Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Children who are infected with human immunodeficiency virus may develop a wide variety of mucocutaneous manifestations, such as skin infections, tumors, and inflammatory skin disorders. The most significant infectious diseases are candidiasis, dermatophytosis, herpes simplex, herpes zoster, and pyoderma. Inflammatory disorders include seborrheic dermatitis, vasculitis, and pyoderma gangrenosum. Kaposi sarcoma is extremely rare in children with the acquired immunodeficiency syndrome.
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PMID:Cutaneous manifestations of pediatric HIV infection. 149 46

Bloom's syndrome (BS) is an autosomal recessive disease characterized by short stature, sensitivity to sunlight, and telangiectasic malar erythema. It is associated to chromosomal breakage, to primary combined immunodeficiency, and to a high incidence of neoplasias. The authors report the case of two siblings with BS and associated immunodeficiency. Both patients were male and 5 (A) and 4 (B) years old at the time of diagnosis. Chronic diarrhea, recurrent otitis media, purulent rhinitis, conjunctivitis and pyodermatitis were reported by patient A. Patient B was admitted with diagnosis of bilateral neuroblastoma and had the tumor resected. Later on, he presented with oral moniliasis, herpetic stomatitis, and skin abscesses. This patient did not have recurrent infections. Immunological evaluation showed normal serum levels of CH50, C3, and C4 for both patients. Serum IgG, IgA, IgM, and salivary IgA levels were: 455 mg/dl, 15mg/dl, 20mg/dl, 0.6mg/dl for A, and 400mg/dl, 15mg/dl, 20mg/dl, and 0.2mg/dl for B, respectively. Serum antipolio antibodies (1, 2, and 3) were normal, and low levels of isohemagglutinins were observed in both patients. T cells subset determination showed: patient A--OKT3 = 66%, OKT4 = 33%, OKT8 = 32%, and 4/8 ratio = 1.0; patient B--OKT3 = 70%, OKT4 = 32%, OKT8 = 34%, and 4/8 ratio = 1.0. In vitro cellular immune response to PHA was depressed only in patient B. Patients karyotype showed chromosomal breaks with sister chromatid exchanges. Neither patient had abnormal alphafetoprotein and carcinoembryonic antigen serum levels. The rarity of such associations justifies the presentation of the cases.
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PMID:[Familial Bloom's syndrome associated with neuroblastoma]. 221 4

This paper presents longitudinal clinical and immunological findings in a patient with a life-long history of pluriorificial pyoderma vegetans, a disease characterized by a distinct immunodeficiency of T lymphocytes. After treatment with thymopentin, 50 mg s.c. three times weekly for 12 weeks, the number of T lymphocytes in peripheral blood normalized, while other impaired immunological functions failed to improve, however. The most impressive observation was the significant clinical improvement of the patient's condition.
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PMID:Thymopentin treatment in a patient with pluriorificial pyoderma vegetans. 389 84

A new familial immunodeficiency disease characterised by recurrent and persistent pyoderma, folliculitis, and atopic dermatitis is described in a father and son. It is accompanied by abnormalities of lymphocyte function (including defective proliferative responses to phytomitogens, and subnormal response in immunoglobulin production after stimulation of the lymphocytes by pokeweed mitogen) and defective leucocyte chemiluminescence responses, which were associated with defective ability for intracellular killing of microbial organisms. The abnormalities of lymphocyte and leucocyte function, as well as the clinical manifestations, responded dramatically to treatment with the histamine-1 antagonist, chlorpheniramine, suggesting that the underlying defect in this disease may relate to defective histamine metabolism or abnormal expression of histamine receptors on lymphocytes and leucocytes.
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PMID:Pyoderma eczema and folliculitis with defective leucocyte and lymphocyte function: a new familial immunodeficiency disease responsive to a histamine-1 antagonist. 613 27

