Cutaneous sarcoid-like drug reaction caused by an anaplastic lymphoma kinase inhibitor.

Anaplastic lymphoma kinase (ALK) rearranged lung cancers represent 4% to 6% of all pulmonary adenocarcinomas, and echinoderm microtubule associated protein like 4 (EML4)-ALK fusions are the most common subgroup. Herein, we report a case of two successive drug reactions due to ALK inhibitors. A 69-year-old female with stage IVB EML4-ALK fused lung adenocarcinoma developed a generalized morbilliform eruption 10 days after starting alectinib. Skin biopsy findings were consistent with a drug reaction. Her findings resolved after alectinib was discontinued. Another ALK inhibitor, lorlatinib was started and she developed multiple asymptomatic cutaneous and oral nodules 4 months later. Biopsies from these nodules showed sarcoidal granulomas without evidence of metastases or infection. ALK inhibitors are associated with numerous adverse events, including various cutaneous eruptions. However, a sarcoidal drug reaction involving the skin has not been reported. Identification of drug reactions to targeted therapy can avoid long-term sequelae and misinterpretation of the clinical findings as disease progression or infection.

Skin concerns in patients with trisomy 21 (Down syndrome): A Mayo Clinic 22-year retrospective review.

BACKGROUND/OBJECTIVES: Trisomy 21 has known associated clinical phenotypes, including skin and soft tissue concerns. However, the overall prevalence and types of findings are largely unclear. METHODS: A retrospective review of children with trisomy 21 and one or more dermatologic diagnoses, seen from 1/1/1994 to 7/1/2016, was performed to record dermatologic diagnoses. If one or more diagnoses were confirmed, further data were collected, including demographics, medical specialty, referrals to dermatology, treatment, complications, and follow-up. RESULTS: One hundred and seventy-four patients with a diagnosis of trisomy 21 aged 18 years or younger were confirmed to have one or more dermatologic diagnoses. In a total of 479 dermatologic diagnoses, superficial mycoses (12%), skin and soft tissue infections (10%), dermatitis (8%), and folliculitis (8%) were most common. Diagnoses were most commonly made as an outpatient (91%) and by general pediatrics (45%) or dermatology (25%). A significant difference (P < .05) in the frequency of various diagnoses made by different specialties was observed. A significant difference (P < .05) in the treatments recommended by different specialties was also observed. Referrals to dermatology were infrequent (6%). However, a frequent change in diagnosis (61%) or treatment (68%) for patients referred to dermatology was observed. CONCLUSIONS: Children with trisomy 21 are most commonly diagnosed with superficial mycoses, skin and soft tissue infections, dermatitis, and folliculitis. The majority of diagnoses were made by general pediatricians and although dermatology referral was rarely necessary, consultations often resulted in change of diagnosis and/or treatment, supporting consideration of early and frequent dermatology referral.

A rare case of childhood mucous membrane pemphigoid involving the oral and genital mucosa.

Mucous membrane pemphigoid (MMP) is a rare chronic immunobullous disease that involves the mucous membranes and may result in significant scarring and complications if diagnosis is delayed. MMP typically occurs in elderly patients, with very few cases reported in children. Here, we present a 12-year-old female patient with childhood-onset oral and genital MMP, clinically suspected to be lichen sclerosus, but eventually diagnosed as MMP after multiple supportive biopsies and confirmatory direct immunofluorescence. Although treatment was challenging, the combined use of systemic corticosteroids, dapsone, and mycophenolate mofetil was ultimately successful in achieving disease control.

A case of extensive acquired progressive lymphangioma.

Acquired progressive lymphangioma (benign lymphangioendothelioma) is a rare lymphatic anomaly of unclear pathogenesis. Excision is generally advised for local disease, although other therapies have been tried. This report describes a unique case of extensive acquired progressive lymphangioma involving the abdomen, genitalia, and lower extremity of a 1-year-old boy. Rapid progression and multisite involvement required exploration of nonsurgical options for management.

Older age is associated with increased early mortality after transjugular intrahepatic portosystemic shunt.

INTRODUCTION & AIM: The role of age as a predictor of mortality after transjugular intra hepatic portosystemic shunt (TIPS) is controversial. Age has been found to be an important predictor of post-TIPS mortality in some, but not all, studies and is not a component of the MELD score. The purpose of this study was to compare the 90-day survival of subjects with cirrhosis age ≥ 70 years with younger subjects undergoing TIPS. MATERIAL AND METHODS: A database of adult with cirrhosis undergoing TIPS from 2003-2011 was analyzed. The primary endpoint was survival 90-days post-TIPS. Survival was analyzed by the Kaplan-Meier method and proportional hazard modeling. RESULTS: 539 subjects met study criteria. 474 (88%) were between the ages of 24-69 and 65 (12%) were age 70-89 years. The groups were similar with respect to the indication for TIPS, mean MELD score and distribution of MELD score. Survival 90-days post-TIPS was 60% in the older cohort compared with 85% in the younger cohort (p < 0.001). Proportional hazards modeling controlled for comorbidities identified age ≥ 70 and MELD score as predictors of early post-TIPS survival. The hazard ratio associated with age increased monotonically, became significant at age ≥ 70 years (HR 3.22; 95% CI 1.81-5.74; p < 0.001) and exceeded the effect of MELD on survival. CONCLUSIONS: Age ≥ 70 was associated with reduced survival within 90 days following TIPS. The findings from this study indicate that age is a relevant consideration in assessing the early mortality risk of TIPS.

