Cases reported "Alopecia"

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1/16. Allogenic hair transplant in a bone marrow transplant recipient.

    BACKGROUND: Autotransplantation of hair normally provides satisfactory correction of baldness. The authors suggest that the allotransplantation of hair can aid patients who have undergone bone marrow transplantation as to correct hematologic disorders. In such patients, autotransplantation of hair may be unsatisfactory because of insufficient donor hair. OBJECTIVE: To determine whether allogeneic hair grafts from bone marrow donors can grow in the bone marrow transplant recipient. methods: The authors performed a standard hair transplant using minigrafts. The patient, who presented a large bald area, had undergone bone marrow transplant due to acute lymphoblastic leukemia. The authors transplanted the patient with hair taken from the same bone marrow donor. RESULTS: The hair had very good growth and the results, according to the authors, are comparable to those of hair autotransplantation. CONCLUSION: Chemotherapy and radiation treatments can often lead to widespread, permanent hair loss. Allotransplantation of hair usually proves unsatisfactory, because skin is strongly antigenic. Bone marrow transplant patients can undergo an allotransplant of hair from the same bone marrow donor if their own donor area is inadequate.
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2/16. Human equivalent of the mouse Nude/SCID phenotype: long-term evaluation of immunologic reconstitution after bone marrow transplantation.

    Human Nude/SCID (severe combined immunodeficiency) is the first severe combined immunodeficiency caused by mutation of the winged-helix-nude (WHN) gene, which is expressed in the thymus but not in the hematopoietic lineage. The disease is characterized by a T-cell defect, congenital alopecia, and nail dystrophy. A Nude/SCID patient who underwent bone marrow transplantation from the human leukocyte antigen-identical heterozygote brother was studied to investigate, in this unique model, the role of the thymus in immunologic reconstitution. Despite an increase in CD3( ), CD4( ), and CD8( ) cells, CD4( ) CD45 RA naive lymphocytes were not regenerated. Conversely, naive CD8( ) cells were normal. After an initial recovery, lymphocyte proliferation to mitogens progressively declined compared with controls and genotypically identical donor cells grown in the WHN( /-) environment. Analysis of the T-cell receptor (TCR) repertoire of CD4( ) cells revealed that only 3 of 18 Vbeta families had an altered CDR3 heterogeneity length profile. Conversely, CD8( ) lymphocytes showed an abnormal distribution in most Vbeta families. These data indicate that the thymus is differentially required in the reconstitution of CD4( ) and CD8( ) naive subsets and in the maintenance of their TCR repertoire complexity. Taken together, these findings suggest that bone marrow transplantation is ineffective in the long-term cure of this form of SCID.
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3/16. zinc therapy of depressed cellular immunity in acrodermatitis enteropathica. Its correction.

    A child with hypogammaglobulinemia and intractable diarrhea underwent parenteral alimentation for five months. A clinical syndrome of acrodermatitis enteropathica subsequently developed associated with a depression in thymus-dependent lymphocyte (T cell) numbers, abnormal T-cell mitogen-induced blast transformation, and anergy to skin test antigens. plasma zinc levels were found to be abnormally low. zinc therapy resulted in dramatic resolution of the clinical manifestations of acrodermatitis enteropathica. Cell-mediated immune function was also restored to normal, suggesting an important role for zinc and possibly other trace metals in cellular immune responses.
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4/16. central nervous system involvement in autoimmune polyglandular syndrome.

    We present a 33 year-old man, admitted because of transient deterioration of visual acuity. magnetic resonance imaging showed diffuse central nervous system (CNS) demyelination, which largely resolved spontaneously within 4 months. The patient fulfilled the diagnostic criteria of APS type III, having autoimmune thyroiditis and alopecia universalis. In this patient, autoimmune activation against CNS antigens is thought to be the cause of reversible CNS demyelination.
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5/16. ANA negative (Ro) lupus erythematosus with multiple major organ involvement: a case report.

