Primary cutaneous cryptococcosis in an elderly man.

Cryptococcus neoformans is an opportunistic yeast with a worldwide distribution. Although well recognized as a cause of cutaneous disease in immunocompromised patients, this rarely occurs in healthy hosts. In this article, we present the case of a farmer who developed cutaneous cryptococcosis at the site of an injury he sustained while cleaning out his barn. Despite an extensive work-up, no evidence of disseminated disease or underlying immunosuppression was found. The patient recovered completely several weeks after beginning treatment with oral fluconazole.

A case of dermatitis herpetiformis with IgA endomysial antibodies but negative direct immunofluorescent findings.

A patient with clinical findings of dermatitis herpetiformis (DH), negative direct immunofluorescent (DIF) findings for junctional IgA deposits in 2 biopsy specimens, and positive for IgA endomysial (AEmA) and tissue transglutaminase (tTG) antibodies responded initially to dapsone. After dapsone had to be discontinued because of side effects, a gluten-free diet and supportive therapy controlled the disease; the AEmA and tTG antibodies became negative. Our data on 10 consecutive DH cases examined by DIF and by serum studies for AEmA and antibodies to tTG, point to frequencies of 90% DIF positive and 70% AEmA and tTG positive cases. The use of both DIF and serum tests for AEmA and tTG reveals DH cases not detected by DIF alone that respond to gluten-free diet. Findings on autoantibodies to tTG, an enzyme that metabolizes gliadin, points to a role of tTG in the immunopathology of gluten-sensitive enteropathy and helps to explain the need for a gluten-free diet in the management of DH cases.

Congenital and childhood dermatofibrosarcoma protuberans: a case report and review of the literature.

Dermatofibrosarcoma protuberans (DFSP) occurs most commonly on the trunk, affects all races, and often develops between the second and fifth decades of life. It is uncommon in childhood and is sometimes mistaken for a vascular lesion, as it often presents as a blue macule or small nodule. Review of the English literature revealed approximately 152 cases of DFSP developing before 16 years of age and only 19 claimed congenital cases. A case of congenital dermatofibrosarcoma protuberans is presented with a literature review and discussion of congenital and childhood presentations of the tumor.

Nonsteroidal anti-inflammatory-induced pseudoporphyria: is there an alternative drug?

Drug-induced pseudoporphyria cutanea tarda has been attributed to many different medications including several nonsteroidal anti-inflammatory drugs (NSAIDs). Often the patients taking these anti-inflammatory medications became part of a treatment dilemma when one of the drugs is deemed the cause of a blistering disease. We present a prototypical case of NSAID-induced pseudoporphyria cutanea tarda and suggest alternative NSAIDs that may be safely used in these patients.

Cowden's syndrome, Lhermitte-Duclos disease, and sclerotic fibroma.

The authors report a case of Lhermitte-Duclos disease, or dysplastic cerebellar gangliocytoma, in which a cutaneous sclerotic fibroma was found incidentally during the second resection of a recurrent cerebellar hamartoma. The association of Lhermitte-Duclos disease and sclerotic fibroma with Cowden's syndrome led to a dermatologic examination and confirmation of the diagnosis of Cowden's syndrome. The combination of both Lhermitte-Duclos disease and sclerotic fibroma with Cowden's syndrome has not previously been reported. A review of the 15 cases of Lhermitte-Duclos disease associated with Cowden's syndrome shows no significant gender predilection. Sclerotic fibromas, once thought to be specific for Cowden's syndrome, also are reviewed.

Consensus workshop on the toxic effects of long-term PUVA therapy.

The possibility that there is an increased risk of melanoma in patients with psoriasis treated with psoralen-UV-A (PUVA) therapy has raised concern on the part of physicians and patients about the long-term safety of this treatment. In response to this concern, the National Psoriasis Foundation sponsored a workshop at which invited participants with expertise in PUVA therapy, psoriasis treatment, melanoma and nonmelanoma skin cancer, and epidemiological and clinical trials were asked to develop a consensus on the following 3 issues: the risk of long-term adverse effects of PUVA therapy with emphasis on nonmelanoma and melanoma skin cancer; the guidelines for physicians and patients for selection and use of PUVA therapy with consideration of the risk-benefit ratio of this treatment compared with the risk-benefit ratios of alternative treatments; and the directions for further evaluation of the long-term effects Of PUVA therapy.

