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Biomedical subjects

J L Jorizzo

Publications and source records attributed to J L Jorizzo.

At least 37 records · Page 2Linked to original sources

Cutaneous small-vessel vasculitis.

Cutaneous small-vessel vasculitis (CSVV) refers to a group of disorders usually characterized by palpable purpura; it is caused by leukocytoclastic vasculitis of postcapillary venules. CSVV can be idiopathic or can be associated with a drug, infection, or underlying systemic disease. Initially, the pathogenesis of CSVV is immune complex related, but in its later stages different pathogenetic mechanisms may intensify the reaction and lymphocytes may predominate in the infiltrate. Cure requires elimination of the cause (ie, drugs, chemicals, infections, food allergens) when possible, as well as therapy with nonsteroidal antiinflammatory agents, corticosteroids, dapsone, potassium iodide, fibrinolytic agents, aminocaproic acid, immunosuppressive agents (ie, cyclophosphamide, azathioprine, methotrexate, cyclosporine) or even monoclonal antibodies, depending on disease severity.

Aminocaproates↗

Papular dermatitis (subacute prurigo, "itchy red bump" disease): pilot study of phototherapy.

BACKGROUND: Some patients with a subacute or chronic pruritic erythematous papular eruption are refractory to treatment. We previously described a number of these patients with papular dermatitis or subacute prurigo. OBJECTIVE: The purpose of this study was to examine the effectiveness of different types of phototherapy for treatment of papular dermatitis. METHODS: We reviewed the medical records of 11 patients who were diagnosed with papular dermatitis and who underwent phototherapy within the last 5 years. RESULTS: Eleven patients had a total of 17 phototherapy courses: psoralen-UVA (PUVA; 9), UVA/UVB light (3), and UVB alone (5). Within the PUVA treatment group, three of nine patients experienced total clearing, and six of nine patients experienced partial improvement. Although patients in all groups relapsed with time, overall the PUVA-treated patients had the best response rate and the best chance of the condition remaining clear after treatment was stopped. CONCLUSION: PUVA may be an effective treatment for papular dermatitis. The frequency of relapse indicates that maintenance treatments may be necessary for long-term control of the disease.

Aged↗

Dermatomyositis: a dermatology-based case series.

BACKGROUND: Dermatomyositis is associated with significant morbidity and occasional mortality. Currently there is no consensus on treatment for patients with dermatomyositis. OBJECTIVE: Our purpose was to review the clinical features and response to therapy of patients with dermatomyositis and compare these data with previous series of patients with dermatomyositis/polymyositis. METHODS: Clinical characteristics of 65 patients seen during a 10-year period were reviewed retrospectively. Twenty-one of these patients were enrolled in a prospective, uncontrolled study of treatment with high-dose prednisone followed by slow tapering. RESULTS: Clinical features were similar to those previously described; however, muscle strength at diagnosis was on average greater in patients in this series than in patients previously reported. Malignancy was present in 5 of 43 adult patients (12%), but was not found in patients with juvenile dermatomyositis. Another connective tissue disease was present in 19% of patients. Twelve patients had dermatomyositis sine myositis. Eighteen of 21 patients (85%) in the prednisone study group had resolution of myositis. CONCLUSION: Patients with dermatomyositis in this series had less active myositis at presentation, but were otherwise similar to patients with dermatomyositis/polymyositis previously reported. Treatment with high-dose daily prednisone followed by slow tapering was effective.

Adult↗

Juvenile dermatomyositis and polymyositis: a follow-up study of long-term sequelae.

