Mark D. P. Davis

Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria

Pyoderma gangrenosum is a rare but significant cause of ulcerations. It is a diagnosis of exclusion. Herein, we suggest diagnostic criteria and some historical perspectives on the diagnosis of pyoderma gangrenosum.

Incidence of Hidradenitis Suppurativa and Associated Factors: A Population-Based Study of Olmsted County, Minnesota

There are no population-based incidence studies of hidradenitis suppurativa (HS). Using the medical record linkage system of the Rochester Epidemiology Project, we sought to determine the incidence of the disease, as well as other associations and characteristics, among HS patients diagnosed in Olmsted County, Minnesota, between 1968 and 2008. Incidence was estimated using the decennial census data for the county. Logistic regression models were fit to evaluate associations between patient characteristics and disease severity. A total of 268 incident cases were identified, with an overall annual age- and sex-adjusted incidence of 6.0 per 100,000. Age-adjusted incidence was significantly higher in women compared with men (8.2 (95% confidence interval (CI), 7.0-9.3) vs. 3.8 (95% CI, 3.0-4.7). The highest incidence was among young women aged 20-29 years (18.4 per 100,000). The incidence has risen over the past four decades, particularly among women. Women were more likely to have axillary and upper anterior torso involvement, whereas men were more likely to have perineal or perianal disease. In addition, 54.9% (140/255) patients were obese; 70.2% were current or former smokers; 42.9% carried a diagnosis of depression; 36.2% carried a diagnosis of acne; and 6% had pilonidal disease. Smoking and gender were significantly associated with more severe disease.

Incidence of Dermatomyositis and Clinically Amyopathic Dermatomyositis: A Population-Based Study in Olmsted County, Minnesota

OBJECTIVES To identify new and existing cases of dermatomyositis and its subtypes in Olmsted County, Minnesota, from 1976 through 2007, and to establish a population-based estimate of the incidence and prevalence of dermatomyositis and amyopathic dermatomyositis. DESIGN Retrospective population-based study. SETTING Community-based epidemiology project. Patients Patients with a diagnosis of dermatomyositis were identified from the Rochester Epidemiology Project. MAIN OUTCOME MEASURES Incidence of dermatomyositis and clinically amyopathic dermatomyositis and risk of malignancy in clinically amyopathic dermatomyositis. RESULTS Of the 29 patients identified, 6 (21%) had the clinically amyopathic subtype of dermatomyositis and 22 (76%) were female. Overall age- and sex-adjusted incidence of dermatomyositis including all subtypes was 9.63 (95% confidence interval [CI], 6.09-13.17) per 1 million persons and 2.08 (95% CI, 0.39-3.77) per 1 million persons for clinically amyopathic dermatomyositis. Age- and sex-adjusted prevalence for all subtypes of dermatomyositis was 21.42 (95% CI, 13.07-29.77) per 100,000 persons. Eight patients (28%) had a malignant condition during the study period; the risk of malignancy (odds ratio) for classic dermatomyositis compared with clinically amyopathic dermatomyositis was 4.61 but was not statistically significant (95% CI, 0.22-96.09) (P=.44). CONCLUSIONS Dermatomyositis is a rare disease, and clinically amyopathic dermatomyositis represents an estimated 20% of all dermatomyositis cases. Larger population-based studies are needed to estimate the risk of malignancy associated with subtypes of dermatomyositis, particularly clinically amyopathic dermatomyositis.

Lupus-Like Syndrome Attributable to Anti―Tumor Necrosis Factor α Therapy in 14 Patients During an 8-Year Period at Mayo Clinic

OBJECTIVE To examine clinical characteristics, laboratory features, and outcomes of patients with lupus-like syndrome attributable to anti-tumor necrosis factor alpha (TNF-alpha) therapy. PATIENTS AND METHODS We performed a retrospective review of patients with lupus-like syndrome attributable to anti-TNF-alpha therapy at Mayo Clinics site in Rochester, MN, between July 1, 2000, and June 30, 2008. RESULTS Of 14 patients (mean age at disease onset, 46.2 years), 12 (86%) were female. Ten patients (71%) had Crohn disease, and 4 (29%) had rheumatoid arthritis. Thirteen patients (93%) originally were treated with infliximab, and 1 (7%) was treated with adalimumab. A lupus-ike syndrome occurred after a mean treatment duration of 16.2 months. Features of lupus included presence of antinuclear antibodies (14 patients [100%]), arthritis (13 patients [93%]), anti-double-stranded-DNA antibodies (10 patients [71%]), cutaneous findings (malar rash, discoid rash, or photosensitivity, 4 patients [29%]), serositis (4 patients [29%]), hematologic abnormalities (4 patients [29%]), oral ulcers (4 patients [29%]), and lupus anticoagulant (1 patient [7%]). No patient had renal or neurologic abnormalities. All patients improved after stopping anti-TNF-alpha therapy (mean time to improvement, 2.9 months). Four (80%) of 5 patients tolerated an alternative TNF-alpha inhibitor (adalimumab, 3 patients; etanercept, 1 patient) without recurrence of lupus-like syndrome. CONCLUSION Compared with previous studies, cutaneous findings were less frequent and arthritis was more frequent in our cohort of patients. Some patients were able to tolerate an alternative TNF-alpha inhibitor without recurrence of lupus-like syndrome.

