Clinical and experimental dermatology
-
Proteus syndrome (PS) is a complex hamartomatous disorder defined by local overgrowth (macrodactyly or hemihypertrophy), subcutaneous tumours and various bone, cutaneous and/or vascular anomalies (VA). VA are manifold in PS, but their prevalence is unknown so far. In order to further characterize PS, we studied the prevalence of VA in 22 PS patients presenting to our outpatient clinic and reviewed 100 PS patients previously reported between 1983 and 2001. ⋯ Unlike Klippel-Trenaunay syndrome, where VA are mostly confined to the hypertrophic limb, major arteriovenous anomalies are rare, and - similar to the other hamartomas and naevi observed in PS (pigmentary naevi, epidermal naevi, subcutaneous tumours, exostoses) - VA appear to be distributed at random sites on the body. We conclude that VA are among the most common findings in PS. Their varying type and distribution lend further support to the concept of somatic mosaicism.
-
Clin. Exp. Dermatol. · May 2004
The distribution of IgG subclasses in the lupus band suggests disease-specific alteration in subclass switching rather than polyclonal B-cell activation.
Deposition of immunoglobulins in the skin of patients with lupus erythematosus (LE), demonstrable as a linear band 'lupus band' at the basement membrane zone (BMZ) by direct immunofluorescence, was first described in 1963. Four decades after the discovery of the lupus band, a basic question regarding the origin of immunoglobulins of the lupus band is still unanswered. Is the lupus band just a manifestation of polyclonal B-cell activation commonly seen in systemic LE (SLE)? The distribution of IgG subclasses deposited in the skin of patients with SLE was identified using immunohistochemistry. The relative restriction of IgG of the lupus band to the IgG3 subclass demonstrated in this study provides evidence against polyclonal B-cell activation as the only cause of the lupus band and suggests disease-specific alteration in subclass switching.
-
Clin. Exp. Dermatol. · May 2004
Clinical TrialEfficacy of imiquimod 5% cream for basal cell carcinoma in transplant patients.
Imiquimod 5% cream has proven to be effective in superficial and nodular basal cell carcinomas in nonimmunosuppressed patients and treating squamous cell carcinomas in situ in transplant patients. The objective of this open-label study was to determine the efficacy of imiquimod 5% cream in treating basal cell carcinoma in transplant patients. At our unit, four renal transplant patients and one cardiac transplant patient were diagnosed with 10 basal cell carcinomas in 2001. ⋯ Five basal cell carcinomas received imiquimod 5% cream at night four times weekly for 6 weeks, without occlusion, and the other five tumours were treated on 5 nights per week for 5 weeks. Biopsies taken 6 weeks after the end of treatment showed no tumour in seven of 10 of the cases. Notably, all four superficial basal cell carcinomas, two of the three of nodular lesions and one of the three of infiltrative cases had completely cleared.