BACKGROUND: Juvenile dermatomyositis/polymyositis (DMS/PM) are childhood inflammatory myopathies that normally affect children between 2 and 15 years of age. The disease is manifested as severe proximal muscle weakness and, in JDMS, as a characteristic cutaneous eruption. It is difficult to predict the course of the disease. This study is aimed at objectively assessing the long-term morbidity of dermatomyositis/polymyositis in children. METHODS: Twelve cases of JDMS/PM identified through chart review were evaluated by physical and dermatologic examination, muscle testing, ultrasonography, and magnetic resonance imaging (MRI). RESULTS: The patient population included 6 male and 6 female patients with a mean age of 18.8 years (standard deviation 12.4). The mean age at onset of JDMS/PM was 5.8 years (SD 2.8), and the mean time from onset of symptoms to beginning of the study was 10.1 years (SD 9.3). The mean duration of corticosteroid therapy was 53.1 months (SD 40.5), with patients reporting an average of 1.7 relapses (SD 1.5). Patients compared their own activity level with that of healthy control subjects, and on a scale of 1 to 5 with 5 being normal, their mean score was 3.9 (SD 0.9). Fifty-eight percent of the patients had at least one residual finding on physical examination, and 78% of those with JDMS had residual dermatologic sequelae. Despite these findings, the mean grade for the group was 4.6 (SD 0.6) for muscle strength, using a scale of 0 to 5 with 0 being no muscle resistance and 5 being normal. In 60% of patients examined by ultrasonography, increased echogenicity indicated possible residual fibrotic change in at least one muscle group. Although MRI showed no active disease process in four patients, scattered intramuscular calcification was found in one patient. CONCLUSIONS: Multiple physical and dermatologic sequelae are commonly present in patients with JDMS/PM on long-term follow-up, but these do not preclude a productive life. Ultrasonography and MRI provide additional noninvasive means of analysis, but because of the current high cost, MRI is not routinely recommended.

Adolescent↗

Norgestimate and ethinyl estradiol in the treatment of acne vulgaris: a randomized, placebo-controlled trial.

OBJECTIVE: To evaluate the efficacy of a triphasic, combination oral contraceptive (OC), (norgestimate-ethinyl estradiol), in comparison with placebo in the treatment of moderate acne vulgaris. METHODS: Two hundred fifty women were enrolled in a multicenter, randomized, double-blind, placebo-controlled clinical trial to evaluate the effectiveness of norgestimate-ethinyl estradiol in the treatment of acne vulgaris. Subjects were 15-49 years old and had moderate acne vulgaris. Each month for 6 months, subjects received either 3 consecutive weeks of active OC treatment followed by 1 week of inactive drug, or 4 consecutive weeks of color-matched placebo tablets. Efficacy was assessed by facial acne lesion counts, the investigator's global assessment, and the subject's self-assessment. Hormone levels were also measured. RESULTS: Despite the large placebo effect inherent in an acne trial (due to, for example, careful skin care, frequent office visits, regression to the mean), of the 164 subjects who completed the study without major protocol deviations, the OC group was significantly better than the placebo group for all primary efficacy measures: inflammatory lesions (mean reduction, 51.4% compared to 34.6%; P = .01), total lesions (mean reduction, 46.4% compared to 33.9%; P = .001); investigator's global assessment (83.3% compared to 62.5%; P = .001). Free testosterone decreased significantly and sex hormone-binding globulin increased significantly in the OC group, but remained unchanged in the placebo group. CONCLUSION: A triphasic combination of norgestimate and ethinyl estradiol is an effective treatment for moderate acne vulgaris in women with no known contraindication to OC therapy.

Acne Vulgaris↗

Tazarotene gel, a new retinoid, for topical therapy of psoriasis: vehicle-controlled study of safety, efficacy, and duration of therapeutic effect.

BACKGROUND: Topical therapy providing initial improvement and maintenance of effect after treatment of the large majority of patients with limited, mild to moderate psoriasis is not presently available. Previous topical retinoids have generally been either ineffective or too irritating for therapy of psoriasis. OBJECTIVE: Our purpose was to evaluate a new topical retinoid, tazarotene, in the treatment of stable plaque psoriasis during treatment and posttreatment periods. METHODS: In a double-blind manner, 324 patients were randomly selected to receive tazarotene 0.1% or 0.05% gel, or vehicle control, once daily for 12 weeks and were then followed up for 12 weeks after treatment. RESULTS: Of the total, 318 patients could be evaluated. Tazarotene gels were superior (p < 0.05) to vehicle, often as early as treatment week 1, in all efficacy measures: plaque elevation, scaling, and erythema; treatment response; percentage treatment success (patients with > or = 50% improvement); and time to initial success. Efficacy was equivalent on target lesion sites (trunk or limbs and knees or elbows) and overall. A sustained therapeutic effect was observed for 12 weeks after treatment. Tazarotene gel was cosmetically acceptable. There was low systemic absorption, limiting toxicity to local irritation. CONCLUSION: Once-daily tazarotene was effective and safe as a topical monotherapy for plaque psoriasis, providing rapid reduction of signs and symptoms.