Incidence of Cutaneous Lupus Erythematosus, 1965-2005: A Population-Based Study

OBJECTIVES To assess trends in the cutaneous variants of lupus erythematosus (CLE) and to ascertain the incidence of CLE over the past 4 decades. DESIGN Retrospective population-based study. SETTING Community-based epidemiology project. PATIENTS All Olmsted County, Minnesota, residents with any subtype of CLE between January 1965 and December 2005. MAIN OUTCOME MEASURES Incidence of CLE and disease progression to systemic LE (SLE). RESULTS A total of 156 patients with newly diagnosed CLE (100 females and 56 males) were identified between 1965 and 2005. The incidence rate (age and sex adjusted to the 2000 US white population) was 4.30 (95% confidence interval [CI], 3.62-4.98) per 100,000. The age- and sex-adjusted prevalence as of January 1, 2006, was 73.24 (95% CI, 58.29-88.19) per 100,000. Nineteen patients with CLE had disease progression to SLE: cumulative incidence at 20 years, 19%; mean (SD) length to progression, 8.2 (6.3) years. Compared with a previously reported incidence of 2.78 (95% CI, 2.08-3.49) per 100,000 for SLE among Rochester, Minnesota, residents in 1965 through 1992, the incidence of CLE in Rochester was 3.08 (95% CI, 2.32-3.83) per 100,000 in 1965 through 1992. CONCLUSIONS The incidence of CLE is comparable to the published incidence of SLE. Our findings double the incidence of the root designation of the disease process known as LE (SLE and CLE).

Calcinosis Cutis Occurring in Association With Autoimmune Connective Tissue Disease: The Mayo Clinic Experience With 78 Patients, 1996-2009

OBJECTIVE To describe characteristics and treatment of patients with calcinosis cutis in the clinical setting of autoimmune connective tissue disease (ACTD). DESIGN Retrospective study. SETTING Tertiary referral center. PATIENTS Seventy-eight patients with calcinosis cutis and ACTD between 1996 and 2009. MAIN OUTCOME MEASURES Clinical features, treatments, and outcomes of patients with calcinosis cutis in the clinical setting of ACTD. RESULTS Of 78 patients (mean age at onset of calcinosis cutis, 40.1 years), 64 (82%) were female. The following diseases were associated with calcinosis cutis: dermatomyositis (n = 30) with classic (n = 15), juvenile (n = 14), and amyopathic (n = 1) subtypes; systemic sclerosis with limited cutaneous scleroderma (n = 24); lupus panniculitis (n = 4); systemic lupus erythematosus (n = 2); mixed connective tissue disease (n = 4); overlap connective tissue disease (n = 6); undifferentiated connective tissue disease (n = 6); polymyositis (n = 1); and rheumatoid arthritis (n = 1). Therapy for calcinosis cutis consisted of medical treatment alone (n = 19), surgical therapy alone (n = 11), combined medical and surgical treatment (n = 17), no treatment (n = 30), and unknown (n = 1). Diltiazem hydrochloride was the most commonly used medical therapy, with 9 of 17 patients having a partial response. Twenty-eight patients had surgical excision of 1 or more lesions of calcinosis cutis: 22 had a complete response, 5 had a partial response, and 1 had no response. CONCLUSIONS Dermatomyositis and systemic sclerosis were the most common ACTDs associated with calcinosis cutis. Although no treatment was uniformly effective, surgical excision of symptomatic lesions and medical treatment with diltiazem provided benefit for some patients.

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.

Confluent and reticulate papillomatosis (Gougerot–Carteaud syndrome): a minocycline‐responsive dermatosis without evidence for yeast in pathogenesis. A study of 39 patients and a proposal of diagnostic criteria

Background  Confluent and reticulate papillomatosis (CRP) (Gougerot–Carteaud syndrome) is a disorder that has been characterized in only small cohorts of patients.

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.

Effectiveness of Intravenous Immunoglobulin Therapy for Skin Disease Other Than Toxic Epidermal Necrolysis: A Retrospective Review of Mayo Clinic Experience

OBJECTIVE To examine retrospectively the use and effectiveness of intravenous immunoglobulin (IVIg) treatment of various skin diseases, primarily immunobullous disease. PATIENTS AND METHODS We identified patients who had received IVIg therapy for skin disease between 1996 and 2003 at the Mayo Clinic in Rochester, Minn, Scottsdale, Ariz, and Jacksonville, Fla, and retrospectively reviewed their medical records. RESULTS Eighteen patients were treated with IVIg for various skin diseases: immunobullous disease in 11 adults (pemphigus vulgaris [7 patients], bullous pemphigold [3], and cicatricial pemphigoid [1]); dermatomyositis (2); mixed connective tissue disease (1); chronic urticaria (1); scleromyxedema (1); leukocytoclastic vasculitis (1); and linear IgA bullous disease (1). Responses of patients by type of disease were as follows: pemphigus vulgaris, 1 partial response (PR) and 6 no response (NR); bullous pemphigoid, 1 complete response (CR) and 2 NR; cicatricial pemphigoid, 1 NR; dermatomyositis, 1 CR and 1 PR; mixed connective tissue disease, 1 CR; chronic urticaria, 1 CR; scleromyxedema, 1 CR; leukocytoclastic vasculitis, 1 PR; and linear IgA bullous disease, 1 CR. Six patients (33%) experienced CR, 3 (17%) had PR, and 9 (50%) had NR to IVIg therapy. All 9 nonresponders were adult patients with immunobullous disease. CONCLUSION Although this was a retrospective study of a small cohort of a mixture of patients, the findings emphasize that our experience with IVIg treatment for skin disease, particularly immunobullous disease, is less favorable than that reported previously. Further studies are needed to verify the efficacy of IVIg for skin disease.