Novel use of poly-L-lactic acid filler for the treatment of facial cutaneous atrophy in patients with connective tissue disease.

BACKGROUND: Patients with connective tissue disease (CTD) often suffer from facial cutaneous defects and resultant facial asymmetry. Unfortunately, these issues have been known to be difficult-to-treat, and concern exists regarding the use of cosmetic procedures in this patient population due to the theoretical risk of disease flare-up or reactivation. Injectable poly-L-lactic acid (PLLA) is one type of filler that has been used to treat skin atrophy in patients with morphea and lupus erythematous panniculitis. However, overall, there is a dearth in literature regarding the safety and efficacy of PLLA filler in patients with CTDs. AIMS: This case series intends to evaluate the safety and efficacy of PLLA filler in treating facial atrophy in patients with CTDs. PATIENTS/METHODS: Three patients underwent various treatment courses involving the use of PLLA filler to treat facial atrophy. RESULTS: Two patients demonstrated significant improvement in facial atrophy following their treatment course. No patient experienced reactivation or exacerbation of their CTD following PLLA injection. CONCLUSION: PLLA filler appears to have good viability as a safe and potentially effective treatment for facial atrophy in patients with CTDs.

Development and Validation of a Risk Prediction Model for In-Hospital Mortality Among Patients With Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis-ABCD-10.

Importance: Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a spectrum of severe mucocutaneous drug reaction associated with significant morbidity and mortality. A previously developed SJS/TEN-specific severity-of-illness model (Score of Toxic Epidermal Necrolysis [SCORTEN]) has been reported to overestimate and underestimate SJS/TEN-related in-hospital mortality in various populations. Objective: To derive a risk prediction model for in-hospital mortality among patients with SJS/TEN and to compare prognostic accuracy with the SCORTEN model in a multi-institutional cohort of patients in the United States. Design, Setting, and Participants: Data from a multicenter cohort of patients 18 years and older treated for SJS/TEN between January 1, 2000, and June 1, 2015, were obtained from inpatient consult databases and electronic medical record systems at 18 medical centers in the United States as part of the Society for Dermatology Hospitalists. A risk model was derived based on data from 370 of these patients. Model discrimination (calculated as area under the receiver operating characteristic curve [AUC]) and calibration (calculated as predicted vs observed mortality, and examined using the Hosmer-Lemeshow goodness-of-fit statistic) were assessed, and the predictive accuracy was compared with that of SCORTEN. All analysis took place between December 2016 and April 2018. Main Outcomes and Measures: In-hospital mortality. Results: Among 370 patients (mean [SD] age 49.0 [19.1] years; 195 [52.7%] women), 54 (15.14%) did not survive to hospital discharge. Five covariates, measured at the time of admission, were independent predictors of in-hospital mortality: age in years (odds ratio [OR], 1.05; 95% CI, 1.02-1.07), body surface area (BSA) in percentage of epidermal detachment (OR, 1.02; 95% CI, 1.01-1.04), serum bicarbonate level below 20 mmol/L (OR, 2.90; 95% CI, 1.43-5.88), active cancer (OR, 4.40; 95% CI, 1.82-10.61), and dialysis prior to admission (OR, 15.94; 95% CI, 3.38-66.30). A severity-of-illness score was calculated by taking the sum of 1 point each for age 50 years or older, epidermal detachment greater than 10% of BSA, and serum bicarbonate level below 20 mmol/L; 2 points for the presence of active cancer; and 3 points for dialysis prior to admission. The score was named ABCD-10 (age, bicarbonate, cancer, dialysis, 10% BSA). The ABCD-10 model showed good discrimination (AUC, 0.816; 95% CI, 0.759-0.872) and calibration (Hosmer-Lemeshow goodness of fit test, P = .30). For SCORTEN, on admission, the AUC was 0.827 (95% CI, 0.774-0.879) and was not significantly different from that of the ABCD-10 model (P = .72). Conclusions and Relevance: In this cohort of patients with SJS/TEN, ABCD-10 accurately predicted in-hospital mortality, with discrimination that was not significantly different from SCORTEN. Additional research is needed to validate ABCD-10 in other populations. Future use of a new mortality prediction model may provide improved prognostic information for contemporary patients, including those enrolled in observational studies and therapeutic trials.

