Cases reported "Syndrome"

Filter by keywords:



Filtering documents. Please wait...

11/24. aortic rupture in a patient with elastosis perforans serpiginosa (Lutz-Miescher).

    A case of aortic rupture in a 30-year-old women with a rare skin disease, elastosis perforans serpiginosa, is presented. Skin lesions are characterized by marked elastic tissue changes. Multiple large arteries and the distal part of the aorta were thinwalled also with aneurysm-like dilatations. Focal fibroelastosis and degenerative changes were present in the vessel walls. The changes in the aorta made the surgical treatment difficult.
- - - - - - - - - -
ranking = 1
keywords = skin disease
(Clic here for more details about this article)

12/24. Blue rubber bleb nevus syndrome.

    A case and the family pedigree with the dominant genodermatosis blue rubber bleb nevus syndrome (BRBNS) are described. It is shown that the mode of inheritance is autosomal dominant with good penetrance and that the affected individuals examined all are healthy apart from the skin disease.
- - - - - - - - - -
ranking = 1
keywords = skin disease
(Clic here for more details about this article)

13/24. Concurrent localized scleroderma and discoid lupus erythematosus. Cutaneous 'mixed' or 'overlap' syndrome.

    Four patients with concurrent, chronic, progessive, localized scleroderma and discoid lupus erythematosus were studied; the condition originated as linear scleroderma in three of them. Three of the four patients were young females at the onset of the first skin disease. Dermatopathologic study confirmed the scleroderma and lupus erythematosus (LE). Direct immunofluorescence showed a positive band test in three cases. Unusual serological results included a positive LE clot test in three cases, a positive extractable nuclear antigen test in one case, and a negative antinuclear antibody test on repeated occasions in all four cases. Rare cutaneous disease similar to systemic, "mixed," or "overlap" connective tissue disease exists and offers an opportunity to study unusual immunologic and pathological events in both scleroderma and LE.
- - - - - - - - - -
ranking = 1
keywords = skin disease
(Clic here for more details about this article)

14/24. Sweet's syndrome leading to acquired cutis laxa (Marshall's syndrome) in an infant with alpha 1-antitrypsin deficiency.

    BACKGROUND: Marshall's syndrome is a rare pediatric skin disease that is characterized by acquired, localized neutrophilic dermatitis (Sweet's disease), followed by loss of elastic tissue in the dermis and cutis laxa. The cause of this syndrome is unknown. alpha 1-Antitrypsin (alpha 1-AT) deficiency is a codominantly inherited disorder of alpha 1-AT, the major serum antiprotease active against a number of serine-type proteases. OBSERVATIONS: The first patient with classic Marshall's syndrome who had coexisting alpha 1-AT deficiency and a review of other cases of Marshall's syndrome are presented, and pathogenic mechanisms are discussed. CONCLUSIONS: A deficiency of alpha 1-AT may allow proteases such as neutrophil elastase to destroy dermal elastin and, thus, produce cutis laxa in Marshall's syndrome. Other cases of acquired cutis laxa should be screened for alpha 1-AT deficiency to further evaluate this association and to enable patients and their families to be counseled about possible systemic complications of alpha 1-AT deficiency.
- - - - - - - - - -
ranking = 1
keywords = skin disease
(Clic here for more details about this article)

15/24. Release of acquired syndactylies in Kindler syndrome.

    Kindler syndrome is a rare, blistering skin disease characterized by acral bullae, poikiloderma, and diffuse cutaneous atrophy. Kindler syndrome has been established as a separate entity from epidermolysis bullosa; however, controversy still remains as to whether Kindler syndrome can be differentiated from Weary's hereditary acrokeratotic poikiloderma. Fusion of the digits secondary to blistering and scarring, "pseudosyndactyly," has been reported in several patients with Kindler syndrome; however, surgical correction of the syndactylies in these patients has not been described. In this report, a patient with Kindler syndrome underwent surgical treatment of acquired syndactylies. Treatment included a tailored approach to preparation of the patient for surgery, surgical separation of fused tissues, selection of donor site for skin-graft harvest, postoperative dressings, splinting, and therapy. Results in our patient 2 years after correction demonstrate that syndactyly release in Kindler syndrome can be accomplished effectively, with improvement in both function and appearance.
- - - - - - - - - -
ranking = 1
keywords = skin disease
(Clic here for more details about this article)

16/24. Homozygous variegate porphyria: an evolving clinical syndrome.

    Variegate porphyria is one of the most frequently encountered genetic conditions in south africa. It is inherited as an autosomal dominant disease and in excess of 300 heterozygous cases have been studied by the Cape Town unit. Despite this, the homozygous condition has not previously been encountered in south africa. We report two cases of homozygous variegate porphyria, one of whom represents the first South African case. We delineate a syndrome principally characterized by growth retardation, developmental delay, epileptic seizures, photosensitivity and an abnormal porphyrin excretion pattern. In addition we describe, in one case, two features not previously reported: skin disease in areas unexposed to light and a severe sensory neuropathy which may account at least in part for the hand deformities of this disorder.
- - - - - - - - - -
ranking = 1
keywords = skin disease
(Clic here for more details about this article)

17/24. Sutton's summer prurigo of the elbows.

    Seasonal variations in skin diseases are most noticeable in those parts of the country with significant changes of seasons. For the past 5 years, I have noticed an unusual recurrent prurigo of the elbows which was originally described by Sutton. This disease, which I feel is related to atopic eczema, usually occurs on the elbows of children during the first few weeks of good spring weather. The disease appears as a papular eczematous dermatitis which is usually limited to the elbows but may also affect the knees, hands and chest. It responds well to topical corticosteroids but has a tendency to recur each spring for several years.
- - - - - - - - - -
ranking = 1
keywords = skin disease
(Clic here for more details about this article)

18/24. Fatal keratitis ichthyosis and deafness syndrome (KIDS). Aural, ocular, and cutaneous histopathology.

    We report a case of KID (keratitis, ichthyosis, and deafness) syndrome in which the patient died at the age of 2 months. Detailed histological study of the affected organs, particularly the inner ear and external auditory meatus, was performed. This case is the first in which previously described principles relating to the biology of the ear canal epithelium have been applied to a patient with skin disease affecting this area.
- - - - - - - - - -
ranking = 1
keywords = skin disease
(Clic here for more details about this article)

19/24. association of hyperimmunoglobulinaemia D syndrome with erythema elevatum diutinum.

    Hyperimmunoglobulinaemia D and periodic fever syndrome was observed in a female patient with erythema elevatum diutinum. The association of this skin disease with hyperimmunoglobulinaemia D may indicate a pathogenetic relationship. The skin lesions responded to dapsone therapy.
- - - - - - - - - -
ranking = 1
keywords = skin disease
(Clic here for more details about this article)

20/24. Reticular erythematous mucinosis syndrome associated with psoriasis-like eruptions.

    We report a case of reticular erythematous mucinosis syndrome associated with psoriasis-like eruptions. Although the two skin diseases are clinically distinct, they may be related in some ways.
- - - - - - - - - -
ranking = 1
keywords = skin disease
(Clic here for more details about this article)
<- Previous || Next ->


Leave a message about 'Syndrome'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.