Sézary syndrome and follicular mucinosis.
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Biomedical subjects
Publications and source records attributed to J K Rivers.
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Survival for melanoma patients with thick primary tumours is notoriously short. A small number of patients with tumours greater than 5.5 mm thick do, however, have protracted survival intervals. Attempts were made to account for this phenomenon by means of histological, cytometric and HLA serotyping analyses. Patients with thick lesions surviving more than 10 years were matched--by sex, age, anatomical site of primary lesion, stage of disease and, whenever possible, by initial surgical therapy--to patients dying of their disease within 5 years. This case-control study on 13 long-term survivors and 13 short-term survivors did not show that any of the following attributes of the primary lesion were useful in predicting survival: Clark's level of invasion, ulceration, mitotic rate, host inflammatory response, tumour regression, tumour necrosis, vascular invasion, satellitosis, radial or vertical growth phase, predominant cell type, histogenetic type, borders, DNA quantification and cytomorphometry. HLA serotyping of long-term survivors showed an excess of antigen DQw1 compared with the general population, although this excess was not statistically significant.
Two women developed well-demarcated eczematous and erythematous plaques localized to the injection sites of subcutaneous preservative-free heparin 72-96 h after heparin administration. The plaques resolved within a week of discontinuing the therapy. Neither epicutaneous testing with preservative-free heparin nor in vitro proliferation assays to heparin and two low molecular weight glycosaminoglycans could elicit a response in either patient. In contrast, both patients developed localized eczematous plaques 48-96 h after re-challenge with intradermal and/or subcutaneous heparin. Delayed hypersensitivity reactions to subcutaneous heparin are uncommon and have not been reported in the Australasian medical literature. Given the frequency with which heparin preparations are used, it is important for physicians and surgeons to be aware of this potential adverse reaction.
The clinical features of 100 dysplastic nevi were tabulated. Although certain characteristics were present in most or all of these melanocytic nevi, there was a marked heterogeneity of other clinical features. The preponderant type of large (greater than or equal to 8 mm) melanocytic nevus in patients with classic dysplastic nevi is a papule or plaque with the following characteristics: multicoloration (various shades of tans, browns, reds, or black); slightly raised height for its broad diameter; mamillated surface; and lack of hypertrichosis. An atlas illustrates some of the clinical varieties of melanocytic nevi in this syndrome.
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A total of 452 patients with dysplastic nevi (DN) were followed prospectively by repetitive, complete cutaneous examinations in order to determine the clinical features of early malignant melanomas (MM) arising in them. Sixteen patients (3.5%) developed 18 newly diagnosed MM during an average follow-up period of 27 months. Twelve of the 18 MM were in situ and all of the primary invasive MM diagnosed prospectively in this follow-up were less than 0.89 mm in Breslow thickness, implying an excellent prognosis. The principal clinical clue to the diagnosis of MM was change in a preexisting pigmented lesion. Total-body photographs were very useful in helping to identify the early MM in these patients.
A group of 420 neonates underwent total cutaneous and oral mucosal examinations during the first week of life. Skin lesions were seen in almost every baby (99.3%). The eight most common dermatoses were desquamation (65.0%), Epstein's pearls (56.0%), sebaceous hyperplasia (48.0%), milia (36%), toxic erythema (34.8%), salmon patch (33.8%), hypertrichosis (29.0%), and Mongolian spot (25.5%). Congenital melanocytic nevi were clinically diagnosed in 9 of 420 babies (2.1%); the majority of the lesions were small, that is, less than 1.5 cm in diameter. These neonates had a dark complexion (all had brown or black hair, and most had an olive skin color) and came from families with no previous history of cutaneous melanoma. In contrast, all 19 babies with a previous family history of melanoma had a fair complexion (blond or light brown hair and alabaster skin color) but no congenital melanocytic nevi. These findings may suggest that small congenital melanocytic nevi are markers for persons with a decreased risk of melanoma, because dark-skinned persons are at a lower risk. On the other hand, small congenital melanocytic nevi may be precursors of melanoma. Only prospective studies will determine the magnitude of this risk and thereby optimize management.