Administration, Topical↗

Adult pityriasis rubra pilaris: a 10-year case series.

BACKGROUND: Pityriasis rubra pilaris (PRP) often has a devastating impact on the lives of patients. Descriptions of its histopathologic features are not uniform. Finding a successful therapy can be challenging. OBJECTIVE: Our purpose was to examine the histopathologic features and response of patients to our standard therapy of an oral retinoid and concomitant or later addition of low-dose weekly methotrexate. METHODS: A retrospective chart review was done on 24 patients with PRP seen from March 1986 to March 1996. Biopsy specimens from 19 patients were reexamined. Telephone follow-up was conducted to determine maintenance of remission. RESULTS: All patients had the adult acquired form of PRP. Biopsy specimens from nine patients were characterized by prototypical findings of PRP, while the others included both typical and other features. Twenty-two patients were treated with either isotretinoin, 40 mg twice daily, or etretinate, 25 to 75 mg/day. Six patients with more disabling involvement had low-dose weekly methotrexate ranging from 5 to 30 mg started concurrently. Five patients had weekly methotrexate added at a later time. Seventeen patients showed 25% to 75% response after 16 weeks of therapy. All patients whose skin cleared maintained their remission. CONCLUSION: Initial oral retinoid plus concurrent or later low-dose weekly methotrexate resulted in 25% to 75% improvement of PRP in 17 of 24 patients after 16 weeks of therapy. Some of the atypical features seen in biopsy specimens emphasize the importance of clinical and histopathologic correlation in establishing the diagnosis.

Administration, Oral↗

Clobetasol propionate emollient 0.05 percent: hypothalamic-pituitary-adrenal-axis safety and four-week clinical efficacy results in plaque-type psoriasis.

Two clinical trials were conducted to evaluate the safety and antipsoriatic efficacy of a new 0.05 percent emollient formulation of clobetasol propionate (CP). In a crossover study of hypothalamic-pituitary-adrenal (HPA)-axis effects in 12 patients with psoriasis or eczema, 1.5 gm of CP emollient, applied to lesions twice daily for seven consecutive days, resulted in fewer patients with serum cortisol concentrations < 10 micrograms/100 mL than CP cream 0.05 percent (1vs 4); such concentrations were seen in two other patients during both treatment phases. A double-blind, randomized, parallel-group clinical trial in patients with moderate to severe plaque-type psoriasis showed that four weeks' treatment with CP emollient 0.43 to 0.5 gm twice daily (n = 35) was significantly more effective than emollient vehicle (n = 39) in reducing total signs/symptoms and scaling by Day 4, erythema and skin thickening by Day 8, and pruritus by Day 15. CP emollient was rated superior to vehicle by Day 4 in physician's gross assessment ratings and by Day 15 in patient's self-assessment ratings. In all assessments, CP emollient continued to be superior to vehicle during the remainder of the treatment period and two-week posttreatment period. No significant differences were observed in tolerability or serum cortisol effects during the course of the study.

Adult↗

Behçet's disease. Report of twenty-five patients from the United States with prominent mucocutaneous involvement.

