[Malignant melanoma: classification and early diagnosis].
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Biomedical subjects
Publications and source records attributed to F Vakilzadeh.
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We report on a rare type of leucoplakia of the lip and the prepuce showing the morphological features of epidermolytic hyperkeratosis. The development of squamous cell carcinoma in one of the patients strongly suggests that epidermolytic leucoplakia may represent a precancerous disorder of the transitional epidermis.
A 5-year-old girl with a unilateral linear systematized skin lesion showing the clinical, histological and ultrastructural features of Hailey-Hailey disease is described. We suggest that this relapsing linear acantholytic dermatosis represents a new distinct entity within the spectrum of unilateral linear skin diseases.
Syringolymphoid hyperplasia with alopecia is a chronic patchy skin eruption consisting of hairless, anhidrotic, reddish-brown papules associated with pseudofollicular hyperkeratosis. Skin biopsies show dense lympho-histiocytic infiltrates aggregated in the dermis and around the hyperplastic sweat ducts and glands, imitating epithelial islands. Occasionally, obliteration of blood vessels may be found. This case report deals with a 52-year-old man suffering since 9 years ago from this distinctive skin disease.
To assess the significance of spontaneous regression in superficial spreading melanoma (SSM), 36 patients with clinical signs of regression in their primary tumor were compared to 200 patients with regular SSMs (controls). SSMs with regression were found to have the following, distinctive clinical features, which were significantly different from controls (P less than 0.05): (1) male predominance (69%), (2) preferential localization on the trunk (80.6%), (3) lower tumor thickness (Breslow), (4) clustering in Clark levels II and III, and (5) a larger surface area. The incidence of metastases was lower in patients with regressing SSMs (13.9%) compared to controls (20.5%) although the time until relapse was slightly shorter (20.6 months versus 28.1 months for controls). These prognostic parameters were not significantly different. However, of the patients who died, 2 of 4 with zones of regression had thin melanomas (less than or equal to 1.5 mm), compared to only 1 of 27 without regression zones (P less than 0.05). SSMs with regression therefore have unique clinical features, which may be related to their pathogenesis, and they may have some prognostic significance.
A 26-year-old man presented with an unusual skin lesion consisting of a hairless area on the left leg studded with reddish-brown papules, together with follicular hyperkeratosis and circumscribed anhidrosis. Skin biopsies showed dense lymphocytic infiltrates in the dermis and around hyperplastic sweat ducts and glands. Similar clinical and histological features have been described in two previous case reports. We propose the term syringolymphoid hyperplasia with alopecia for this unusual skin disease.
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A leukoplakic lesion of the lip showing the histologic features of epidermolytic hyperkeratosis above solar elastosis was investigated by electron microscopy. The ultrastructural alterations observed in the upper epidermal layers corresponded in the main with those of epidermolytic hyperkeratosis in other skin diseases. The keratinocytes showed irregularly formed tonofilaments, a marked intracellular edema, and premature cornification. In addition, there were discrete subcellular signs of premalignancy in the cells of the basal and suprabasal layers. These alterations suggest that the epidermolytic leukoplakia represents a rare histopathologic variant of actinic cheilitis.
A leukoplakic lesion of the prepuce is described with the histologic and ultrastructural features of epidermolytic hyperkeratosis. The lesion represents most likely a variant of epidermolytic leukoplakia recently observed in the vermilion border of the lip.
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Multiple dental cusps, protruding from the crowns of both deciduous and permanent incisors, were observed in a 4-year-old girl affected with hypomelanosis of Ito. This unusual dental anomaly has been described in one previous report of this neurocutaneous syndrome. In contrast to ordinary talon cusps, the dental outgrowths observed in hypomelanosis of Ito appear to be of hamartomatous origin. Apparently, these dental deformities are characteristic of hypomelanosis of Ito and may help to identify the syndrome in cases where other findings are minimal or atypical.
Reported in this paper are two patients on whom gestational herpes was diagnosed by clinical examination as well as by optico-immunofluorescent checks. Direct immunofluorescence technique has now enabled diagnosis with higher accuracy of gestational herpes, a dermatosis specific of pregnancy, with diagnosis proper being preceded by identification of C3 and C5 complement fixation. In both cases, symptoms had appeared in the second trimester. They had started from the umbilical region and then affected the entire trunk and extremities. In one of the patients, symptoms disappeared immediately after delivery, while exacerbation was recordable from the second in the puerperium. The pathological process was controlled in either case by topical application of corticosteroids. The symptoms of these cases, diagnosed by optico-immunofluorescent methods, are compared with clinical reports about gestational herpes cases in earlier literature.
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A wide-spread cactus dermatitis (Sabra dermatitis) occurred in a two-year-old child after contact with Opuntia microdasys.
A monoclonal gammopathy was observed in three patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. Deposition of monoclonal immunoglobulins in the skin was not detected by direct immunofluorescence microscopy. In contrast to scleromyxedema (lichen myxedematosus), from which scleredema can be distinguished clinically and histologically, the monoclonal immunoglobulins in two cases were of IgG2-kappa and IgG3-kappa type. Only one of the three patients had IgG1-lambda paraproteinemia, which is frequently seen in scleromyxedema. Our findings suggest that diffuse scleredema may be characterized by paraproteinemia but that the possible role of monoclonal immunoglobulins in the pathogenesis of this disease has yet to be resolved.
A 75-year-old male with eosinophilic pustulosis Ofuji complicated by superinfection with Pseudomonas aeruginosa and Proteus mirabilis is described. This is the first case of eosinophilic pustulosis with an antiepidermal antibody. This antibody was directed against the intercellular substance of the lower epidermis and it was detected by direct immunofluorescence in the patient's normal and lesional skin. As revealed by indirect immunofluorescence the patient's serum reacted with intercellular substance of human lower epidermis but not with guinea pig esophagus. Possibly the detected autoantibody is not characteristic for eosinophilic pustulosis Ofuji but rather an accompanying feature of this case similar to the antiepidermal antibodies found in patients with drug reactions and burns.
A subungual solitary glomus tumour was examined by both light and electron microscopy. By light microscopy, typical tumour tissue was seen, surrounded by connective tissue. By electron microscopy, a very close morphological relationship was noted between non-myelinated nerve fibres and mast cells. These mast cells could be divided into two groups: the mast cells of the first group contain numerous mature granules and show few lamellopodia. The distance to the nerve fibre bundles ranged between 2000 and 20 nm. In the second group, the mast cells always showed direct contact with nerve fibers. They had many lamellopodia and contained almost exclusively immature granules. In some cases, invaginations of lamellopodia and broader cytoplasmatic processes into the view that mast cells may play an important part in the function of neurons.