Impetigo herpetiformis and Staphylococcus aureus lymphadenitis in a pregnant adolescent.

We report a 15-year-old primagravida female with a history of chronic plaque psoriasis who developed impetigo herpetiformis at 28 weeks gestation. Culture of a needle aspirate from a tender, enlarged cervical lymph node grew Staphylococcus aureus. The patient improved rapidly on wet dressings, topical midpotency corticosteroids, and intravenous nafcillin. The remainder of her pregnancy was uncomplicated. We speculate that both pregnancy and infection led to this pustular flare of her psoriasis.

The safety of etretinate as long-term therapy for psoriasis: results of the etretinate follow-up study.

BACKGROUND: Etretinate is an aromatic retinoid given orally to treat severe psoriasis, a chronic disease that often requires long-term therapy. OBJECTIVE: We assessed the safety of long-term therapy with etretinate for psoriasis. METHODS: This 5-year prospective study of a cohort of 956 patients with psoriasis treated with etretinate assessed the frequency of adverse events in relation to total use and in relation to the frequency of these events in control populations. RESULTS: Our data do not provide evidence for an increased risk of cardiovascular disease, cancer, diabetes, or inflammatory bowel disease in association with long-term etretinate use. Although some patients reported that joint problems improved with the use of etretinate, a greater number associated the use of etretinate with joint problems. CONCLUSION: With proper patient selection and monitoring, long-term etretinate therapy (up to 4 years) does not appear to be accompanied by a substantial increased risk of major adverse effects.

Assessing the preferences of patients with psoriasis. A quantitative, utility approach.

BACKGROUND AND DESIGN: Patient preferences for health outcomes can be explicitly assessed and expressed in quantitative terms known as utilities. Three standard methods for utility assessment have been used to quantify patient preferences, but these methods have not previously been applied to skin disease. Eighty-seven patients with psoriasis from a tertiary medical center were interviewed, using an interactive, computer-based utility assessment questionnaire, U-Titer. Utilities for three categories of psoriasis severity and potential adverse outcomes of methotrexate therapy were assessed by the vertical rating scale, time trade-off, and standard gamble. RESULTS: Patients assigned a broad range of utilities for each of the health states. Utilities obtained by the vertical rating scale did not correlate well with utilities obtained by standard gamble or time trade-off methods. However, utilities assessed by standard gamble and time trade-off were not significantly different. Patient characteristics such as age, gender, and education were not correlated with utility and did not explain the variation. Indicators of the patients' disease severity were not predictive of utilities for the assessed health states. The relatively high utility for liver biopsy suggests that there is less patient aversion to the procedure than suspected. CONCLUSIONS: Utilities, or quantitative measures of patient preferences for health states, are measurable and vary widely for mild, moderate, and severe psoriasis and possible adverse outcomes of methotrexate treatment. The process of elucidating individual patient utilities for various health outcomes can be used to incorporate patient preferences into the process of clinical decision making. Guidelines that are based solely on severity of symptoms, without input from patients on how they value such symptoms, must be questioned.

Detection of hepatotoxicity associated with methotrexate therapy for psoriasis.

To determine whether liver function tests and clinical and demographic information would predict methotrexate-associated hepatotoxicity, we identified 78 patients who had undergone 147 liver biopsies associated with methotrexate therapy for psoriasis. The joint sensitivity of aspartate aminotransferase, alkaline phosphatase, and total bilirubin values in detecting abnormal results from a biopsy specimen obtained after treatment was .86; the predictive value of negative test results was .93. A logistic regression model significantly predicted the presence of abnormal (grade III or higher) liver biopsy specimen results. The concordance index was .92 (perfect, 1.0). Regression coefficients may be used along with information from a specific patient to calculate the predicted probability of an abnormal result from a liver biopsy specimen after treatment. We conclude that this multivariate risk estimation model significantly predicts the likelihood of positive findings from liver biopsy specimens in this patient population. The clinical use of this model awaits further validation.