1/13. Disseminated BCG infection following bone marrow transplantation for X-linked severe combined immunodeficiency.An 8-month-old boy with X-linked severe combined immunodeficiency (XSCID) developed disseminated bacille Calmette-Guerin (BCG) infection following BCG vaccination at birth. He initially presented with an abscess at the site of BCG vaccination and was begun on three-drug antituberculous treatment (rifampicin, isoniazid, and pyrazinimide). Dissemination was subclinical prior to a human leukocyte antigen (HLA)-identical bone marrow transplant (BMT) from his sister, following which he presented with an acute erythroderma. A skin biopsy specimen revealed granulomas with epithelial histiocytes and giant cells in the reticular dermis, and numerous acid-fast bacilli (AFB) were present on Ziehl-Nielsen stain. A diagnosis of disseminated BCG disease was made. Despite the addition of a fourth antituberculous agent, ethambutol, he did not recover and developed numerous skin abscesses over the following weeks. Examination of pus from these lesions demonstrated numerous AFB. clarithromycin was added as a fifth antituberculous agent. Despite five-drug antituberculous therapy and monthly intravenous immunoglobulin infusions, recurrent abscesses containing AFB developed intermittently until 7 months posttransplant. At follow-up 1 year post-BMT he showed good general physical improvement. All abscesses had healed with scarring, and no further skin lesions had occurred.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/13. Paraneoplastic erythroderma in a prostate cancer patient.BACKGROUND: Erythroderma is an inflammation of the skin, which can be triggered by various diseases as psoriasis, allergies, side effects of medication, infections or malignant tumors. Caused by these various etiologic possibilities patients require extensive diagnostic effort. PATIENT: We report a case of a 71-year-old man presenting with an erythroderma of unknown etiology. Therapy with corticosteroids was not successful. A complete remission was reached by therapy with cyclosporine A, 350 mg/day. Finally, an increased prostate specific antigene (PSA) value was found and a prostate cancer was diagnosed in the patient. RESULTS: After definitive radiotherapy of the carcinoma (total dose 74 Gy, 5 x 2 Gy/week), the cyclosporine A was displaced without recurrence of erythroderma. CONCLUSION: In this case, we consider the erythroderma to have been a paraneoplastic effect of the prostate carcinoma. In male patients with erythroderma an early PSA test should be performed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/13. Tannic-acid staining material on high endothelial venules and lymphocytes in skin and peripheral lymph nodes in staphylococcus aureus-associated erythroderma.The recognition and binding of glycoprotein receptors on lymphocytes to specific antigens present on high endothelial venules (HEV) precedes the egress of lymphocytes from the blood stream into the tissues. In this paper, we report the presence of HEVs with tannic-acid staining material (TASM HEVs) in staphylococcus aureus-associated erythroderma, which allow the migration of CD8 lymphocytes from the bloodstream into the epidermis. TASM positivity is also expressed on lymphocytes within the regional lymph nodes, and by intravascular lymphocytes prior to leaving the TASM HEV. It is proposed that TASM positivity may represent a molecule, which may function in binding lymphocytes to HEVs prior to egress from the HEV. (TASM is lost from lymphocytes after leaving the HEVs). The expression of TASM positivity may form an essential part of the CD8 lymphocyte-HEV recognition system, and may be the means whereby CD8 lymphocytes generated in the regional lymph nodes by various mitogens (in this case by staphylococcal mitogens) may 'home' to specific sites within the epidermis. TASM positivity on both the HEVs and lymphocytes may serve as a convenient marker of such a system.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/13. adult T cell leukemia: a case with massive hyperimmunoglobulinemia E.adult T cell leukemia, an endemic disease in the southwestern part of japan, is characterized (1) by a short survival, (2) by leukemic T cells in peripheral blood that have lobulated nuclei and helper/inducer surface phenotypes, and (3) by cutaneous involvement. A 34-year-old man who had a history of atopic dermatitis was seen at our clinic because of generalized erythroderma and lymphadenopathy. His clinical course was rather chronic as compared with that of prototypic adult T cell leukemia; however, typical leukemia cells were observed in specimens taken from his peripheral blood and skin. The diagnosis of adult T cell leukemia was established by the patient's positive serum antibody reaction to adult T cell leukemia-associated antigen and monoclonal integration of virus genome in the patient's leukemia cell dna. Interesting and characteristic of the patient were the very high levels of serum immunoglobulin e. With the use of an in vitro immunoglobulin production system with mitogen, the patient's T lymphocytes enhanced the differentiation of B cells, both from the patient and from a normal adult, into immunoglobulin e-producing cells. Therefore it may be speculated that T cells functioning as immunoglobulin e-specific helpers were transformed to leukemia cells by human T-lymphotropic virus type I. Continuous antigen stimulation of the patient's atopic diathesis also may be a factor.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