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Multicenter Retrospective Study of 377 Adult Patients from the United States.

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare, severe mucocutaneous reaction with few large cohorts reported. This multicenter retrospective study included patients with SJS/TEN seen by inpatient consultative dermatologists at 18 academic medical centers in the United States. A total of 377 adult patients with SJS/TEN between January 1, 2000 and June 1, 2015 were entered, including 260 of 377 (69%) from 2010 onward. The most frequent cause of SJS/TEN was medication reaction in 338 of 377 (89.7%), most often to trimethoprim/sulfamethoxazole (89/338; 26.3%). Most patients were managed in an intensive care (100/368; 27.2%) or burn unit (151/368; 41.0%). Most received pharmacologic therapy (266/376; 70.7%) versus supportive care alone (110/376; 29.3%)-typically corticosteroids (113/266; 42.5%), intravenous immunoglobulin (94/266; 35.3%), or both therapies (54/266; 20.3%). Based on day 1 SCORTEN predicted mortality, approximately 78 in-hospital deaths were expected (77.7/368; 21%), but the observed mortality of 54 patients (54/368; 14.7%) was significantly lower (standardized mortality ratio = 0.70; 95% confidence interval = 0.58-0.79). Stratified by therapy received, the standardized mortality ratio was lowest among those receiving both steroids and intravenous immunoglobulin (standardized mortality ratio = 0.52; 95% confidence interval 0.21-0.79). This large cohort provides contemporary information regarding US patients with SJS/TEN. Mortality, although substantial, was significantly lower than predicted. Although the precise role of pharmacotherapy remains unclear, co-administration of corticosteroids and intravenous immunoglobulin, among other therapies, may warrant further study.

A pilot study evaluating biomarker development for drug-induced chronic eczematous eruptions of aging individuals.

BACKGROUND: Identifying the drug(s) responsible for drug-induced chronic eczematous eruptions of aging individuals (CEEA) is a clinical challenge in patients on multiple medications. Reliable in vitro testing methods and biomarkers are needed to identify the causative agent and allow simultaneous assessment of T-cell responses to multiple drugs being taken concurrently. This study examined the feasibility of using in vitro, drug-specific T cell activation responses as a biomarker for drug-induced CEEA. METHODS: This was a single center, proof-of-concept pilot study at the University of Utah Hospital, Salt Lake City, Utah. Eight aging study subjects having a history suggestive of chronic eczematous drug eruptions suspected to have resulted from calcium channel blocker (CCB) and/or hydrochlorothiazide (HCTZ) hypersensitivity plus three matched aging control subjects were identified. Drug patch testing for CCB and/or HCTZ, in vitro drug antigen-induced lymphocyte proliferation assays, and multianalyte-determined cytokine release assays were performed before and after HCTZ and/or CCB incubation. RESULTS: All study and control subject blood samples tested failed to demonstrate detectable enhanced lymphocyte proliferation or cytokine release to in vitro CCBs or HCTZ challenge when tested with a fairly wide range of drug concentrations. Additionally, none of the enrolled patients developed a positive patch test to CCBs and/or HCTZ. CONCLUSIONS: This pilot study aimed to correlate in vitro drug-induced T lymphocyte transformation and cytokine production with the presence of drug-induced CEEA. Failure to identify T cell proliferative responses to CCB drug antigens in our in vitro studies could have, in part, resulted from a pharmacologic inhibiting effect of CCB on T cell activation.

Safety of non-ablative fractional laser for acne scars within 1 month after treatment with oral isotretinoin: A randomized split-face controlled trial.