Trends in patch-test results and allergen changes in the standard series: a Mayo Clinic 5-year retrospective review (January 1, 2006, to December 31, 2010).

BACKGROUND: Patch testing is essential for identification of culprits causing allergic contact dermatitis. OBJECTIVE: We sought to identify trends and allergen changes in our standard series during 2006 to 2010, compared with our previous report (2001-2005). METHODS: We conducted a retrospective review of patch-test results. RESULTS: A total of 3115 patients were tested with a mean of 73.0 allergens. Since our prior report, 8 allergens were added to the standard series; 14 were deleted. Significantly higher rates of allergic positive reaction were documented for carba mix, 3%, and Disperse Orange 3, 1%. Rates were lower for 10 allergens: neomycin sulfate, 20%; gold sodium thiosulfate, 0.5%; hexahydro-1,3,5-tris(2-hydroxyethyl)triazine, 1%; disperse blue 124, 1%; disperse blue 106, 1%; diazolidinyl urea, 1%; hexylresorcinol, 0.25%; diazolidinyl urea, 1% aqueous; 2-bromo-2-nitropropane-1,3-diol, 0.25%; and lidocaine, 5%. Many final patch-test readings for many allergens were categorized as mild reactions (erythema only). Overall allergenicity and irritancy rates declined significantly since our prior report. Results were generally comparable with those in a North American Contact Dermatitis Group report from 2005 to 2006. LIMITATIONS: This was a retrospective study; there is a lack of long-term follow-up. CONCLUSIONS: Since our previous report, our standard series composition has changed, and overall rates of allergenicity and irritancy have decreased. Notably, many final patch-test readings showed mild reactions.

Constraints to caring: service to medically indigent patients by allopathic and osteopathic physicians.

Most U.S. physicians limit their care of medically indigent populations, rendering millions of patients underserved. Using survey data from a national sample of 669 allopathic and osteopathic physicians, this paper explores the professional, demographic, and attitudinal correlates of care of medically indigent (Medicaid and uninsured) patients. Separate bivariate and multivariate data analyses for allopathic and osteopathic physicians generate path models of the influences of physician characteristics on care of indigent patients. For both physician groups, professional characteristics such as practice type, location, medical specialty, and perceptions of the medical system shape care of indigent patients. Differential patterns of association emerge, with care provided by osteopathic physicians most significantly influenced by private practice, while medical specialty and employment in community clinics shapes indigent care provided by allopathic physicians. Results highlight the utility of comparative analyses in discerning the mechanisms through which professional characteristics influence indigent care by diverse health providers.

Treatment of severe drug reactions by hemodialysis.

BACKGROUND: Extracorporeal treatments such as hemodialysis and plasma exchange are lifesaving measures in the treatment of drug poisoning. This treatment method generally is not used for severe cutaneous and systemic drug reactions. METHODS: Here, we describe three cases wherein hemodialysis therapy was instrumental in reversing the adverse drug reaction. RESULTS: In the cases of severe cutaneous drug reactions reviewed, patients presented with linear immunoglobulin A bullous dermatosis, acute generalized exanthematous pustulosis, and toxic epidermal necrolysis. Salvage treatment with hemodialysis therapy drastically influenced the course of disease, resulting in remission. CONCLUSIONS: This novel and highly effective treatment option is not considered in current algorithms for adverse drug reactions. Hence, in addition to the rarity of these reactions, the main limitation of the study is the small number of patients. Hemodialysis can substantially alter the prognosis and, in some cases, be a lifesaving treatment for patients with severe adverse cutaneous drug reaction associated with systemic toxicity.

Benzalkonium Chloride: A Known Irritant and Novel Allergen.

BACKGROUND: Benzalkonium chloride (BAK), a detergent and preservative found in health care and household products, is an established irritant, yet BAK is seldom considered to cause allergic contact dermatitis. We have, however, observed positive patch test reactions more often than is typically reported. From 2001 through 2005 and 2006 through 2010, BAK was among the top 10 most frequent allergens in our standard series. OBJECTIVE: The aim of this study was to review the Mayo Clinic experience from 2000 to 2012 with patch testing to BAK. METHODS: An electronic patch test database was used to acquire results of patients who underwent patch testing for BAK 0.1% aqueous after it was introduced to the standard series in 2000 until 2012. Previous reports (1998-2000, 2001-2005, 2006-2010) from our institution were also reviewed. CONCLUSIONS: Our study showed BAK to be an allergen of increasing importance. From 1998 through 2000, 2001 through 2005, and 2006 through 2010, the rate of allergic patch test results to BAK increased. More than half of the reactions in each period studied were graded as macular erythema, with at least one third of all reactions deemed to be relevant. Irritancy rates were consistently low.

