Biomedical subjects
Publications and source records attributed to A Trattner.
We report a case of the association of blue rubber bleb nevus syndrome with diffuse angiokeratoma, without any evidence of enzyme deficiencies. The lesions of both disorders had a late-onset appearance.
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Eccrine poroma is a benign, slow growing, solitary adnexal tumor. Malignant degeneration may take place in longstanding solitary lesions; in such cases cutaneous and fatal visceral metastases have occurred. Our goal was to determine whether flow cytometry yields useful diagnostic and prognostic information on benign and malignant eccrine poroma. Flow cytometric analysis of the nuclear DNA ploidy pattern was performed on four samples of eccrine poroma and five samples of malignant eccrine poroma. All the histograms of the eccrine poromas were diploid. Two of five specimens (40%) of the eccrine porocarcinoma were aneuploid, and the other three (60%) were diploid. The diploid pattern represents another expression of the benignancy of eccrine poroma. Since abnormal DNA content is often correlated with tumor grade, the aneuploid DNA histogram of 40% of the patients with malignant eccrine poroma is not a surprising finding in this cytologically malignant neoplasm.
Flow cytometric DNA analysis of various tumours has indicated a correlation between the degree of malignancy and ploidy; results which could have clinical significance. We analysed the ploidy of Kaposi's sarcoma (KS) tumours, and classified the results according to clinical history and histological findings. We found that patients on steroid treatment had an aneuploid pattern, and most of the patients with classic-type KS had a diploid pattern on flow cytometry.
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A 57-year-old woman who had undergone lumpectomy for infiltrating duct-cell carcinoma of the right breast was found to have morphea after receiving radiation therapy. Three years later she developed a vesicular eruption on the area of the morphea, which was diagnosed as bullous morphea. The histologic findings of the vesicular component were characteristic of lichen sclerosus et atrophicus. It is suggested that in some cases bullous morphea represents a secondary appearance of bullous lichen sclerosus et atrophicus on a lesion of morphea.
Darier disease is an autosomal dominant abnormality of epidermal differentiation characterized clinically by the presence of hyperkeratotic papules on the skin and histologically by the loss of cell cohesion and by disorderly keratinization. Two groups recently found evidence that the gene whose mutations underlie this disease is located at chromosome 12q23-q24.1, a site on chromosome 12 that clearly is distal to the type II keratin gene cluster. We report here evidence for sublocalization to a 5-cM region of that site in an additional ten families of European and Middle Eastern ancestry with a combined lod score in excess of 20.
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BACKGROUND: Darier's disease is an acantholytic dyskeratotic genodermatosis with autosomal dominant inheritance. A predictive diagnostic marker for this disorder would be beneficial because of the relatively late onset and the large number of sporadic cases of the disease. The dermatoglyphic features of patients with Darier's disease were examined to determine whether they have a common pattern. METHODS: Ink prints of fingers and palms obtained from 11 patients of both sexes with sporadic and familial Darier's disease were analyzed and compared with those of normal subjects. RESULTS: No significant quantitative or qualitative differences were found between the dermatoglyphic features of our patients and those of a healthy population, except for punctate interruptions of the skin ridges that indicate pitting, a well-known manifestation of Darier's disease. CONCLUSIONS: These results refute the conclusions of a previous publication claiming that there is a common characteristic dermatoglyphic feature in patients with this dermatosis.
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Low-dose low molecular heparin inhibits T lymphocyte-mediated autoimmune disease and allograft reactions in mice in vivo and in vitro. High doses of heparin are not effective. The purpose of this preliminary study was to analyze the effect of low-dose, low molecular weight heparin (Enoxaparin, Clexane) on the expression of patch testing in patients with contact dermatitis. Eleven patients with allergic contact dermatitis, and positive patch tests reactions, were given a single subcutaneous injection of Clexane 3 mg (0.03 ml) and were reevaluated for positive reactions after the injection. Eight out of 21 positive reactions (38%) became negative after the injection and 4 out of 21 reactions (19%) changed from ++ before the injection to + after. An impressive improvement was observed in 3 patients with chronic allergic contact dermatitis. Low-dose low molecular weight heparin inhibits the elicitation of allergic contact dermatitis.
A patient noted the appearance of a few asymptomatic lesions on both areolae eighteen months after delivery of her last son. Histologic examination showed typical seborrheic keratosis. A brief review of the differential diagnosis of skin problems of the nipple and the areola is provided.
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BACKGROUND: Four epidemiologic types of Kaposi sarcoma (KS) are known: classic KS, endemic African KS, epidemic or acquired immunodeficiency syndrome-related KS, and KS associated with immunosuppressive therapy. In most of the latter patients, KS was reported to have developed after organ transplantation, particularly renal transplantation. Thirty-nine patients who have not had a transplant have been reported to have KS associated with corticosteroid therapy. METHODS: The authors studied 10 patients with the appearance of KS during corticosteroid therapy (6 men, 4 women; age range, 42-79 years) who were treated with corticosteroids for autoimmune disorders (5 patients), lymphoproliferative disorders (2 patients), and diseases unrelated to the immune system (3 patients). RESULTS: Genetically programmed susceptibility to corticosteroid-related KS was suggested by the descent of the study patients as well as most of those reported previously. The prognosis was guarded in all the study patients. CONCLUSIONS: Corticosteroids should be withdrawn to achieve clinical remission.
BACKGROUND: von Willebrand factor (vWF) is synthesized almost exclusively by endothelial cells and is stored there as ultra-high-molecular-weight multimers. The vWF multimers that are detected in the plasma are smaller than those stored within the endothelium. In two previous studies, comprising small series of cases with classic Kaposi's sarcoma (KS), an endothelium-derived tumor, increased levels of plasma von Willebrand factor antigen (VWF:Ag, the antigenic structure) were reported, suggesting that vWF:Ag may be a marker of endothelium proliferation. OBJECTIVE: Our purpose was to investigate the quantitative as well as qualitative alterations of plasma vWF in a large series of patients with classic KS at various stages of the disease. METHODS: Levels of plasma vWF:Ag were studied in 38 patients with classic KS confined to the skin at various stages of the disease and compared with a control group. Thirty-three patients had active KS (i.e., with skin lesions) and five were in remission. In five patients with active KS multimeric analysis of plasma vWF was also performed. RESULTS: The levels of vWF:Ag were significantly higher among KS patients than in the control group (n = 29, p < 0.01). Levels of vWF:Ag were also significantly higher in patients with active disease as compared with those in remission (p < 0.05). No correlation was found between vWF:Ag levels and the extent of KS. Analysis of the multimeric pattern of plasma vWF showed enhanced staining of all bands, particularly the intermediate and high molecular weight forms, which resemble the endothelial forms as opposed to normal circulating vWF multimers. CONCLUSION: Quantitative as well as qualitative alterations in plasma vWF were found in patients with KS, which may reflect the destruction or activation of endothelial cells within the lesions. vWF:Ag may serve as a marker of disease activity in classic KS; however, it is not a good marker for the extent of the disease.
BACKGROUND: We review our experience with nine patients with partial unilateral lentiginosis (PUL), a rare pigmentary disorder. OBJECTIVE: Our purpose was to define the characteristics of PUL and to discuss the differential diagnosis. METHODS: The records of nine patients with PUL were reviewed. A literature review on diagnosis, association with other disorders, and differential diagnosis is presented. RESULTS: PUL is a rare benign disorder that has no known inheritance pattern and has no commonly associated abnormalities. CONCLUSION: Careful history and physical examination may enable the distinction between PUL, nevus spilus, and other more serious genetic disorders associated with lentiginosis.