Clinical features, causes, treatments, and outcomes of peristomal pyoderma gangrenosum (PPG) in 44 patients: The Mayo Clinic experience, 1996 through 2013.

BACKGROUND: There are limited large case series of peristomal pyoderma gangrenosum (PPG), an uncommon cause of recalcitrant peristomal ulceration. OBJECTIVE: We sought to further characterize the clinical features, causes, treatments, and outcomes of PPG. METHODS: We conducted a retrospective chart review of patients with PPG seen at Mayo Clinic from January 1996 to July 2013. RESULTS: A total of 44 patients had PPG (mean age, 46 years; 32 women [73%]); 41 (93%) had inflammatory bowel disease. Mean time to PPG onset after stoma surgery was 5.2 months (excluding 1 outlier). Systemic therapies included corticosteroids (66%), immunosuppressants (41%), biologics (36%), and a combination of systemic treatments (36%). Mean time to reach a complete response was 10.7 weeks. Stoma closure had the greatest complete response (4 of 4 patients, no recurrences). Recurrence after any treatment was documented in 23 of 38 (61%) patients. Stoma relocation/revision recurred in 10 of 15 (67%) patients. Remission occurred in 29 of 31 (94%) patients. LIMITATIONS: Small sample size and retrospective study design are limitations. CONCLUSION: PPG is strongly associated with inflammatory bowel disease, is predominant in women, and has a prolonged time to onset and high recurrence rate. Systemic corticosteroid or combination therapies and surgical closure can be effective treatments. Timely recognition and management are paramount to achieving early remission.

Penile and Scrotal Swelling: An Underrecognized Presentation of Crohn's Disease.

BACKGROUND: 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 7-16 yrs). 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.

Cutaneous microemboli from hydrophilic polymer after endovascular procedures.

BACKGROUND: Multiple devices and coatings assist with endovascular insertion of sheaths, catheters, and guide wires. Hydrophilic polymer coatings, a common component of endovascular surgical devices, reportedly cause microvascular obstruction and embolization, with various sequelae in organs and soft tissue. OBJECTIVE: We sought to describe clinical and histopathologic features of cutaneous manifestations of hydrophilic polymer gel emboli. METHODS: We evaluated the clinical and histopathologic characteristics of 8 patients with cutaneous complications of hydrophilic polymer gel emboli who presented in May 2013 through February 2015. RESULTS: Sudden onset of lower extremity livedo racemosa, purpuric patches, or both, occurred hours to days after endovascular procedures involving the aorta. Histopathologic evaluation showed basophilic lamellated material, consistent with hydrophilic polymer gel emboli, within small dermal vessels. LIMITATIONS: This was a retrospective study with small sample size and not controlled for all similar procedures in this population. CONCLUSION: Hydrophilic polymer gel coatings in endovascular devices can embolize to skin and cause microvascular occlusion, presenting as livedo racemosa, purpura, or both. Given the number of patients observed over a short period, this phenomenon may be underappreciated. Hydrophilic polymer gel emboli should be considered in differential diagnosis of livedo racemosa and purpura after endovascular procedure.

Postoperative pyoderma gangrenosum (PG): the Mayo Clinic experience of 20 years from 1994 through 2014.

BACKGROUND: Postoperative pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by the development of PG-type lesions within surgical sites. OBJECTIVE: We sought to characterize postoperative PG as a distinct subtype of PG for earlier recognition and prevention of improper therapy. METHODS: We conducted a retrospective chart review of patients with nonperistomal postoperative PG at Mayo Clinic from 1994 to 2014.x RESULTS: Eighteen patients had postoperative PG with an average age of 58 years. Fifteen (83%) were female. Among patients with postoperative PG, 4 (22%) had an associated systemic disease traditionally associated with PG. Sites of postoperative PG included 7 breast (38%), 7 abdomen (38%), 1 back, 1 shoulder, 1 ankle, and 1 scrotum, witxxh breast reconstruction being the most common surgery. The average time to symptoms was 11 days. No patients had a fever. Eight (44%) had documented anemia and 5 (27%) had leukocytosis. Antibiotics and systemic corticosteroids were initiated in 10 (56%) and 14 (83%), respectively. Debridement was done in 11 (61%) patients. LIMITATIONS: Small sample size and retrospective study are limitations. CONCLUSION: Postoperative PG is a rare surgical complication with predilection for the breast and abdomen of females and has less association with systemic disease than idiopathic PG. Early recognition may prevent unnecessary debridements and morbidity.

Diffuse dermal angiomatosis of the breast: clinicopathologic study of 5 patients.

