McEvoy, Marian T.

AbstractBackgroundAcute generalized exanthematous pustulosis (AGEP) is a rare skin condition typically caused by medications. The objective of this study was to examine the clinical features, causes, and outcomes of AGEP at a sole tertiary care center.MethodsA retrospective review of patients with AGEP (European Study of Severe Cutaneous Adverse Reactions score of ≥ 5) seen at Mayo Clinic (Rochester, MN, USA) between January 1, 1996, and December 31, 2013, was conducted.ResultsOf 28 patients (mean age at onset: 56 years), 17 (61%) were women. The development of AGEP was attributed to medications in 25 patients (89%), with clindamycin the most common culprit (six patients). Three patients (11%) had mucous membrane involvement, and 21 (75%) showed systemic involvement. Ten patients (36%) received systemic corticosteroids for treatment of AGEP. Skin findings resolved within 15 days in 26 patients (93%) (mean time to resolution: 7.6 days). In three patients (11%), generalized skin eruptions or dermatitis developed weeks to months after the resolution of AGEP. Twenty‐four patients (86%) had a personal history of drug reactions before the development of AGEP.ConclusionsA previous history of drug reactions and clindamycin causation were more common in the present cohort than in prior reports. A small subset of patients experienced new‐onset non‐AGEP skin eruptions within a few months of the resolution of AGEP.

AbstractBackground: Penile and scrotal swelling can occur as an extraintestinal manifestation of Crohn's disease (CD) and is thought to be an uncommon form of metastatic CD (MCD). Because of the rarity of this manifestation, much is unknown concerning the presentation, treatment, and response to therapy in children with genital MCD. Methods: Boys ages 1 to 17 years presenting with genital edema and a confirmed diagnosis or strong suspicion of CD who were evaluated at the Mayo Clinic between 1996 and 2014 were included for review. We sought to characterize the clinical and pathologic features of genital MCD and response to treatment in our cohort of patients. Results: Eight patients with genital MCD were identified from our institution (mean age 11.4 yrs, range 7–16yrs). Seven (88%) patients experienced cutaneous symptoms before a formal diagnosis of CD was made, and two of the seven had no adverse gastrointestinal symptoms at that time. Patients were prescribed an average of 3.4 medications (topical and systemic) for management of their gastrointestinal CD and MCD. Conclusions: Penile and scrotal swelling can occur as an extraintestinal manifestation of CD and is thought to be an uncommon form of MCD. In boys, genital swelling typically precedes intestinal CD. Treatment of the underlying CD with systemic medications was most helpful in this series. An evaluation for CD is necessary in all patients presenting with unexplained genital swelling.

AbstractBackgroundThe pemphigus group is characterized by the presence of circulating immunoglobulins against desmosomes. IgG/IgA pemphigus is defined by the presence of IgG and IgA cell surface deposits upon direct immunofluorescence (DIF) and/or circulating IgG and IgA autoantibodies upon indirect immunofluorescence. Previous reports of patients with IgG/IgA pemphigus are sparse. Whether IgG/IgA pemphigus is best classified as a subtype of IgG (classic) pemphigus or IgA pemphigus, or as a distinct entity, has yet to be determined.ObjectivesWe compared the features of patients with IgG/IgA pemphigus to those of IgG pemphigus and IgA pemphigus.MethodsRetrospective clinicopathologic study of patients with IgG, IgG/IgA, and IgA pemphigus evaluated at our clinic (1993–2013).ResultsWe included 26, 13, and seven patients with IgG, IgG/IgA, and IgA pemphigus, respectively. Patients with IgG/IgA pemphigus did not differ significantly from patients with IgG pemphigus in terms of clinical and microscopic features, DIF findings, anti‐desmoglein antibody values, and treatments required. However, patients with IgG/IgA pemphigus were significantly different from patients with IgA pemphigus regarding intertriginous distribution (P = 0.038) and pustular lesions (P < 0.001), acantholysis (P = 0.043), and presence of intercellular C3 deposits on DIF (P < 0.001).ConclusionComparative clinicopathologic data imply that IgG/IgA pemphigus may best be regarded as a variant of IgG pemphigus and distinct from IgA pemphigus.

Summary Background/Purpose Ultraviolet A1 ( UVA 1) phototherapy has been used for over 15 years in the United States, primarily for the treatment of localized sclerosis and various sclerosing disorders. The objective was to describe use of UVA 1 for dermatoses beyond localized sclerosis at two academic institutions. Methods Data from 83 patients treated with low‐ (20–40 J/cm 2 ), medium‐ (>40–80 J/cm 2 ), and high‐ (>80–120 J/cm 2 ) dose UVA 1 phototherapy was retrospectively analyzed. The mean individual treatment dose (J/cm 2 ), the mean number of sessions, and the mean total dose (J/cm 2 ) were evaluated. Effectiveness was assessed by reviewing clinical examination notes from office visits. Results Good therapeutic efficacy was seen in patients with systemic sclerosis ( SS , 16 patients), graft‐versus‐host disease ( GVHD , 25 patients), and nephrogenic systemic fibrosis ( NSF , 17 patients). A statistically significant a dose–response association was observed in the cases of SS , GVHD and NSF . Likelihood of clinical improvement from UVA 1 phototherapy was very likely for medium‐ and high‐dose regimens in SS , while this level of improvement was only observed in GVHD and NSF patients receiving high‐dose UVA 1. Conclusion UVA 1 phototherapy is effective and safe in the treatment of GVHD , NSF , SS , and mast cell disorders. High‐dose regimens appear to be more effective than medium‐ and low‐dose regimens for NSF and GVHD , while medium‐ and high‐dose regimens outperform low‐dose UVA 1 in SS .