BACKGROUND: Behçet's disease is a multisystem disease that is rare in the United States. OBJECTIVE: The purpose of our study was to assess the characteristics and treatment of a series of patients with Behçet's disease in the United States. METHODS: A retrospective clinical review of 25 patients with Behçet's disease was performed, and histopathologic findings and therapeutic modalities were reviewed. RESULTS: All patients had oral and genital aphthae, and 22 of 25 patients had cutaneous lesions consistent with Behçet's disease. Eight of 25 patients had relatively severe systemic disease. Nine of 14 biopsy specimens showed a neutrophilic vascular reaction. Our therapeutic "ladder" included aggressive topical and intralesional corticosteroids, colchicine, dapsone, methotrexate, and thalidomide; we reserved systemic corticosteroids and immunosuppressive medications for severe ocular or severe systemic disease. CONCLUSION: This series of patients with Behçet's disease was characterized by patients with prominent mucocutaneous involvement and a low prevalence of ocular involvement. These findings may be attributed to patient selection from referral to a university dermatology clinic.

Administration, Topical↗

Mucocutaneous criteria for the diagnosis of Behçet's disease: an analysis of clinicopathologic data from multiple international centers.

BACKGROUND: Although four of five of the new international criteria for the diagnosis of Behçet's disease relate to mucocutaneous lesions, disagreement exists as to the exact nature of cutaneous lesions (e.g., vessel-based vs follicular). OBJECTIVE: Our purpose was to review clinical data, clinical photographs, and skin biopsy specimens from multiple medical centers throughout the world to monitor current practice in the implementation of mucocutaneous diagnostic criteria for Behçet's disease. METHODS: Ten medical centers responded to a request to collaborate by sending clinical data, photographs of cutaneous lesions, and biopsy specimens from 22 patients. RESULTS: Of specimens from 22 patients, 14 revealed a histopathologic pattern of neutrophils containing perivascular and interstitial inflammation, whereas specimens from three patients revealed only mononuclear cells in a vessel-based pattern. Biopsy specimens from three patients revealed primarily folliculocentric inflammation and an additional two specimens were from erythema nodosum-like lesions. CONCLUSION: Perivascular inflammation was the predominant histopathologic finding in specimens of cutaneous lesions in this clinical series. Folliculocentric lesions could not be predicted on the basis of review of clinical photographs. Histopathologic assessment of cutaneous lesions is crucial if the proposal is accepted that exclusion of folliculocentric lesions is important to ensure accurate implementation of diagnostic criteria in patients with suspected Behçet's disease.

Adult↗

Working classification of vasculitis.

Vasculitis is defined as angiocentric segmental inflammation of the blood vessels and fibrinoid necrosis of the vessel wall. Classification of vasculitis is a controversial problem, because of the difficulty in incorporating globally different criteria such as histologic features, size of affected blood vessels, etiology, pathogenesis and other factors in a single classification schema. In this paper we review the principal classifications and make a novel attempt to classify vasculitis on the basis of the size of affected vessels. We hope that a new generation of basic and clinical investigators can achieve a better understanding of the pathogenesis of various vasculitis-related syndromes and that this understanding will facilitate future classification schemas.

Humans↗

Dermatomyositis with normal muscle enzyme concentrations. A single-blind study of the diagnostic value of magnetic resonance imaging and ultrasound.

BACKGROUND AND METHODS: It is well documented that the cutaneous lesions of dermatomyositis may precede clinical myositis or may occur in the absence of any muscle disease detectable by current diagnostic criteria. In this single-blind study, we used magnetic resonance imaging (MRI) and ultrasound to evaluate five patients who presented with classical clinicopathologic dermatomyositis, but with normal levels of serum muscle enzymes. This patients group has not been previously studied with these techniques. Patients who served as positive and negative control subjects were also examined. RESULTS: Ultrasonography revealed hyperechogenicity, and MRI revealed high signals on T2-weighted images in several muscle groups of the patient with active myositis (positive control). Increased echogenicity was also noted in the deltoid region of one patient who had previously had a normal muscle biopsy finding. In the same patient, MRI revealed inflammatory changes in the lumbar paraspinal muscles. Another patient, with all previous study results being normal, had MRI evidence of T2 high signals in the gluteus minimus. CONCLUSIONS: Noninvasive examinations such as MRI and ultrasound are beneficial as adjunctive means of examination in the evaluation of patients with dermatomyositis sine myositis or dermatomyositis. Future studies may suggest additional uses for these tests, including serial evaluation of patients, noninvasive confirmation of diagnosis in pediatric patients, or in directing muscle biopsy, thus increasing sensitivity. Ultrasound appears to be the more cost-effective and simple test; but MRI, although more expensive, may be more sensitive and specific.