BACKGROUND: Diffuse dermal angiomatosis (DDA) is a rare skin condition considered to be a type of reactive angioendotheliomatosis. Histologic features are quite characteristic. It has been reported in association with vaso-occlusive disease, trauma, or underlying hypercoagulability. In the past, it was thought to be most common on the lower extremities. OBJECTIVE: The purpose of this study was to describe the clinical and histologic features of 5 patients with DDA. METHODS: The clinical and histologic features of 5 patients with DDA were evaluated. RESULTS: Five women (47-58 years old) had DDA of the breast. Histologic examination showed a diffuse proliferation of benign endothelial cells between the collagen bundles throughout the dermis. LIMITATIONS: The main limitation of our study is the limited number of patients. CONCLUSION: Involvement of the breast is much more common than previously reported. Smoking seems to be a strong risk factor for the disease. Revascularization, oral corticosteroids, and oral anticoagulation have all been reported to be somewhat successful in the treatment of DDA of the breast.

Bullous dermatitis artefacta.

Bullous artefactual dermatoses are rare and may be induced by various techniques, including chemicals, heat, or electrical current. Proving a factitial etiology and identifying the mechanism of injury may be difficult. We describe the clinical features and histopathology of 2 patients with bullous disease induced by electrical current or heat. Physical examination in both patients demonstrated geometrically shaped tense bullae. Skin biopsies revealed epidermal necrosis overlying a pauci-inflammatory subepidermal cleft, with homogenization of underlying superficial dermal collagen. In 1 of the 2 patients, there was prominent vertical elongation of keratinocyte nuclei and also of cytoplasmic processes. Direct immunofluorescence study of skin plus testing of serum by indirect immunofluorescence and enzyme-linked immunosorbent assay for BP180 and BP230 antibodies revealed no evidence for immunobullous disease in either patient. Vertical elongation of keratinocyte nuclei, often attributed to a polarization effect of electrical current, is characteristic of electrical burn but also may be induced by thermal injury. These 2 patients highlight the importance of histopathology in confirming a diagnosis of bullous dermatitis artefacta.

Pemphigus vegetans variant of IgA pemphigus, a variant of IgA pemphigus and other autoimmune blistering disorders.

Pyodermatitis-pyostomatitis vegetans (PPV) constitutes an inflammatory mucocutaneous dermatosis that is associated with inflammatory bowel disease. Clinically, PPV appears as pustules on mucosal surfaces and as vegetating exudative plaques on intertriginous surfaces. It is typically a clinical diagnosis supported by histological findings. Microscopic findings include epidermal hyperplasia, focal acantholysis, and a dense mixed inflammatory infiltrate with intraepithelial and subepithelial eosinophilic microabscesses. In the recent literature, immunofluorescence has been thought to be negative in PPV or, if positive, an aberrant finding. Herein, we report 2 cases of PPV associated with inflammatory bowel disease, which display intercellular IgA deposits. Although these cases may represent isolated epiphenomena, it is possible that the paucity of PPV cases with immunofluorescent studies hitherto has led to an oversight of an interesting association between intercellular IgA and PPV.

Pediatric hospital dermatology: experience with inpatient and consult services at the Mayo Clinic.

Data 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.

Wet dressing therapy in conjunction with topical corticosteroids is effective for rapid control of severe pediatric atopic dermatitis: experience with 218 patients over 30 years at Mayo Clinic.

BACKGROUND: At our institution, hospitalization for intensive treatment (combining wet dressings and topical corticosteroids) is a primary intervention for severe pediatric atopic dermatitis. Prior reports of this treatment are limited. OBJECTIVE: We sought to review the efficacy of wet dressings for pediatric atopic dermatitis. METHODS: We reviewed records of pediatric patients hospitalized from January 1, 1980, through April 20, 2010, who received intensive topical treatments for atopic dermatitis. RESULTS: In total, 218 pediatric patients had widespread atopic dermatitis severe enough to warrant hospitalization, despite prior outpatient topical treatments and other interventions such as immunomodulating agents, phototherapy, dietary manipulation, or contact allergen avoidance. Mean (SD) age was 5.97 (4.91) years (range, 2 months-17 years); 141 patients (65%) were female. There were 266 hospitalizations: 192 patients (72%) had one admission, 15 (6%) had two admissions, and 11 (4%) had 3 or more admissions. Mean (SD) duration of hospitalization was 3.61 (2.23) days (range, 1-16 days). Upon discharge, all patients showed improvement. In 239 of 266 hospitalizations, patient records showed quantification of improvement (global assessment): 121 (45%) had 75% to 100% improvement, 102 (38%) had 50% to 75% improvement, and 16 (6%) had 25% to 50% improvement. LIMITATIONS: This was a retrospective study. CONCLUSION: Intensive inpatient treatment (with wet dressings and topical corticosteroids) was highly effective in controlling severe and recalcitrant atopic dermatitis. Intensive topical treatment, although underused, is an effective first-line approach for patients with severe atopic dermatitis.