BACKGROUND AND OBJECTIVE: Based on reports of poor wound healing and scarring, it is currently recommended that patients wait 6 months after completion of oral isotretinoin therapy before the safe initiation of laser treatment. Our aim was to evaluate the safety of non-ablative fractional laser (NAFL) treatment for acne scars within 1 month after isotretinoin therapy. STUDY DESIGN/METHODS: This was a randomized split-face controlled trial involving 10 patients with acne scars who had completed isotretinoin treatment. All patients received three treatments each spaced 4 weeks apart with an erbium-doped 1550 nm NAFL on one side of the face within 1 month after isotretinoin therapy. The untreated side acted as a control. Wound healing and adverse effects as well as acne scar improvement were evaluated by two blinded dermatologists. RESULTS: All patients demonstrated normal wound healing post NAFL treatments, and neither hypertrophic scars nor keloids were observed. Acne scar improvement was satisfactory. CONCLUSION: NAFL treatment for acne scarring appears to be well tolerated within 1 month of completing isotretinoin treatment. Dermatologists should reevaluate the current recommendation to wait 6 months after isotretinoin treatment for acne scar revision with lasers. Other larger studies are necessary to further challenge this dogma. Lasers Surg. Med. 49:886-890, 2017. © 2017 Wiley Periodicals, Inc.

Fractional carbon dioxide laser ablation for the treatment of microcystic lymphatic malformations (lymphangioma circumscriptum) in an adult patient with Klippel-Trenaunay syndrome.

BACKGROUND: Lymphangioma circumscriptum (LC) is a rare, benign, vascular malformation of dilated superficial lymphatic channels. Treatment is typically undertaken for cosmetic reasons or complications such as fluid drainage, pain, and infection risk that can negatively impact quality of life. Management of LC remains challenging because of high recurrence rates, regardless of the treatment modality chosen. OBJECTIVE: Treatment of LC with the fully-ablative carbon dioxide (CO2 ) laser has been reported widely, however, treatment with the fractionated CO2 laser has been limited to one case in the pediatric population. Our objective was to test the usefulness of the fractionated 10,600-nm CO2 laser in the management of symptomatic LC in an adult. RESULTS: We report a case of a 27-year-old female with Klippel-Trenaunay Syndrome (KTS) who presented with a 5-year history of well-circumscribed lesions on the right lateral and anterior thigh that drained clear lymphatic and serosanguineous fluid, respectively. These lesions caused her significant emotional distress because of extensive drainage. After only one treatment utilizing the fractionated 10,600-nm CO2 laser, the patient had improvement with cessation of fluid drainage, and the lesions are still asymptomatic 6 months since initial treatment. CONCLUSION: The favorable results observed in our patient suggest that fractionated CO2 laser may be an additional well-tolerated, low downtime option for palliation of symptoms in patients with LC. We recognize that short-term follow-up cannot conclusively demonstrate effectiveness and durability of this treatment and that, given the high risk of recurrence, a longer-term observation is needed before making definite conclusions. Lasers Surg. Med. 47:539-541, 2015. © 2015 Wiley Periodicals, Inc.

A review of graft versus host disease.

Children undergoing hematopoietic stem cell transplant often require intensive care support due to their underlying disease, sepsis, infection, hemorrhage, respiratory failure and organ dysfunction. The majority of children requiring intensive care support have an allogeneic donor. These children carry a higher likelihood of graft versus host disease complicating their medical management. Understanding the process of graft versus host disease is important in the shared care of these children between pediatric intensive care physicians and the bone marrow transplant team.

Chronic eczematous eruptions in the aging: further support for an association with exposure to calcium channel blockers.

