Marian T. McEvoy

Incidence and Clinical Predictors of Psoriatic Arthritis in Patients With Psoriasis: A Population-Based Study

OBJECTIVE To determine the incidence and disease-specific predictors of clinically recognized psoriatic arthritis (PsA) in patients with psoriasis. METHODS We identified an incidence cohort of psoriasis subjects age >/=18 years diagnosed between January 1, 1970 and December 31, 1999 in a population-based setting. Psoriasis diagnoses were validated by confirmatory diagnosis in the medical record. Incident and clinically recognized PsA subjects were classified according to the Classification of Psoriatic Arthritis (CASPAR) criteria. Cox proportional hazards models were used to identify predictors of PsA within the psoriasis cohort. RESULTS The psoriasis incidence cohort comprised 1,633 subjects. Of these, 40 were diagnosed with PsA concurrently with psoriasis and were excluded from analysis. The remaining 1,593 psoriasis subjects had a mean age of 43 years and 50% were men. Over 20,936 person-years of followup, 57 subjects were clinically recognized with new-onset PsA, with a cumulative incidence of 1.7% (95% confidence interval [95% CI] 1.0-2.3%), 3.1% (95% CI 2.2-4.1%), and 5.1% (95% CI 3.7-6.6%) at 5, 10, and 20 years following psoriasis incidence, respectively. Psoriasis features associated with higher risk of PsA were scalp lesions (hazard ratio [HR] 3.89, 95% CI 2.18-6.94), nail dystrophy (HR 2.93, 95% CI 1.68-5.12), and intergluteal/perianal lesions (HR 2.35, 95% CI 1.32-4.19). Calendar year was not associated with risk of PsA (P = 0.15), indicating that the likelihood of PsA in psoriasis subjects did not change over time. CONCLUSION In this population-based study, <10% of patients with psoriasis developed clinically recognized PsA during a 30-year period. Psoriasis features associated with a higher likelihood of PsA were nail dystrophy, scalp lesions, and intergluteal/perianal psoriasis.

A New Autosomal Dominant Disorder of Pyogenic Sterile Arthritis, Pyoderma Gangrenosum, and Acne: PAPA Syndrome

OBJECTIVE To describe a multigenerational family with transmission of an autosomal dominant disorder characterized by pyogenic arthritis, pyoderma gangrenosum, and severe cystic acne. MATERIAL AND METHODS We present a detailed case report of a 39-year-old man with arthritic changes in several joints, pyoderma gangrenosum, and cystic acne. Several relatives from three generations of his family underwent clinical and genetic investigations. The findings in this kindred are reported. RESULTS Ten affected family members in three generations manifested variable expression of a pauciarticular, nonaxial, destructive, corticosteroid-responsive arthritis that began in childhood; pyoderma gangrenosum; and severe cystic acne in adolescence and beyond. Other less commonly associated features included adult-onset insulin-dependent diabetes mellitus, proteinuria, abscess formation at the site of parenteral injections, and cytopenias attributable to sulfonamide medications. Laboratory evaluation was nondiagnostic. Genetic studies excluded linkage to the major histocompatibility locus. CONCLUSION The acronym of PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne) is suggested for this newly recognized pleiotropic autosomal dominant disorder. The nature of the genetic alteration in PAPA syndrome is unknown.

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.

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.

Exaggerated arthropod-bite lesions in patients with chronic lymphocytic leukemia: A clinical, histopathologic, and immunopathologic study of eight patients ☆ ☆☆ ★

BACKGROUND Unusual papulovesicular lesions resembling arthropod bites have been described in patients with chronic lymphocytic leukemia (CLL). OBJECTIVE Our purpose was to describe and characterize further the clinical, histopathologic, and immunopathologic features of these lesions. METHODS Eight patients were identified retrospectively who had CLL and characteristic skin lesions. Clinical and histologic features were recorded. Skin biopsy specimens were analyzed immunohistochemically for eosinophil granule major basic protein, eosinophil-derived neurotoxin, neutrophil elastase, and mast cell tryptase. RESULTS The clinical features, including the lesional distribution, suggested arthropod bites, although most patients could not recall having been bitten. Mixed T- and B-cell lymphoid cell infiltrates were present within lesions, along with prominent eosinophil infiltration and eosinophil granule protein deposition. CONCLUSION Exuberant papulovesicular lesions develop in patients with CLL apparently as an exaggerated response to arthropod bites. Prominent eosinophil infiltration and degranulation within these lesions likely contribute to the severity of symptoms.