Trends in lipid profiles in patients with psoriasis: a population-based analysis.

BACKGROUND: Psoriasis is associated with an atherogenic lipid profile but longitudinal changes in lipids around disease onset are unknown. The purpose of our study is to examine the effect of psoriasis onset on serum lipid profiles. METHODS: We compared changes in lipid profiles in a population based incident cohort of 689 patients with psoriasis and 717 non-psoriasis subjects. All lipid measures performed 5 years before and after psoriasis incidence/index date were abstracted. Random-effects models adjusting for age, sex and calendar year were used to examine trends in lipid profiles. RESULTS: There were significant declines in total cholesterol (TC) and low-density lipoprotein (LDL) levels during the 5 years before and after psoriasis incidence/index date in both the psoriasis and the non-psoriasis cohorts, with a greater decrease noted in the TC levels (p=0.022) and LDL (p=0.054) in the non-psoriasis cohort. High-density lipoprotein (HDL) levels increased significantly both before and after psoriasis incidence date in the psoriasis cohort. Triglyceride (TG) levels were significantly higher (p<0.001), and HDL levels significantly lower (p=0.013) in patients with psoriasis compared to non-psoriasis subjects. There were no differences in prescriptions for lipid lowering drugs between the two cohorts. CONCLUSIONS: Patients with psoriasis had a significant decrease in TC and LDL levels during the 5 years before psoriasis incidence. Higher mean TG and lower mean HDL levels were noted in the 5 years before psoriasis incidence. These changes are unlikely to be caused by lipid lowering treatment alone and require further exploration.

Retrospective analysis of 578 inpatient dermatology consultations in hematology and hematopoietic stem cell transplant patients.

BACKGROUND: Hospitalized patients with hematologic malignancies are medically complex and commonly affected by dermatologic conditions. METHODS: Retrospective chart review from January 1, 2014, to December 31, 2018, at Rochester Methodist Hospital (Rochester, Minnesota, USA). Patients hospitalized on hematology and BMT services receiving dermatology consultation were included. RESULTS: In all, 578 consultations (63% male, median age 61 years) were reviewed. Drug reactions (22%), infection (17%), and malignant neoplasm (10%) accounted for nearly half of diagnoses. Exanthematous drug reaction (10%), graft-versus-host disease (7%), and lymphoma or leukemia cutis (6%) were the commonest individual diagnoses. There were significantly more drug reactions in severe neutropenia (33.2% vs. 15.0%), neutrophilic dermatoses in myeloid neoplasm (5.2% vs. 0.3%), and viral infection in lymphoid neoplasm (8.3% vs. 1.2%). Consultation frequently altered treatment (68%), diagnostic workup (63%), and the primary service's initial diagnostic impression (53%). Biopsies were performed in 52% of consultations and helped secure a diagnosis 73% of the time. A total of 16.4% of consultations did not receive a definitive final diagnosis, and 18.5% were resolved in one visit. CONCLUSION: This is the largest study to date of hospital dermatology consultation in hematology patients. Biopsies are utilized frequently and are diagnostically useful. The complexity of this patient population is evidenced by the fact that a final diagnosis remains elusive in a number of cases despite the multiple visits required for the vast majority of consultations. Nevertheless, dermatology consultation alters diagnosis and treatment in the majority of patients, highlighting the critical role dermatologists have in the care of these patients.

Retrospective analysis of 450 emergency department dermatology consultations: An analysis of in-person and teledermatology consultations from 2015 to 2019.

INTRODUCTION: Dermatologic complaints are a common reason for emergency department visits. METHODS: Retrospective chart review from 1 January 2015 to 31 December 2019. Patients in the Mayo Clinic Emergency Department receiving dermatology consultation were included. RESULTS: Dermatitis (24.7%, n = 113), infection (20.4%, n = 93), and drug reaction (10.3%, n = 47) accounted for the majority of diagnoses. Emergency department providers often provide no diagnosis (38%) or a differential diagnosis (22%), and dermatology consultation frequently alters diagnosis (46%) and treatment (83%). Patients receiving in-person consultations are admitted more frequently than those receiving teledermatology consultations (40% vs. 16%, p < 0.001). Primary diagnostic concordance with subsequent dermatology evaluation is high for in-person (94%) and teledermatology (88%) consultations. DISCUSSION: This is the largest study of emergency department dermatology consultations in the United States and the first to compare in-person and teledermatology emergency department consultation utilization in clinical practice. These modalities are utilized in a complementary fashion at our institution, with severe dermatologic diagnoses seen in-person. The valuable role of emergency department dermatologists is highlighted by frequent changes to diagnosis and treatment plans that result from dermatology consultation. Furthermore, our data suggest that teledermatology is an effective modality with the potential to expand access to dermatologic expertise in the emergency department setting.