AbstractBackgroundIntensive treatment options are lacking for patients with severe skin disease recalcitrant to outpatient therapy. The availability of inpatient dermatology care has almost disappeared in many parts of the United States. One possible solution for this is a day treatment center for patients with severe dermatologic disease recalcitrant to outpatient therapy.MethodsDescriptive study based on retrospective medical record review of all patients attending the day hospital for treatment in 2010. We collected data on patient demographics, distribution of admission diagnosis, treatments used, and length of stay.ResultsA total of 211 patients had 235 admissions. Mean age was 57.7 years (range 3.8–92.1 years). The most common indications for admission were dermatitis (139 admissions [59.2%]), psoriasis (58 [24.7%]), and mycosis fungoides (eight [3.4%]). The main treatment interventions were wet dressings (195 admissions [83.0%]) and Goeckerman treatment (38 [16.2%]). The median number of days of treatment was three (interquartile range, 2–5 days) for wet dressings and 22 days (interquartile range, 21–24 days) for Goeckerman therapy.ConclusionsThe dermatology day treatment center provided intensive skin‐directed therapy and filled a critical practice gap in the management of our patients with severe widespread skin diseases recalcitrant to outpatient therapy. We propose that the dermatology day treatment center is a valuable model that could be considered by both private practitioners and academic centers in the United States as an important adjunct to fill the gap in availability of intensive topical treatments for patients with severe skin disease.

AbstractBackgroundDermatology consultations are frequently requested by inpatient hospital services. As inpatient dermatology services in the USA decline, dermatology hospital consultations are becoming increasingly important.ObjectivesWe aim to describe the spectrum of skin diseases encountered and the health care subspecialties requesting dermatology hospital consultations.MethodsWe performed a retrospective chart review of adult patient (age: ≥18 years) dermatology hospital consultations from January 1 to December 31, 2010. We examined patient demographic characteristics, consultation requesting services, and consultation diagnoses.ResultsAmong dermatology services, 614 patients had 674 separate inpatient dermatology consultations during 2010. Of these patients, 55.9% were male (mean age: 59 years). In total, 205 consultations (30.4%) were requested by the internal medicine subspecialty, 137 (20.3%) by the hematology and oncology subspecialty, and 93 (13.8%) by the surgical subspecialty. The most common conditions seen by the hospital dermatology consulting service were skin infections (n = 125, 18.5%), dermatitis (n = 120, 17.8%), drug eruptions (n = 87, 12.9%), chronic wounds and ulcers (n = 55, 8.1%), cutaneous neoplasms (n = 39, 5.8%), graft‐versus‐host disease (n = 37, 5.5%), ecchymosis, purpura simplex or petechia (n = 26, 3.8%), intertrigo (n = 21, 3.1%), and urticaria (n = 20, 3.0%).ConclusionsThe majority of consultations conducted by the dermatology hospital consulting service were for the management of common skin diseases, such as cutaneous infections, dermatitis, and drug eruptions. Most consultations were requested by the departments of internal medicine, hematology and oncology, and surgical services.

AbstractData describing the management of pediatric patients admitted to a hospital under the care of a dermatologist and dermatology hospital consults for pediatric inpatients are limited. We aim to describe the role of an inpatient hospital service jointly run by dermatology and pediatrics and the activities of a pediatric dermatology hospital consult service. We retrospectively identified pediatric (age < 18 yrs) dermatology inpatients and hospital consult patients from January 1, 2009, through December 31, 2010. We examined patient demographics, indications for admission, length of stay, treatment provided, consult‐requesting service, and consult diagnosis. One hundred eight admissions were by a dermatologist. The mean age was 5.8 years; the median length of stay was 3 days. Indications for admission included atopic dermatitis (86.1%), psoriasis (3.7%), and eczema herpeticum (2.8%). The main treatment provided was wet dressings (97.2%). Eighty‐three dermatology hospital consults were requested. The mean age was 7.4 years. The main indications for dermatology consultation included drug rash (12.1%), cutaneous infections (12.1%), contact dermatitis (9.6%), psoriasis (8.4%), atopic dermatitis (6.0%), and hemangiomas (6.0%). This study describes the utility of the hospital pediatric dermatology inpatient and consult services in treating patients with severe skin disease.

Objective. To examine the utility of the Framingham risk score (FRS) in estimating cardiovascular risk in psoriasis.Methods. We compared the predicted 10-year risk of cardiovascular events, namely, cardiovascular death, myocardial infarction, heart failure, percutaneous transluminal coronary angioplasty, and coronary artery bypass grafting using the FRS, to the observed risk of cardiovascular events in a population-based cohort of patients with psoriasis. Patients with incident or prevalent adult-onset psoriasis aged 30–79 years without prior history of cardiovascular disease were included.Results. Among the 1197 patients with predicted risk scores, the median FRS was 6.0%, while the observed 10-year cardiovascular risk was 6.9% (standardized incidence ratio (SIR): 1.14; 95% confidence interval (CI): 0.92–1.42). The SIR was not elevated for women nor for men. The differences between observed and predicted cardiovascular risks in patients <60 years (SIR: 1.01; 95% CI: 0.73–1.41) or ≥60 years (SIR: 1.26; 95% CI: 0.95–1.68) were not statistically significant.Conclusion. There was no apparent difference between observed and predicted cardiovascular risks in patients with psoriasis in our study. FRS reasonably estimated cardiovascular risk in both men and women as well as in younger and older psoriasis patients, suggesting that FRS can be used in risk stratification in psoriasis without further adjustment.