5/13. Fatal erythroderma (suspected graft-versus-host disease) after cholecystectomy. Retrospective analysis.Fatal postoperative erythroderma (POE) developed in a 52-year-old woman with gallstones who underwent elective cholecystectomy. During surgery, she was transfused with 3 units of unirradiated packed red cells stored in the liquid state for at least 4 days after collection. The POE is believed to have been the result of transfusion-associated graft-versus-host disease (TA-GVHD). The diagnosis of GVHD was based upon the characteristic clinical picture and retrospective HLA typing. The implicated donor was homozygous for an HLA haplotype that appeared to be shared with the recipient: A24-CBL-Bw52-DR2-DRw52-DQw1, the most common haplotype in the Japanese population. This case raises the possibility that a transfusion of relatively fresh blood from a donor who has no hla antigens incompatible with the recipient may result in GVHD in patients with no apparent immunoincompetence who are undergoing relatively minor surgery with no chemotherapy or radiation therapy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/13. Functional properties in Sezary cells with an unusual phenotype.The immunological and functional characteristics of Sezary cells with an unusual phenotype are reported. The clinical, histologic, and hematologic picture was typical for sezary syndrome. Studies with monoclonal antibodies showed that 80% Sezary cells had an CD3 , CD4 , CD5 , CD7-, CD8-, Leu-7 , Leu-8-, Leu-11-, OKM1- phenotype. By two-color immunofluorescence assay 80% FACS-sorted Leu-7 cells coexpressed CD4 antigen and did not express the myeloid antigen OKM1, CD8, and antigens characteristic of immature T cells. The cells had no NK activity but did display a high helper activity. Unseparated and FACS-sorted Leu-7 and Leu-7- Sezary cells did not respond to mitogens but were able to grow in the presence of exogenous IL-2. FACS sorted Leu-7- cells, cultured for 7 days in the presence of 20% IL-2, acquired the receptors for Leu-7. IL-2 and IFN-gamma production was studied in unseparated Leu-7 and Leu-7- FACS-sorted Sezary cells. IL-2 production was lower than in normal cells. The addition of PHA or PHA plus TPA led to an increase in IL-2 production. Also IFN-gamma production was marked lower than in normal controls but increased after 7-day culture in exogenous IL-2. In conclusion in this case the Sezary cells may represent a neoplastic expansion of the CD3 , CD4 , CD5 , Leu-7 , Leu-11- subpopulation which is equivalent to the 2-4% of the Leu-7 population in normal lymphocytes.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
7/13. Erythroderma associated with mixed lymphocyte-endothelial cell interaction and staphylococcus aureus infection.We report 11 consecutive cases of erythroderma, a high percentage of which were associated with the growth of staphylococcus aureus in cultures from blood, joint fluid or skin. In biopsies from all the patients we found close morphological associations between lymphocytes and endothelial cells, with some of the lymphocytes showing features of blastoid transformation to T helper lymphocytes. In extreme cases, sheets of T cells, including T helper lymphocytes, formed a syncytium with endothelial cells in the dermis. Marked capillary proliferation was noted both on light and electron microscopy. We suggest that erythroderma is precipitated by antigens such as protein A, a potent T cell mitogen present on the cell surface of staphylococcus aureus, or by drugs, such as phenytoin. These antigens induce antigen presentation by individual endothelial cells, leading to T helper transformation and lymphocyte proliferation. Endothelial proliferation resulting from lymphocyte-endothelial interaction results in the vascular proliferation associated with this syndrome.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
8/13. 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.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/13. Normolipemic papular xanthomatosis in erythrodermic atopic dermatitis.We describe papular xanthomatosis that progressively developed in a patient with long-standing erythrodermic atopic dermatitis and normal lipid metabolism and without an associated systemic disease. light microscopy showed a lobulated aggregate of sometimes foamy histiocytes. Ultrastructurally, these histiocytes contained lipid inclusions and lacked features of Langerhans or epithelioid cells. Other granulomatous skin diseases such as tuberculosis, sarcoidosis, or foreign body granuloma were excluded by histologic study, polarizing microscopic examination, electron microscopy, and microbiologic investigations. Nevertheless, these xanthomas showed an antigen expression pattern similar to that found in noninfectious granulomas (CD1a-, MS-1-, CD11c , MRP-8/-14 , 25F9 , RM 3/1 /-, CD36( ), indicating that normolipemic papular xanthomatosis may be reactive process and should not be included among the true cutaneous non-Langerhans cell histiocytoses.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/13. Intractable infant diarrhea associated with phenotypic abnormalities and immunodeficiency.We report on eight children with severe diarrhea beginning in the first 6 months of life (< 1 month in six cases), who had a number of features in common. All were small for gestational age and had an abnormal phenotype, including facial dysmorphism, hypertelorism, and woolly, easily removable hair with trichorhexis nodosa. Two were products of consanguineous marriages. Severe secretory diarrhea persisted despite bowel rest (n = 7). Jejunal biopsy specimens showed total or subtotal villous atrophy with crypt necrosis, and inconstant T-cell activation in some cases (n = 3). colon biopsy specimens showed moderate nonspecific colitis. All the patients had defective antibody responses despite normal serum immunoglobulin levels, and defective antigen-specific skin tests despite positive proliferative responses in vitro. Three had monoclonal hyper-immunoglobulinemia A. The course was marked by diffuse erythroderma in two cases and mental retardation in three. Treatment included bowel rest, intravenous administration of immune globulins, administration of corticosteroids (n = 6) and cyclosporine (n = 2), and bone marrow transplantation (n = 1). Five patients died between the ages of 2 and 5 years (of sepsis or cirrhosis), two are being fed enterally, and one continues to receive total parenteral nutrition. The cause of the combined low birth weight, dysmorphism, severe diarrhea, trichorrhexis, and immunodeficiency is unclear. These features may constitute a specific syndrome within the group of intractable diarrheas of infancy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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