Incidence of psoriasis in children: a population-based study.

BACKGROUND: Although psoriasis is considered to have a dual peak in age of onset, currently no studies exist regarding the incidence of psoriasis in children. OBJECTIVE: The objective of this study was to determine the incidence of psoriasis in childhood. METHODS: A population-based incidence cohort of patients aged younger than 18 years first given the diagnosis of psoriasis between January 1, 1970, and December 31, 1999, was assembled. The complete medical record of each child was reviewed and psoriasis diagnosis was validated by a confirmatory diagnosis in the medical record by a dermatologist or medical record review by a dermatologist. Age- and sex-specific incidence rates were calculated and were age and sex adjusted to 2000 US white population. RESULTS: The overall age- and sex-adjusted annual incidence of pediatric psoriasis was 40.8 per 100,000 (95% confidence interval: 36.6-45.1). When psoriasis diagnosis was restricted to dermatologist-confirmed subjects in the medical record, the incidence was 33.2 per 100,000 (95% confidence interval: 29.3-37.0). Incidence of psoriasis in children increased significantly over time from 29.6 per 100,000 in 1970 through 1974 to 62.7 per 100,000 in 1995 through 1999 (P < .001). Chronic plaque psoriasis was the most common type (73.7%), and the most commonly involved sites were the extremities (59.9%) and the scalp (46.8%). LIMITATIONS: The population studied was a mostly white population in the upper Midwest. CONCLUSION: The incidence of pediatric psoriasis increases with increasing age. There is no apparent dual peak in incidence. The incidence of pediatric psoriasis increased in recent years in both boys and girls.

Results of Goeckerman treatment for psoriasis in children: a 21-year retrospective review.

Goeckerman treatment has been used for the management of widespread psoriasis in children for several decades at Mayo Clinic. We aimed to review our institutional experience with the effectiveness of Goeckerman treatment for psoriasis in children. We retrospectively reviewed the records of pediatric patients who underwent Goeckerman treatment over a 21-year period (1983-2003). The main outcome measure was improvement in psoriasis. During the study period, 65 children received Goeckerman treatment for predominantly widespread, recalcitrant psoriasis. The mean age was 11.6 years (range, 3 mos to 18 yrs), and the female-to-male ratio was 2:1. Psoriasis improved in all patients: 55 patients (85%) had >80% clearance of their psoriasis. The only adverse effect was folliculitis, occurring in 27 patients (42%). Mean duration of follow-up was 2.6 years (range, 17 days-18.2 yrs); average duration of remission was 2.6 years (range, 2 mos-12.79 yrs). Goeckerman treatment is an effective treatment for widespread psoriasis in children.

Association of Autoimmune Vasculitis and Incident Atrial Fibrillation: A Population-Based Case-Control Study.

Background Recent investigations suggest that inflammation and autoimmunity might have a role in the pathophysiology of atrial fibrillation (AF). Given that abnormal ventriculovascular coupling often coexists with AF, we hypothesize that autoimmune vasculitis plays a significant role in the pathogenetic mechanism of AF. Methods and Results A standardized retrospective population-based case-control study was conducted to evaluate the association between autoimmune vasculitis and AF, and all-cause mortality. The study included 8459 patients with a new diagnosis of AF and 8459 age-, sex-, and registration calendar year-matched controls in Olmsted County, Minnesota, between January 1, 1980 and December 31, 2010. The association of each clinical characteristic, diagnosis, and treatment was assessed using conditional logistic regression to account for the matched case-control study design. Cox proportional hazards regression models and Kaplan-Meier curves were used to detect independent predictors of mortality and examine cumulative survival. Of a total of 16 918 patients (mean age 72.3+14.4 years; 48.7% women), 320 (1.9%) were diagnosed with autoimmune vasculitis before the index date during the 30-year period. Among the cases, the prevalence of any autoimmune vasculitis was 2.3%, whereas the frequency of autoimmune vasculitis in controls was 1.5% (P<0.001). After adjusting for potential confounders, the odds of autoimmune vasculitis in AF cases was 1.5 times higher than in controls (odds ratio, 1.47; 95% CI, 1.04-2.01; P=0.03). Patients with AF and autoimmune vasculitis had worse 5-year survival than those without autoimmune vasculitis or AF (44.7% versus 77.2%; log-rank P<0.001). Conclusions Autoimmune vasculitis is significantly associated with AF and independently confers worse survival. These observations may represent one mechanism linking autoimmunity and inflammation to the pathogenesis and prognosis of AF.