IMPORTANCE: Dermatologists frequently encounter patients of advanced age presenting with chronic eczematous eruptions of uncertain etiology. When a drug-induced cutaneous eruption is suspected, identifying the responsible drug(s) is a complex clinical challenge. OBJECTIVE: To determine whether certain drug classes, and in particular calcium channel blockers, are associated with chronic eczematous eruptions in the aging (CEEA) in the United States. DESIGN: Retrospective case-control study. SETTING: Ambulatory patients from the Department of Dermatology, University of Utah School of Medicine, Salt Lake City. PATIENTS: The cases consisted of 94 patients 50 years and older presenting with otherwise unexplainable symmetrical eczematous eruptions of at least 2 months' duration between January 1, 2005, and December 31, 2011. Inclusion criteria also included histopathologic changes of spongiotic and/or interface dermatitis and clinical suspicion for a drug-induced cutaneous eruption. The controls consisted of 132 age-, sex-, and race-matched patients presenting with benign dermatologic conditions. A subgroup analysis on cases whose skin biopsy specimens showed a pattern of inflammation that is conventionally thought to be associated with eczematous drug eruptions (ie, eczematous and interface dermatitis) was also performed. MAIN OUTCOMES AND MEASURES: Specific drug classes associated with otherwise unexplainable CEEA. RESULTS: A statistically significant difference in drug class use between cases and controls for calcium channel blockers and thiazides was noted. For calcium channel blockers and thiazides, the matched odds ratios were 4.21 (95% CI, 1.77-9.97; P = .001) and 2.07 (95% CI, 1.08-3.96; P = .03) respectively. The histopathological pattern subgroup analysis failed to show any statistically significant associations. CONCLUSIONS: The findings of this study further support an association of calcium channel blockers, as well as thiazides, with CEEA in the United States.

Porokeratotic eccrine ostial and dermal duct nevus treated with a combination erbium/CO2 laser: a case and brief review.

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is an uncommon disease that presents early in childhood and is characterized by keratotic papules, often in a linear configuration. We describe a 12-year-old girl with characteristic lesions of PEODDN and describe her response to treatment with a combination CO2/Erbium laser. We also briefly review the literature on PEODDN.

A unique presentation of an Epstein-Barr virus-associated natural killer/T-cell lymphoproliferative disorder in a white male adolescent.

BACKGROUND: Extranasal natural killer (NK)/T-cell lymphoma and aggressive NK-cell leukemia are strongly associated with Epstein-Barr virus (EBV) and most often occur in middle-aged individuals. Overlap between these 2 diagnoses is rare. In addition, pathologic findings for these 2 diagnoses are typically notable for necrosis, apoptosis, angioinvasion, and angiodestruction. OBSERVATIONS: We describe a 15-year-old male adolescent who had painful subcutaneous nodules and plaques over his anterior thighs, shins, and lower abdomen while receiving anti-tumor necrosis factor therapy with infliximab. He also was noted to have pulmonary nodules, liver nodules, hepatosplenomegaly, thrombocytopenia, and transaminitis. A skin biopsy revealed atypical small to intermediate-sized EBV-positive lymphoid cells of NK-cell origin infiltrating the subcutaneous adipose tissue, mimicking subcutaneous T-cell lymphoma. Similar atypical EBV-positive lymphocytes were noted in the bone marrow, liver, stomach, and colon. This patient had a rapidly fatal disease course. CONCLUSIONS: We report a unique clinical and histological presentation most consistent with an extranasal NK/T-cell lymphoma and aggressive NK-cell leukemia overlap, although our case may represent a disease entity completely new to the literature. In addition, we report the first case to our knowledge of an EBV-positive NK/T-cell lymphoma developing in the setting of tumor necrosis factor inhibitor therapy.

Primary localized cutaneous nodular amyloidosis and CREST syndrome: a case report and review of the literature.

Primary localized cutaneous nodular amyloidosis (PLCNA) is a form of primary localized cutaneous amyloidosis (PLCA) that presents as yellowish waxy nodules on the extremities, face, trunk, or genitalia. We report the case of a patient with PLCNA and CREST (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. A diagnosis of her extensive PLCNA was made after biopsy specimens from the bilateral shins stained positive for amyloid extending from the superficial papillary dermis to the subcutis. Results of a workup were negative for paraproteinemia or signs of systemic amyloidosis and have remained so after 8 years of follow-up. We present a review of the literature describing the presentation and histopathology of the varying forms of amyloidosis.