We quantified the histologic features of 100 consecutive dysplastic nevi. Although there is heterogeneity in the microscopic attributes of dysplastic nevi, certain features that are seen repeatedly should enable the histologic identification of these melanocytic neoplasms. These features include a central dermal nevocytic component with a peripheral extension of a junctional component, elongated epidermal rete ridges, bridging of nests of melanocytes at the dermo-epidermal junction, nests of melanocytes at the sides of rete ridges as well as at their bases, and concentric eosinophilic fibrosis. Cytologic features of melanocytes were not as valuable as architectural features of individual nevi in classifying these lesions. We also provide an atlas of selected histologic features that depicts some aspects of this heterogeneity.
Corticosteroids may mediate some of their anti-inflammatory effects via induction of a specific 38 kDa protein, lipocortin-I. Anti-lipocortin-I antibodies (ALA) were measured by enzyme-linked immunosorbent assay (ELISA) in 23 patients with plaque-type psoriasis alone (NAP), in 21 patients with psoriasis and arthritis (PA), and in 67 healthy controls. Only two of 23 NAP patients had elevated ALA, whereas six of 21 PA patients had raised levels of ALA (P = 0.2). Sero-negative polyarthritis was the most common pattern of joint disease in those PA patients with elevated ALA (4/6). ALA levels did not correlate with the extent or severity of cutaneous involvement, and are unlikely to be involved in the pathogenesis of cutaneous psoriasis.
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A case of naproxen-induced bullous photodermatitis ("pseudoporphyria") is presented. A 37-year-old woman with systemic lupus erythematosus developed tense bullae on her hands and lips, and was found to have a clinical and histological condition that was similar to porphyria cutanea tarda without the associated haematological abnormalities. The lesions remitted upon the cessation of naproxen therapy. Although this adverse effect of naproxen has been seen most frequently in patients from Australia and New Zealand, it has not been reported in the Australian medical literature previously.
A total of 452 white patients, classified into four dysplastic nevi groups, were followed prospectively by repetitive, complete cutaneous examinations using total-body photographs taken on entry into the study. Sixteen patients (3.5%) developed 18 newly diagnosed malignant melanomas (MM) during an average follow-up period of 27 months. Twelve of the 18 MM were in situ, and all of the six primary invasive MM diagnosed prospectively in this follow-up were less than 0.89 mm in Breslow thickness, implying an excellent prognosis. Compared with reference populations, the number of MM detected significantly exceeded the number estimated to occur in the comparable age-matched control groups. These data support the concept of repetitive follow-ups of all groups of patients with dysplastic nevi.
Variables were compared in patients with clinical stage I superficial spreading melanoma who had been divided into three age-groups. The study included 736 consecutive patients who were prospectively entered into the data base of the Melanoma Cooperative Group of New York University Medical Center. Compared with the younger patients ( less than 40 years), older patients (greater than or equal to 60 years) had superficial spreading melanomas that were, on average, of greater thickness, level, and diameter. Younger patients were more likely than older patients to show evidence that their melanomas arose from preexisting nevocytic nevi. There was no difference in the 10-year survival between groups when melanomas were matched by thickness. Thus it is as important to perform periodic total cutaneous examinations in the elderly as it is in younger persons, and age alone should not determine management strategies.
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The effects on 31 normal subjects following exposure to sunbeds containing UVA lamps with minimal UVB emission have been compared in a double-blind study with the effects on nine control subjects of a similar exposure course three times weekly for 4 weeks to sunbeds emitting visible light. On previously untanned areas, all those subjects on active treatment developed a mild tan; in tanned areas they all developed a moderate tan, while all control subjects developed a minimal to mild tan. The mean protection factor against later UVB-induced erythema was 3.2 +/- 0.3 after the active course and 1.6 +/- 0.2 among the controls. Significantly more frequent adverse cutaneous effects for active subjects were pruritus, erythema, freckling, burning sensation, dryness and polymorphic light eruption. Cutaneous Langerhans cell numbers, and blood CD3+ (pan T-cell) and CD4+ (helper T-cell) lymphocyte subsets were reduced in both active and control groups. CD8+ (cytotoxic/suppressor T-cell) counts were similarly but not significantly reduced in both groups. Pityrosporum yeast counts were significantly reduced in both groups. The changes found in both groups seem attributable to small amounts of UVB emission from both active and control lamps.