Trends in incidence of adult-onset psoriasis over three decades: a population-based study.

BACKGROUND: Incidence studies of psoriasis are rare, mainly due to lack of established epidemiological criteria and the variable disease course. The objective of this study is to determine time trends in incidence and survival of psoriasis patients over three decades. METHODS: We identified a population-based incidence cohort of 1633 subjects aged > or = 18 years first diagnosed with psoriasis between January 1, 1970 and January 1, 2000. The complete medical records for each potential psoriasis subject were reviewed and diagnosis was validated by either a confirmatory diagnosis in the medical record by a dermatologist or medical record review by a dermatologist. Age- and sex-specific incidence rates were calculated and were age- and sex-adjusted to the 2000 US white population. RESULTS: The overall age- and sex-adjusted annual incidence of psoriasis was 78.9 per 100,000 (95% confidence interval [CI]: 75.0-82.9). When psoriasis diagnosis was restricted to dermatologist-confirmed subjects, the incidence was 62.3 per 100,000 (95% CI: 58.8-65.8). Incidence of psoriasis increased significantly over time from 50.8 in the period 1970-1974 to reach 100.5 per 100,000 in the 1995-1999 time period (P = .001). Although the overall incidence was higher in males than in females (P = .003), incidence in females was highest in the sixth decade of life (90.7 per 100,000). Survival was similar to that found in the general population (P = .36). LIMITATIONS: The study population was mostly white and limited to adult psoriasis patients. CONCLUSION: The annual incidence of psoriasis almost doubled between the 1970s and 2000. The reasons for this increase in incidence are currently unknown, but could include a variety of factors, including a true change in incidence or changes in the diagnosing patterns over time.

Staphylococcal scalded skin syndrome and toxic shock syndrome after tooth extraction.

Staphylococcus aureus may cause cutaneous and systemic infections such as staphylococcal scalded skin syndrome (SSSS) and toxic shock syndrome (TSS). Although exfoliative toxins A and B, which cause SSSS, and TSS toxin-1 may be produced by different strains of S aureus, the two syndromes rarely occur simultaneously. We describe a patient admitted to the intensive care department with an exfoliative generalized erythroderma, signs of shock, and biopsy specimen findings consistent with SSSS. This patient presented after a tooth extraction. Symptoms recurred after dismissal, and he was readmitted. Subsequent test results were positive for exfoliative toxin B and TSS toxin-1, suggesting concurrent SSSS and TSS. For patients with acute, exfoliative, generalized erythroderma, TSS and SSSS should be considered. Although rare, it is possible to see both syndromes present concurrently.

Potential misclassification of patients with psoriasis in electronic databases.

BACKGROUND: Electronic claims and medical record databases are increasingly used for observational studies of psoriasis. The purpose of this study was to assess the validity of psoriasis diagnostic codes in an electronic database. METHODS: This study was performed in a population-based setting in Olmsted County, Minnesota, where all diagnoses and procedures from all health care providers in a large community are indexed and recorded in an electronic database. The database was searched for patients aged 18 years or older with diagnostic codes consistent with psoriasis for the time period January 1, 1976, to January 1, 2000. The complete medical records of all patients were reviewed manually for validation of psoriasis diagnoses. RESULTS: We reviewed the complete medical records of 2556 patients with at least one diagnostic code consistent with psoriasis. Based on medical record review, 1458 (57.0%) patients were confirmed to have psoriasis, of which the majority (81%) received confirmation by a dermatologist. The most commonly used diagnostic codes for psoriasis were International Classification of Diseases, Ninth Revision 696.1 (psoriasis, not otherwise specified) with a positive predictive value of 68.7% (95% confidence interval: 66.5%, 70.9%). Increasing frequency of codes in a given time window was associated with positive predictive values. However, positive predictive value for only one code in a 5-year time window was still as high as 60% (95% confidence interval: 57%, 63%). LIMITATIONS: Differences between individual electronic medical record databases may limit the ability to form a general conclusion from these findings. The remitting, relapsing course of psoriasis and the heterogeneity in clinical presentation create challenges in case ascertainment. CONCLUSION: Electronic identification of patients with psoriasis by diagnostic codes alone may lead to misclassification in database studies.