Patch testing with the standard series when receiving immunosuppressive medications.

BACKGROUND: Debate surrounds the validity of patch testing for allergic contact dermatitis in patients taking immunosuppressants and the extent to which these medications suppress patch test reactions. Previous studies have demonstrated that corticosteroids can suppress immune reactions measured in patch testing. Because immunomodulators are increasingly used, their impact on patch testing also requires evaluation. OBJECTIVES: This study aimed to report patch test results in patients taking specific immunosuppressants and to review relevant literature. METHODS: A retrospective review of patients patch tested with the standard series at Mayo Clinic in Rochester, Minnesota, between January 1, 2008, and December 31, 2011, was conducted. CONCLUSIONS: Positive patch test results occur during methotrexate therapy. How reactions would differ with no immunosuppression is unclear.

Increased incidence of cutaneous nontuberculous mycobacterial infection, 1980 to 2009: a population-based study.

OBJECTIVES: To determine the incidence and clinical characteristics of cutaneous nontuberculous mycobacterial (NTM) infection during the past 30 years and whether the predominant species have changed. PATIENTS AND METHODS: Using Rochester Epidemiology Project data, we identified Olmsted County, Minnesota, residents with cutaneous NTM infections between January 1, 1980, and December 31, 2009, examining the incidence of infection, patient demographic and clinical features, the mycobacterium species, and therapy. RESULTS: Forty patients (median age, 47 years; 58% female [23 of 40]) had positive NTM cultures plus 1 or more clinical signs. The overall age- and sex-adjusted incidence of cutaneous NTM infection was 1.3 per 100,000 person-years (95% CI, 0.9-1.7 per 100,000 person-years). The incidence increased with age at diagnosis (P=.003) and was higher in 2000 to 2009 (2.0 per 100,000 person-years; 95% CI, 1.3-2.8 per 100,000 person-years) than in 1980 to 1999 (0.7 per 100,000 person-years; 95% CI, 0.3-1.1 per 100,000 person-years) (P=.002). The distal extremities were the most common sites of infection (27 of 39 patients [69%]). No patient had human immunodeficiency virus infection, but 23% (9 of 39) were immunosuppressed. Of the identifiable causes, traumatic injuries were the most frequent (22 of 29 patients [76%]). The most common species were Mycobacterium marinum (17 of 38 patients [45%]) and Mycobacterium chelonae/Mycobacterium abscessus (12 of 38 patients [32%]). In the past decade (2000-2009), 15 of 24 species (63%) were rapidly growing mycobacteria compared with only 4 of 14 species (29%) earlier (1980-1999) (P=.04). CONCLUSION: The incidence of cutaneous NTM infection increased nearly 3-fold during the study period. Rapidly growing mycobacteria were predominant during the past decade.

Patch testing with textile allergens: the mayo clinic experience.

BACKGROUND: Recognition of allergic contact dermatitis attributed to textile dyes and resins is steadily increasing. OBJECTIVE: This study aims to review the results of patch testing with a textile series at our institution and to compare with previously published reports. METHODS: We performed a retrospective review of results in patients who underwent patch testing using a series of textile dyes and resins from January 1, 2000, through September 30, 2011. RESULTS: A total of 671 patients (mean age, 56.5 years; female, 65.9%) were patch tested with the textile series (42 dyes and resins). These patients were also generally tested with the standard patch test series (n = 620). Of the patients, 219 (32.6%) demonstrated allergic reaction to 1 or more textile dyes and resins, and 71 (10.6%) manifested irritant reactions. The most frequent allergens were disperse blue 106 1% (8.3%), disperse blue 124 1% (8.0%), and melamine formaldehyde 7% (8.0%). Of patients tested with the standard series, 36 (5.8%) showed a positive reaction to the traditional textile screening allergen p-phenylenediamine 1%. CONCLUSIONS: Supplementing the standard series with the textile series increased detection of textile allergies. In patients suspected of textile allergy, addition of the textile series is necessary for appropriate diagnosis.

Two years after a quality improvement intervention for chronic kidney disease care in a primary care office.

Implementation of evidence-based Kidney Disease Outcomes Quality Initiative (KDOQI) guidelines is of increasing clinical importance. This study evaluates the long-term impact of a chronic kidney disease (CKD) quality improvement (QI) project. A retrospective chart review was performed at a family practice that completed a QI project 2 years previously. In a study population of 195 patients, CKD recognition decreased during the maintenance period from 70% to 60.8% (P = 1.98), from a baseline of 38.1%, and anemia recognition declined from 70% to 50% (P =.132), from a baseline of 35%. Evaluation for parathyroid hormone, vitamin D, and phosphate decreased from 44% to 33% (P =.216), from a baseline of 4.8%. Referrals to nephrologists decreased from 77% to 61% (P = .369), from a baseline of 14%. The decrement in KDOQI guideline compliance during the maintenance period was not statistically significant, nor was there a return to baseline values. This suggests that the intervention provided the education and reinforcement necessary to effect long-term change.