Linear IgA dermatosis: Association with malignancy

We report the association of linear IgA dermatosis with hematologic malignancy (chronic lymphatic leukemia and plasmacytoma) in two patients. Ten documented cases of linear IgA dermatosis and internal malignancy have been reported in the literature. The possible association of malignancy and linear IgA dermatosis is discussed.

Neutrophilic dermatosis of the hands: four new cases and review of the literature

Background  Isolated or predominantly hand involvement in Sweets syndrome, pyoderma gangrenosum, or pustular vasculitis is a rare presentation in the spectrum of neutrophilic dermatoses and is often associated with an occult malignancy or other systemic inflammatory disorder. When these disorders occur on the hands, they are often clinically indistinguishable, but they can sometimes be separated histologically by the presence of papillary dermal edema (Sweets syndrome), ulceration and necrosis (pyoderma gangrenosum), or vasculitis (pustular vasculitis). These distinctions may be arbitrary, however, and reflect differences in the temporal course of the disease and in the degree of inflammation at the time of biopsy.

Early cutaneous gene transcription changes in adult atopic dermatitis and potential clinical implications.

Abstract:  Atopic dermatitis (AD) is a common pruritic dermatitis with macroscopically non‐lesional skin that is often abnormal. Therefore, we used high‐density oligonucleotide arrays to identify cutaneous gene transcription changes associated with early AD inflammation as potential disease control targets. Skin biopsy specimens analysed included normal skin from five healthy non‐atopic adults and both minimally lesional skin and nearby or contralateral non‐lesional skin from six adult AD patients. Data were analysed on an individual gene basis and to identify biologically relevant gene networks. Transcription levels of selected genes were also analysed by quantitative PCR. Differential transcription occurring early in AD skin was indicated for (i) individual genes such as C‐C chemokine ligand (CCL)18, CCL13, and interferon‐α2 (IFNα2), (ii) genes associated with peroxisome proliferator‐activated receptor (PPAR)α‐ and PPARγ‐regulated transcription, and possibly for (iii) immunoglobulin J‐chain and heavy chain isotype transcripts. These data suggest that local changes in immunoglobulin‐associated transcription may favour IgE over secretory immunoglobulin (multimeric IgM and IgA) expression in AD skin. Decreased PPAR activity appears common to both AD and psoriasis, and reduced cutaneous IFNα2 transcription also appears characteristic of AD. Identification of these genes and pathways will direct future research towards controlling AD.

A medical-center-wide, multidisciplinary approach to the problem of natural rubber latex allergy

Latex is a common cause of occupational allergy in health care workers; latex-sensitized patients are at increased risk of allergic reactions in medical environments. Skin test reagents and latex-specific immunoglobulin E immunoassays were established for diagnosis of latex allergy. Inhibition immunoassays were developed for measuring latex aeroallergens and latex allergens in rubber products. A registry of latex-sensitive employees was established. High-allergen gloves were removed from the medical center inventory; latex aeroallergen levels subsequently declined. Despite an increasing number of gloves used annually, expenditures for gloves in 1994 were lower than in previous years. Latex-sensitive individuals can be identified using skin tests or immunoassays. Latex aeroallergen levels in medical environments can be reduced substantially at lower cost by using powder-free rubber gloves with lower allergen content.

Herpes zoster after varicella immunization

cine was licensed in the United States in 1995. The vaccine is safe, immunogenic, and highly protective against severe varicella in healthy children and adults and in certain immunocompromised patients.1-3 It was initially hoped that herpes zoster might not occur after immunization. However, Oka vaccine-type varicella-zoster virus can cause latent infection in children with acute lymphocytic leukemia.4,5 We describe herpes zoster caused by vaccine strain varicella-zoster virus in a child.