Adult↗

Low-dose methotrexate treatment in elderly patients with bullous pemphigoid.

BACKGROUND: Various treatments have been used for elderly patients with bullous pemphigoid. The most common is moderate-dose to high-dose prednisone, either alone or in combination with other oral or topical agents. OBJECTIVE: The aim of this study was to evaluate the efficacy of low-dose methotrexate as a steroid-sparing agent in patients with bullous pemphigoid. METHODS. A retrospective chart review of 34 patients with bullous pemphigoid seen from 1989 through the first half of 1993 was conducted. The diagnosis of bullous pemphigoid was confirmed by direct or indirect immunofluorescence microscopy, or salt-split skin sections. Eight of 34 elderly (> 60 years old) patients with therapy-resistant bullous pemphigoid received low-dose weekly methotrexate (average, 5 to 10 mg) in combination with oral prednisone. RESULTS: Patients who received combination therapy required significantly lower (p < or = 0.02) doses of prednisone to control their disease at 1 month compared with baseline doses. CONCLUSION: In this elderly patient population the side effect profile of the methotrexate was acceptable and well tolerated. We therefore recommend methotrexate and low-dose corticosteroid as first-line combination therapy for selected elderly patients with bullous pemphigoid.

Administration, Oral↗

Epidermotropic metastatic malignant melanoma simulating melanoma in situ. A report of 10 examples from two patients.

Epidermotropic metastatic malignant melanoma (EMMM) is a well-recognized entity that can simulate primary malignant melanoma, and in the past reports of numerous (> or = 100) such lesions were misconstrued as multiple primary lesions. We present the cases of two patients who had not only numerous epidermotropic metastases that simulated primary melanomas but also 10 lesions that mimicked melanoma in situ. After removal of a primary malignant melanoma, the two patients developed 35 and 22 epidermotropic metastases over a 4-year period before dying with brain and pulmonary metastases, respectively. In these patients, 29 and 22 of the skin lesions were excised, respectively. All had epidermotropic metastases, of which seven and three showed pagetoid melanocytes in a pagetoid pattern exclusively within the epidermis and epithelial structures of adnexa, an "epidermal only" (in situ) pattern. Other lesions showed a continuum of dermal involvement, from the more conventional description of EMMM with the extent of dermal involvement > or = epidermal (n = 2) to epidermal involvement > dermal disease (n = 36). This histologic spectrum of dermal versus epidermal involvement in conjunction with the extremely small size (2.8 mm average histologic diameter), symmetry, large number, and time course of development argues strongly that these lesions represent metastases rather than multiple primary melanomas. The lesions illustrate the diagnostic dilemma posed by EMMM that simulates primary melanoma and further exhibits an "epidermal only" (melanoma in situ) pattern.

Adult↗

Cutaneous changes of dermatomyositis in patients with normal muscle enzymes: dermatomyositis sine myositis?

BACKGROUND: Dermatomyositis sine myositis may have various connotations. Controversy exists as to nomenclature, degree of evaluation required, therapy, and course (e.g., does true dermatomyositis of the skin only exist?). OBJECTIVE: The purpose of this study was to assess prospectively patients with the clinicopathologic features of dermatomyositis and normal muscle enzyme serum levels to determine their course in terms of the onset of muscle disease. METHODS: Thirteen patients were studied by complete history and clinical examination, laboratory studies, electromyography, and skin and muscle biopsy. They were observed for 1 to 6 years. RESULTS: Patients were classifiable into three groups: (1) cutaneous changes only, (2) cutaneous changes only at baseline with subsequent development of myositis, and (3) cutaneous changes with normal muscle enzyme serum levels at baseline but with myositis demonstrated by electromyography and/or muscle biopsy specimens. CONCLUSION: Significantly different prognostic and therapeutic implications are present in patients with dermatomyositis with normal muscle enzyme serum levels depending on the results of electromyography, muscle biopsy, and clinical observation.

Adolescent↗