Review article: a therapeutic update on dermatomyositis/polymyositis.
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Dermatomyositis↗
Biomedical subjects
J L Jorizzo
Publications and source records attributed to J L Jorizzo.
Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions.
Pyoderma gangrenosum (PG) is an idiopathic, inflammatory, ulcerative disease of undetermined cause. The diagnosis is based on clinical and pathologic features and requires exclusion of conditions that produce ulcerations. An atypical bullous variant (atypical pyoderma gangrenosum, APG) exists with clinical features similar to those of Sweet syndrome. Because PG is a rare disease, few large case series have been reported. Pyoderma gangrenosum was first recognized as a unique disease entity in the first half of the 20th century. Cumulative knowledge of PG is based on a handful of case series and multiple individual case reports. To augment that knowledge, we present our experience with a large number of patients over a significant time. We performed a retrospective analysis of the medical records of 86 patients with PG who were evaluated and treated over 12 years at 2 university-based dermatology departments. The mean (+/- standard deviation) age of onset of PG and APG, respectively, was 44.6 +/- 19.7 years and 52.2 +/- 15.3 years. Lower extremity involvement was most common in PG, whereas upper extremity involvement was most common in APG. Associated relevant systemic diseases were seen in 50% of patients. Inflammatory bowel disease was the most common association in patients with PG, whereas hematologic disease or malignancy was most common in those with APG. Although a few patients were managed with local measures or nonimmunosuppressive treatment, the majority required oral corticosteroid therapy, often with systemic immunosuppressive treatment. PG patients required a mean 11.5 +/- 11.1 months of treatment to achieve remission compared with 9.0 +/- 13.7 months for patients with APG. Five patients (5.8%) had disease that was extremely refractory to multiple intensive therapies. The prognosis and disease associations for PG and APG appear to be different. Compared with PG, APG is more often associated with hematologic disease or malignancy, and remits more quickly.
Adolescent↗
Behçet's disease and complex aphthosis.
Behçet's disease is a complex multisystem disease diagnosed by means of clinical criteria. Clinical features include oral and genital aphthae, pustular vasculitic cutaneous lesions, and ocular, gastrointestinal, and vascular manifestations. We believe that complex aphthosis, characterized by oral or oral and genital ulcers, may be a forme fruste of Behçet's disease. Although the pathogenesis of both Behçet's disease and complex aphthosis remain unknown, immune factors, infectious agents, and effector mechanisms are implicated. Treatment is based on the severity of systemic involvement and includes topical therapies as well as colchicine, dapsone, thalidomide, and immunosuppressive agents.
Behcet Syndrome↗
Destructive procedures are the standard of care for treatment of actinic keratoses.
BACKGROUND: Actinic keratoses are premalignant lesions resulting from exposure to carcinogens. Recently, some Medicare carriers have limited reimbursement for destruction of actinic keratoses to those lesions unresponsive to topical 5-fluorouracil treatment. OBJECTIVE: Our purpose was to determine whether this policy meets the community standard of care for treatment of actinic keratoses. METHODS: Data from the 1993 and 1994 National Ambulatory Medical Care Survey were used to determine the frequencies at which different treatments are used for actinic keratoses. These were compared with the frequencies at which procedures and medical therapies are used to treat control conditions (warts, psoriasis, acne, and dermatitis) to determine whether procedures are done because they are available or out of medical necessity. RESULTS: Procedures were performed during 78% of visits for actinic keratoses. 5-Fluorouracil was used at 3.6% of visits, and at 39% of these visits a procedure was also performed. There were no observations of use of 5-fluorouracil alone at a first visit for actinic keratosis. Procedures were less likely to be performed at visits for warts, psoriasis, acne, or dermatitis, which indicates that reimbursable procedures are performed not simply because they are available. CONCLUSION: Procedures are performed to destroy actinic keratoses out of medical need. Medicare policies mandating initial use of 5-fluorouracil as initial treatment of actinic keratoses do not represent the community standard of care for treatment of these lesions.
Antimetabolites, Antineoplastic↗
Generalized pustular eruptions associated with oral terbinafine.
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Antifungal Agents↗
Mastocytosis with skin manifestations: current status.
AIM: To review our present knowledge about mastocyte origin, mastocytosis classification and management. METHODS: Literature review. RESULTS: Mastocytoses are chronic and recurrent disorders with symptoms which might either be limited only to the skin or to internal organs as well. The mastocytes, coming from bone marrow progenitor cells, migrate to tissues where they participate in inflammation and in cellular immunity as well as in the metabolism of connective and osseous tissues. Their proliferation causes the appearance of mastocytoses. The classification of the clinical manifestations of the mastocytoses into cutaneous, reactive (under the influence of the degranulator factors) and systemic disease, facilitates dialog among clinicians. Determination of prognosis and appropriate therapeutic regimens depend on individual features. CONCLUSIONS: Mastocytosis diagnosis is verified by histological study of skin lesion biopsy material. Management is symptomatic and unfortunately does not eradicate the disease.
Bone and Bones↗
An infant with firm, fixed plaques.
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Failure to Thrive↗
Livedoid vasculopathy: what is it?
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Humans↗
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↗
The efficacy of metronidazole 1% cream once daily compared with metronidazole 1% cream twice daily and their vehicles in rosacea: a double-blind clinical trial.
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Administration, Topical↗
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↗