Diagnosis of Sézary syndrome in a patient with generalized pruritus based on early molecular study and flow cytometry.
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Biomedical subjects
Publications and source records attributed to H V Dubin.
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We describe a 48-year-old woman who had worsening psoriasis, photosensitive reticular erythematous mucinosis (REM), and acne rosacea. The psoriasis was unresponsive to etretinate, and the REM was unsuccessfully treated with hydroxychloroquine. Cyclosporine (6 mg/kg/day) successfully cleared the psoriasis in 8 weeks but did not improve the REM and acne rosacea. Possible causes for the lack of response of the REM to cyclosporine are discussed.
Aggressive B-cell non-Hodgkin's lymphoma frequently complicates human immunodeficiency virus type 1 infection. Although extranodal disease is an important feature of this condition, the incidence of cutaneous involvement has not been determined. A review of 754 published cases of B-cell non-Hodgkin's lymphoma associated with human immunodeficiency virus type 1 revealed an incidence of cutaneous extranodal involvement of 8.2%, approximately equal to that seen in non-Hodgkin's lymphoma not associated with human immunodeficiency virus. Lymphoma was the initial manifestation of acquired immunodeficiency syndrome in two thirds of these cases. In particular, although the head and neck are commonly involved in non-Hodgkin's lymphoma not associated with human immunodeficiency virus, scalp involvement is uncommon in human immunodeficiency virus-associated B-cell non-Hodgkin's lymphoma, and we report the second case. The natural history and proposed pathogenesis of human immunodeficiency virus-associated non-Hodgkin's lymphoma are discussed.
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Juvenile dermatitis herpetiformis occurred in a 20-month-old girl. She had granular lgA, C3, and fibrin bound to the basement membrane zone of the skin by direct immunofluorescence and negative serum antibodies against the skin on indirect immunofluorescence. The HLA typing of peripheral lymphocytes was A1, Aw30, B8, Bw51 without clinical evidence of malabsorption syndrome. A rapid improvement was observed on dapsone therapy. These findings strongly suggest that juvenile dermatitis hepetiformis is a disease entity different from chronic bullous dermatosis of childhood.
Xanthomas and lipoprotein abnormalities are important to all physicians. Many factors affect the synthesis and degradation of cholesterol and triglyceride, the lipids of major importance in these disorders. Lipids travel in serum bound to protein moieties, forming lipoproteins. Hyperlipoproteinemias may be either primary or secondary to other diseases. Primary hyperlipoproteinemias recently have been reclassified, based on genetic studies. The four types of xanthomas include tendinous, tuberous, eruptive, and planar. Since xanthomas are usually not diagnostic of a specific disease entity, history, physical, and laboratory data are required for appropriate classification and treatment.
Pyoderma gangrenosum has been associated with myelogenous leukemia and plasma cell dyscrasia. When associated with leukemia, pyoderma gangrenosum often has a distinctive clinical presentation with an advancing bullous margin. The pathogenesis of this disorder is unknown, although defective immune mechanisms may be operative. The occurrence of pyoderma gangrenosum and agnogenic myeloid metaplasia in the same patient has now been reported sufficiently to make it a recognized association.
The association between ulcerative colitis and various abnormalities of the skin, especially pyoderma gangrenosum, has been accepted for many years. The underlying aberrations in either of the organ systems, as well as the basis of their association, however, remain obscure. Several large series of ulcerative colitis patients are reviewed to emphasize the important concomitant cutaneous findings, and investigations into the pathogenesis of pyoderma gangrenosum are discussed.
Oriental sore (acute cutaneous leishmaniasis) is usually a self-limited infection of the skin caused by the protozoan Leishmania tropica. This disease is endemic to the Mediterranean, Asia, Africa, and the Middle East, and is seen in this country among travellers and immigrants. We found four cases at the University of Michigan and diagnosed and treated the disorder. Some of the treatments currently recommended for this disease are potentially toxic, and none have been proved to be of real value. Uncomplicated, acute cutaneous leishmaniasis (L tropica) may best be managed by withholding treatment and observing the patient.
Granuloma faciale is a well-defined entity that almost exclusively involves the face, although it has been reported in extrafacial locations. The infrequent reports of extrafacial lesions may reflect the inconspicuous nature of extrafacial lesions or a failure to specifically examine the patient for such lesions.
A patient who had multiple hemangiomas and for whom results of coagulation studies were consistent with hemangioma-thrombocytopenia (Kasabach-Merritt) syndrome and a microangiopathic hemolytic process was asymptomatic prior to surgical removal of a lipoma. Postoperatively, she developed bleeding secondary to a disseminated intravascular coagulopathy. Her bleeding was controlled by anticoagulation therapy. A review of the literature provides evidence suggesting that the hemangioma-thrombocytopenia syndrome is due to a disseminated intravascular coagulopathy initiated within the hemangioma. Surgical trauma may precipitate exacerbations of the coagulopathy in these patients and all unnecessary surgical procedures should be avoided.
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