Kissing café-au-lait macule of the eyelids: report of the first case.
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Biomedical subjects
Publications and source records attributed to T Ruzicka.
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Methylprednisolone aceponate (MPA) has been shown to provide rapid, reliable and highly effective treatment of eczematous disorders, with an efficacy comparable to that of most reference topical corticosteroids. It also has excellent local and systemic tolerability. MPA is effective in the treatment of facial and scalp eczema and sunburn and has shown promising results in the treatment of psoriasis. Its rapid efficacy and lack of undesirable local and/or systemic side effects make MPA particularly suitable for use in children and infants. The wide range of formulations (0.1%) of MPA, including cream, ointment, fatty ointment, milk and solution, enable treatment to be tailored to the individual patient. In addition, MPA has the advantage of once-daily application compared with twice-daily treatment for other topical corticosteroids, thereby improving patient safety and promoting patient compliance but without compromising efficacy.
BACKGROUND: The effect of specific immunotherapy (SIT) on eczema in atopic dermatitis is not known. Therefore, a multi-centre, randomized dose-response trial, double-blind with respect to the efficacy of a biologically standardized depot house dust mite preparation was performed. METHODS: Eighty-nine adults with a chronic course of atopic dermatitis, SCORAD >or=40 and allergic sensitization to house dust mites [CAP-FEIA >or=3] were included, of whom 51 completed the study. Subcutaneous SIT with a house dust mite preparation (Dermatophagoides pteronyssinus/D. farinae) applying maintenance doses of 20, 2,000 and 20,000 SQ-U in weekly intervals for 1 year. The main outcome measures addressed the change of the SCORAD as average of the values after 9 and 12 months of SIT in comparison with the value at baseline. RESULTS: The SCORAD declined in the three dose groups in a dose-dependent manner (P = 0.0368, Jonckheere-Terpstra test) and was significantly lower in the two high-dose groups (2,000, 20,000 SQ-U) compared with the low-dose group of 20 SQ-U (P = 0.0379, U-test) after 1 year of SIT. The use of topical corticosteroids was significantly reduced with higher doses (P = 0.0007, Mantel-Haenszel chi-square test). CONCLUSIONS: Allergen-SIT for 1 year with a house dust mite preparation is able to improve the eczema in patients with atopic dermatitis who are sensitized to house dust mite allergens and reduces the need for topical corticosteroids. SIT may be valuable in the treatment of this chronic skin disease.
Cathepsin (Cat) L is an important lysosomal proteinase involved in a variety of cellular functions including intracellular protein turnover, epidermal homeostasis and hair development. Hurpin (serpinB13) is a cross-class specific serine protease inhibitor of Cat L. We have analysed the expression and localization of Cat L and hurpin in various inflammatory and neoplastic diseases by immunohistochemistry. Whereas Cat L expression in normal skin was below detection limit, immunoreactivity was detected in chronic inflammatory dermatoses. The highest expression of Cat L was found in psoriasis, atopic eczema and squamous cell carcinoma (SCC) samples. Samples of Lupus erythematosus, folliculitis, acne inversa, chronic leg ulcers and pyoderma gangrenosum demonstrated similar but lower expression for Cat L. In malignant cells of SCC, basal cell carcinoma and malignant melanoma, characteristic staining patterns were observed for Cat L, with more abundant expression at the periphery of the tumor. Expanding our previous work, we found that the expression of hurpin was confined mainly to the basal layer in normal skin samples, whereas hurpin was overexpressed and redistributed in diseased skin. The localization of hurpin in dermatoses and neoplasias differed from that in normal skin in that the highest expression was found in the outermost layers of the granular and upper spinous layers. Similarly to Cat L, the highest expression for hurpin was found in psoriasis and SCC. The results presented here summarize for the first time the expression of the protease Cat L and its inhibitor hurpin in a broad spectrum of skin diseases.
A 34-year-old female patient with a three year history of generalized granuloma annulare was treated systemically with dapsone (DADPS). Six weeks after the onset of treatment, the patient developed an extensive tonsillitis of the base of the tongue with fever and malaise. Routine laboratory work showed a leukocytopenia with agranulocytosis. Further investigation revealed a marked decrease of the enzyme activity of N-acetyltransferase 2, which plays an important role in dapsone metabolism. Treatment included the cessation of dapsone, antibiotic coverage, and G-CSF leading to the rapid improvement of symptoms and normalization of leukocyte counts. Dapsone-induced angina agranulocytotica is a rare event and is interpreted as an idiosyncratic reaction. Depending on genetic polymorphisms of various enzymes, dapsone can be metabolized to immunologically or toxicologically relevant intermediates. Because of the risk of severe hematologic reactions, dapsone should only be employed for solid indications and with appropriate monitoring.
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Lichen sclerosus et atrophicus is a chronic inflammatory disease that mainly affects women in the 5th decade. Although lichen sclerosus most often affects the anogenital region, it may occur in other cutaneous or mucosal sites. Increased fibroblast activity causes cutaneous sclerosis. Recent studies have identified lichen sclerosus as an autoantibody-mediated chronic inflammatory dermatosis. Autoantibodies against the extracellular matrix protein-1 are present in up to 80% of affected patients. In addition to the well-accepted therapy with potent corticosteroids, promising results have been obtained using calcineurin antagonists in the treatment of lichen sclerosus. Interdisciplinary management with regular monitoring can improve the clinical manifestations and quality of life.
Since their approval, topical calcineurin inhibitors have proven to be effective medications in the treatment of atopic eczema. On March 10, 2005, the Food and Drug Administration (FDA) followed the advice of the Pediatric Advisory Council and issued a "black box warning" for the use of Protopic (Tacrolimus) und Elidel (Pimecrolimus). This FDA alert has caused worldwide uncertainty among parents and patients. Several dermatological societies have issued critical position statements. This report reviews the existing information underlying the warning and places it into the context of current knowledge.
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