    Anti-nuclear antibody (ANA) negative systemic lupus erythematosus (SLE) occurs in about 4-13% of SLE cases. A small group of ANA negative SLE patients with positive anti-Ro antibodies usually present with typical vasculitic skin lesions which can be associated with photosensitivity, renal disease, congenital heart block or neonatal lupus. We present a case of a persistently ANA negative patient who presented with joint pain, rashes, mouth ulcer and alopecia. Clinical diagnosis of systemic lupus erythematosus was made even though ANA was negative. She was started on steroids and went into remission. Later, she developed several episodes of convulsions associated with fever and prominent vasculitic lesions. The patient was also found to have microscopic hematuria, proteinuria, anemia and thrombocytopenia. Renal biopsy showed lupus nephritis class 1B. Due to the prominent skin lesions, we performed anti-extractable nuclear antigens (ENA) antibodies test and anti-Ro turned out to be positive. The final diagnosis was ANA negative SLE (Ro lupus) with cutaneous, renal, musculoskeletal, hematological and cerebral Involvement.
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6/16. Simultaneous primary infection with HIV and CMV leading to severe pancytopenia, hepatitis, nephritis, perimyocarditis, myositis, and alopecia totalis.

    Simultaneous primary infection with HIV and CMV in an 18-year-old woman led to an acute cytotoxic reaction, manifesting as pancytopenia, hepatitis, nephritis, perimyocarditis, and myositis. Within 14 days parameters indicating acute cell damage reverted to normal. Two weeks later transient alopecia totalis developed. Initially, HIV-antigen but no hiv antibodies were present. Within 3 weeks HIV-IgG antibodies appeared while HIV antigen disappeared. Anti-CMV-IgM was clearly and anti-CMV-IgG questionably positive; IgM persisted further, while IgG remained definitely undetectable. We speculate that a particular HIV-induced imbalance of the immune system led to a generalized severe cytotoxic reaction to a simultaneous infection with CMV.
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7/16. Chronic active hepatitis associated with vitiligo, nail dystrophy, alopecia and a new variant of LKM antibodies.

    In this report we describe the case of a 7-year-old boy, suffering from autoimmune-type chronic active hepatitis (AI-CAH) associated with vitiligo, nail dystrophy, alopecia areata and a variant of liver kidney microsomal (LKM) autoantibodies. This patient's antibodies are different from LKM-1 which are directed against cytochrome P450 db1. They react predominantly with perivenous hepatocytes in contrast to LKM-1 antibodies which homogeneously stain the whole liver lobule in immunofluorescence. In Western blot analysis this LKM variant reacts with a liver microsomal protein of approx. 50 kDa, but not with recombinant LKM-1 (cytochrome P450 db1) antigen. Immunosuppressive treatment led to a normalization of liver histology after 1 year and a significant improvement of vitiligo and alopecia areata. In summary, a case of autoimmune-type chronic active hepatitis is presented which is associated with a new variant of LKM antibodies reacting with a 50 kDa microsomal protein different from cytochrome P450 db1. Furthermore, this patient suffers from extrahepatic syndromes (alopecia, nail dystrophy) that have not been described previously in LKM antibody-positive chronic active hepatitis.
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8/16. 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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9/16. Herpes gestationis. A possible association with autoimmune thyrotoxicosis (Graves' disease).

    Herpes gestationis (HG) is almost identical to bullous pemphigoid (BP) in its immunopathology. Although BP has been reported in association with autoimmune diseases, no such association has previously been noted with HG. We describe three patients with HG who also had Graves' disease, and, in addition, one of these patients had alopecia totalis and ulcerative colitis. All three patients possessed the human lymphocyte antigen HLA-B8.
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10/16. Cronkhite-canada syndrome: report of a case with bacteriologic, immunologic, and electron microscopic studies.

    A case of Cronkhite-canada syndrome is presented. The patient developed protein-losing enteropathy, generalized gastrointestional polyposis, skin pigmentation, onychodystrophy, and alopecia. This patient also exhibited certain unusual features such as: fluctuating dermal manifestations, sclerodermalike skin changes, elevated levels of carcinoembryonic antigen, retinal detachment, cataracts, and cerebellar atrophy. Bacteriologic studies of small intestinal fluid, chromosomal analysis, immunologic investigations, and morphologic studies failed to provide any etiologic clues.
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