Cases reported "Edema"

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1/10. Rofecoxib-induced instant aquagenic wrinkling of the palms.

    An 18-year-old woman presented with a 3-week complaint of exaggerated palmar wrinkling and swelling following brief exposure (1-2 minutes) of her hands to water. She had a history of mixed connective tissue disease and had been started on rofecoxib therapy 1 month prior to the onset of her skin symptoms. Discontinuation of rofecoxib was followed by resolution of symptoms within a period of 3 weeks. Similar palmar skin changes following water exposure have been reported to occur in cystic fibrosis and are thought to be due to increased salt content of the skin and secondary increased water-binding capacity. Rofecoxib is a selective COX-2 inhibitor that has been shown to increase sodium reabsorption in the kidney via effects on prostaglandin E2 and the renal vasculature. The COX-2 protein is also expressed in keratinocytes and plays a role in keratinocyte differentiation. Prostaglandin E2 also plays a role in keratinocyte proliferation and differentiation. Thus rofecoxib may cause increased sodium reabsorption in the skin, as it does in the kidney. The rofecoxib-associated elevation in skin sodium may increase keratin water-binding capacity and cause exaggerated aquagenic wrinkling of the skin, as occurs in cystic fibrosis.
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2/10. Inflammatory familial palmoplantar keratoderma: Greither's disease?

    A case of a familial palmoplantar inflammatory keratoderma with autosomal dominant inheritance is reported. Associated clinical features included vasomotor troubles and hyperhidrosis consistent with a diagnosis of Greither's disease. light microscopy was nonspecific. Electron microscopy showed aggregated tonofilaments around the nucleus, without true clumps. desmosomes were numerous and cell-cell junctions showed an imbricated pattern, well demonstrated in the stratum corneum. The diagnosis of Greither's keratoderma is discussed.
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3/10. Aquagenic syringeal acrokeratoderma.

    A case of a 32-year-old woman with aquagenic syringeal acrokeratoderma is presented. This case is the eleventh to report this condition. As with previously reported cases, the condition presents in young women and results in edema of the palms with visibly prominent eccrine ducts after brief exposure to water. The patient responded to aluminum chloride applied topically. Prior cases are reviewed.
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4/10. Aquagenic syringeal acrokeratoderma: report of a case with histologic findings.

    Aquagenic syringeal acrokeratoderma is a rare acquired condition characterized by painful symmetric swelling and hypopigmentation of the palms and lateral fingers, which develops after brief exposure to water. Histopathologic examination suggests that an aberration in the eccrine sweat gland apparatus may be the underlying cause of this condition. The "hand-in-the-bucket sign," in which patients arrive in their physician's office with their hand in a bucket of water to more readily demonstrate their lesions, is such a common presentation that it almost can be regarded as pathognomonic. All 12 cases reported to date have been in young females. We report a case of aquagenic syringeal acrokeratoderma in a male with unique histologic findings.
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5/10. Intra-arterial injection of dextropropoxyphene into hand arteries.

    We report a case of deliberate intra-arterial injection of dextropropoxyphene into the palms, leading to chronic oedema of both hands. The aetiology is discussed. This cause must be borne in mind in the diagnosis of chronic oedema of the hands.
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6/10. Erythroderma with spongiotic dermatitis. association with common variable hypogammaglobulinemia.

    Two middle-aged men presented with generalized erythroderma, diffuse alopecia, and hyperkeratosis of the palms and soles. Histopathologic study demonstrated spongiosis (epidermal intercellular edema) with a perivascular lymphohistiocytic infiltrate. Complete immunologic evaluation demonstrated that both patients had panhypogammaglobulinemia and markedly depressed in vitro pokeweed mitogen-induced immunoglobulin secretion. One of the patients also showed poor lymphocyte responses in vitro to T cell mitogens and antigens and had a decreased ratio of helper to suppressor cells. In both patients, the cutaneous lesions improved with systemic corticosteroids, but no significant alteration in the immunologic abnormalities was observed. This report illustrates that chronic erythroderma may be the presenting clinical manifestation of common variable hypogammaglobulinemia.
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7/10. Neu-Laxova syndrome: report of two cases.

    Two cases of Neu-Laxova syndrome are reported. The pathological features are described. There is marked swelling of the palms and soles, which is due to massive fat and myxomatous connective tissue proliferation in addition to edema. lissencephaly with hypoplasia of cortical descending fibers is observed, along with cerebellar hypoplasia. The occurrence of 2 microcephalic male and female infants dying shortly after birth in the same sibship with 1 normal baby in between confirms similar findings by others and supports an autosomal-recessive inheritance. The relevance of pathological examination in further delineation of the phenotypic appearance is discussed.
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8/10. Dermatologic signs in toxic shock syndrome--clues to diagnosis.

    A diffuse scarlatiniform erythroderma, bulbar conjunctival hyperemia, and striking palmar edema were impressive findings in two patients who developed toxic shock syndrome (TSS). In addition to the rash which is always seen, the latter two features have been observed in high frequency in this condition and when present are useful aids in the diagnosis of this disease.
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9/10. Eosinophilic fasciitis in south africa. A report of 2 cases.

    Eosinophilic fasciitis was first described by Shulman in 1974 and is characterized by oedema of the extremities, peripheral eosinophilia, hypergammaglobulinaemia and inflammation of the deep fascia. The condition has not been previously reported in south africa. We describe 2 cases seen at the rheumatology Clinic of the Johannesburg Hospital recently. The most striking clinical features were the marked palmar fascial thickening and oedema of the hands and feet. The latter is said to be uncommon in this syndrome and mimicked the early changes of scleroderma. Furthermore, blood eosinophilia may also occur in scleroderma. Our patients illustrated the extremely variable laboratory features in this disorder. In accordance with previous findings in the literature, visceral involvement was absent in our cases. We suggest that in patients without Raynaud's phenomenon who present with scleroderma-like changes of the distal extremities, a diagnosis of eosinophilic fasciitis should be excluded by a full-thickness biopsy.
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10/10. Nevo syndrome.

    We report on a patient with Nevo syndrome manifesting intrauterine and postpartum overgrowth, accelerated osseous maturation, dolichocephaly, highly arched palate, large, low-set ears, cryptorchidism, delayed neuropsychological development, hypotonia, adema, contractures of the hands and feet, a single a transverse palmar crease, and tapering digits. After meningococcal sepsis at age 6 months, he remained decerebrate. Thereafter, overgrowth and especially weight gain were extremely accelerated until his death at age 18 months, at which time his height was 103 cm and his weight was 23 kg. In addition to low plasma concentrations of growth hormone and insulin-like growth factor, severe insulin resistance was observed. It is presumed that a selective defect in insulin-stimulated glucose uptake, with preservation of anabolic effect, was one of the causes of his "overgrowth without growth hormone," at least in the last 12 months of life after severe brain damage.
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