobulins, antibodies that precipitate at colder temperatures in vitro, are a relatively common cause of renal disease in HCV infection. The cryoglobulin proteins can form occlusive aggregates in small glomerular capillary lumina or deposit in other areas of the glomerulus, resulting in hypocomplementemia, proteinuria, hematuria, and renal injury. The typical biopsy pattern is that of membranoproliferative glomerulonephritis (MPGN). There are, however, other HCV-related patterns of glomerular injury. Anti-neutrophil cytoplasmic antibodies (ANCA) are known to exist in HCV-infected patients. In many reported cases, ANCA serologic testing may appear positive due to cross-reactivity of the immune assays; however, the biopsy findings do not support ANCA-associated crescentic glomerulonephritis (GN)/vasculitis as the primary cause of glomerular injury. There are rare reports of microscopic polyangiitis (MPA) p-ANCA vasculitis, in patients with HCV infection. In comparison with the MPGN pattern of cryoglobulinemic glomerular injury, biopsies from these HCV-infected patients with concomitant MPA revealed a crescentic GN, associated with normal serum complement levels. We present a case of HCV-associated glomerular disease with the surprising biopsy finding of necrotizing and crescentic p-ANCA GN, with a background, low-grade mesangial immune complex GN. Thus, p-ANCA disease should also be considered in HCV-infected patients, in addition to the more typical lesions of MPGN or cryoglobulinemic GN.

Usefulness of intra-procedural cone-beam computed tomography in modified balloon-occluded retrograde transvenous obliteration of gastric varices.

AIM: To evaluate whether intra-procedural cone-beam computed tomography (CBCT) performed during modified balloon-occluded retrograde transvenous obliteration (mBRTO) can accurately determine technical success of complete variceal obliteration. METHODS: From June 2012 to December 2014, 15 patients who received CBCT during mBRTO for treatment of portal hypertensive gastric variceal bleeding were retrospectively evaluated. Three-dimensional (3D) CBCT images were performed and evaluated prior to the end of the procedure, and these were further analyzed and compared to the pre-procedure contrast-enhanced computed tomography to determine the technical success of mBRTO including: Complete occlusion/obliteration of: (1) gastrorenal shunt (GRS); (2) gastric varices; and (3) afferent feeding veins. Post-mBRTO contrast-enhanced CT was used to confirm the accuracy and diagnostic value of CBCT within 2-3 d. RESULTS: Intra-procedural 3D-CBCT images were 100% accurate in determining the technical success of mBRTO in all 15 cases. CBCT demonstrated complete occlusion/obliteration of GRS, gastric varices, collaterals and afferent feeding veins during mBRTO, which was confirmed with post-mBRTO CT. Two patients showed incomplete obliteration of gastric varices and feeding veins on CBCT, which therefore required additional gelfoam injections to complete the procedure. No patient required additional procedures or other interventions during their follow-up period (684 ± 279 d). CONCLUSION: CBCT during mBRTO appears to accurately and immediately determine the technical success of mBRTO. This may improve the technical and clinical success/outcome of mBRTO and reduce additional procedure time in the future.