The surgeon, genetics, and malignant melanoma.
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Biomedical subjects
Publications and source records attributed to R M Fusaro.
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Since window glass absorbs sunlight below 320 nm, it provides a means of assessing sensitivity to longer wavelengths, i.e. UVA and visible radiation. Positive responses to the query of whether symptoms develop in the auto with the windows up must now be interpreted with regard to the possible presence of tinted plastic film on side and rear windows. These films block nearly all UVA radiation, as does the plastic interleaf of windshields. Thus, occupants of an auto equipped with plastic film receive photoprotection from UVB radiation and well into the UVA region. We define three classes of topical sunscreens: (1) conventional UVB screens, (2) broad-spectrum preparations containing a UVB screen and a UVA absorber and (3) browning agents such as dihydroxyacetone (DHA) that produce a skin coloration that absorbs in the low end of the visible region, with overlap into long-wavelength UVA. By considering responses of photosensitive persons in autos with tinted or untinted windows, coupled with efficacy of appropriate sunscreens, we produced an algorithm defining three photosensitivity subsets. Persons sensitive to long-wavelength UVA and/or visible radiation will benefit from tinted auto windows. In particular, patients with lupus erythematosus (LE) have actively promoted legislation allowing tinted windows. Support for their position is documented by recent reports of induction of lesions in LE patients by exposure to UVA and visible radiation. The brown color produced by DHA is a useful adjunct to the screening action of broad-spectrum sunscreens. Development of a durable color overnight allows application of the DHA preparation in the evening, thus eliminating possible interference with sunscreen use during the day.
There are no premonitory physical signs or biomarkers which can identify the genotypic status in Lynch syndrome II. Diagnosis is therefore dependent on the pedigree, with attention to cancer of all anatomic sites, inclusive of those cardinal features of its natural history. The tumor spectrum in Lynch syndrome II has continued to expand commensurately with increasing interest in this disorder. We report a family showing the constant cancer features of this syndrome but, in addition, occurrences of carcinoma of the bile duct, urologic system, and extremely early-onset carcinoma of the pancreas, in patients in the direct genetic lineage who were considered to be candidates for having inherited the deleterious genotype. Diagnosis of Lynch syndrome II is crucial in targeting its surveillance and management.
We evaluated the photoprotective efficacy of a broad-spectrum sunscreen containing a UVA screen (butyl methoxydibenzoylmethane) and a UVB screen (octyl dimethyl p-aminobenzoic acid) in patients with hereditary polymorphic light eruption. At least 18 of the 30 patients who enrolled in the study were sensitive to sunlight through window glass, an indication of UVA sensitivity. Of the 21 patients who completed the clinical trial, the physician's evaluation was that 18 (86%) obtained good to excellent results. Self-evaluation by the patients revealed that 16 (76%) noted more photoprotection than from previous treatments.
The role of host factors in the etiology of pancreatic cancer has received a paucity of systematic investigation. Anecdotal reports and one population-based study have supported the concept that familial clustering of this disease exists. We have studied a kindred with a cancer-associated genodermatosis known as familial atypical multiple mole melanoma (FAMMM) syndrome (hereditary dysplastic nervus syndrome). Three key relatives have manifested pancreatic carcinoma. Since FAMMM may account for as much as 10% of the total malignant melanoma burden, its association with pancreatic cancer harbors important public health implications. Given the fact that the etiology of pancreatic cancer remains enigmatic, it is important to investigate all possible clues to its causality, including the potential role of host factors.
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Two cases of mucinous cysts of the vulva are reported. The embryology of the lower female genital tract is described, including the Müllerian duct system, cloaca and urogenital sinus. The relationship of the Wolffian duct system is also included in the discussion. It has been felt in the past that these vulvar cysts are Müllerian-derived. However, it is now deemed more plausible that they are from the entodermally-derived urogenital sinus epithelium. These cysts pose no threat to the patient but are interesting from the standpoint of embryogenesis.
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The FAMMM syndrome consists of the familial occurrence of cutaneous malignant melanoma and atypical nevi (dysplastic nevi), and is inherited as an autosomal dominant trait. Conflicting results have been reported on the question of whether the syndrome includes increased susceptibility to non-melanoma cancers. We have studied cancer of all anatomic sites and histologies in nine FAMMM families which were ascertained in a pigmented lesions clinic in the Netherlands. We evaluated two hypotheses: that the number of systemic cancers observed in the families was excessive, compared to expected incidence, based on Dutch incidence data, and that there was variation (or heterogeneity) among families in the frequency of systemic cancer. A significant excess of systemic cancer (especially digestive tract cancer) was observed. Significant heterogeneity was also found among the families; three of the nine families had marked excess in numbers of systemic cancers, and the remaining families had normal numbers of cancers among the known FAMMM gene carriers and their first degree relatives. Thus, we provide evidence of increased susceptibility to systemic cancer occurring in conjunction with the FAMMM syndrome in a subset of this resource.
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A flexible system of classifying topical corticosteroid proprietaries is proposed to help the clinician understand the relative antiinflammatory potency of these products. This system has the advantage of indicating not only the relative potency of a specific proprietary preparation but also noting the number of divisions in the classification. It allows for new product development and keeps the clinician informed of the change by the number of divisions being used. The system also has the potential for the classification of relative severity of both local and systemic side effects of corticosteroids.
Three case reports of primary periorbital dermatitis are presented. The etiology of these were molluscum contagiosum, Microsporum canis, and Coccidioides immitis. In each case, the presenting complaint was unusual since the primary lesion started in the periorbital region.
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Dihydroxyacetone (DHA), a browning agent, protects photosensitive rats and humans against long ultraviolet radiation (UVA, 320-400 nm) and visible (blue) light. The photoprotective efficacy of DHA and structurally similar compounds was assessed as prevention of edema in the paws of psoralen-sensitized rats, after exposure to blacklight fluorescent lamps. Methylglyoxal produced a yellow-brown color and provided nearly the same protection as DHA, whereas monohydroxyacetone did not color the skin and afforded little or no protection. Glyceraldehyde provided a moderate amount of protection, which was enhanced by prior exposure of the agent to alkaline pH. A solution of 5-hydroxymethylfurfuraldehyde was yellow and provided minimal protection by staining the skin rather than browning it. We conclude that the ability to produce a brown color in skin is a useful criterion for screening compounds for photoprotective efficacy against UVA radiation.
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