This is a report of a 24-year follow-up of a man now 33 years of age, who suffers almost continuously from severe inflammatory lesions of the lips, nose and eyelids, with increased susceptibility to respiratory infections since early childhood. The condition, previously described as "pyo-rhino-blepharo-stomatitis vegetans (McCarthy)", was treated with systemic corticosteroids and antimicrobial agents for years, but failed to improve until the immune status of the patient was checked after withdrawal of the steroids. T lymphocytes were found to be abnormal as to count in peripheral blood and various functional qualities determined in vivo and in vitro. For treatment, levamisole and thymopoietin pentapeptide (TP-5) were given. Subsequently each drug induced rapid and complete clearing of all lesions, but was followed by the recurrence of facial periorificial lesions after drug withdrawal. Change of the regimen by administering either inosiplex orally or commercial calf thymus extract parenterally, remained ineffective. During therapy with levamisole as well as TP-5, the number of T lymphocytes in peripheral blood normalized, yet impaired functions failed to improve. There was an elevated ratio of T-suppressor/T-inducer cells in blood using OKT antibodies. In vitro testing of different functions of polymorphonuclear leucocytes revealed normal results except for a slight decrease of chemotactic activity during levamisole. In view of the long clinical course, the mass of clinical and immunological data collected over decades, and the therapeutic results as a whole, the disease can be characterized as a peculiar type of pluriorificial pyoderma vegetans, caused by a distinct immunodeficiency of T lymphocytes.
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PMID:[Pyoderma vegetans of facial orifices in T-cell immunodeficiency]. 660 52

Neonatal, young adult, and aged specific pathogen-free cats were experimentally infected with cat-passaged Petaluma strain of feline immunodeficiency virus. The primary stage of illness occurred 6-8 weeks following infection in cats of all ages, but it differed in severity and clinical signs. Generalized lymphadenopathy persisted for the entire 42-week study period in neonatally infected cats, was transient in young adults, but inapparent in aged cats. Only two aged cats became chronically and severely ill during the study. One aged cat died with severe necrotizing transmural enteritis, while a second developed chronic generalized staphylococcal pyoderma that was partially controlled with antibiotics. Neutropenia appeared 6-8 weeks following infection in cats of all ages, but was more severe in newborn and aged cats than in young adults. A persistent decrease in CD4+/CD8+ T lymphocyte ratios, due to both increased CD8+ and decreased CD4+ T lymphocytes, occurred in the neonatal and aged cats. Decreased CD4+/CD8+ T lymphocyte ratios in the young adult cats was due solely to decreased CD4+ T lymphocytes. Antibody response to FIV virus, as measured by ELISA to recombinant FIV p24 antigen, was lower in aged cats than the other age groups during the first 6 weeks after infection. Antibody levels were not significantly different among the three age groups thereafter. Although there are some differences between FIV infection of cats and HIV infection of human beings, age at infection influences the severity of disease in both species.
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PMID:The effect of age on the course of experimental feline immunodeficiency virus infection in cats. 825 37

We studied the epidemiology, morbidity, and etiology of dermatologic and non-human immunodeficiency virus venereologic disease (Derm/STD) aboard a deployed aircraft carrier to revise Derm/STD training objectives for shipboard primary care providers. Onboard supplies for treatment of Derm/STD were also evaluated. Over 3 months, 929 Derm/STD patients were treated for 1,320 diagnoses generating 2,011 visits. Derm/STD caused 22% of the total morbidity. Pyoderma alone accounted for nearly one-half of that morbidity and involved many work-center groups. Air wing, aircraft maintenance, and engineering work-center groups had lower burdens of pyoderma. Bacterial cultures were performed on 248 exudative dermatoses. Staphylococcus aureus was the dominant pathogen and was overwhelmingly sensitive in vitro to common, inexpensive antibiotics. Strategies to encourage prevention, earlier diagnosis, and rapid treatment of Derm/STD by deployed primary care providers are discussed.
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PMID:The epidemiology of dermatologic and venereologic disease in a deployed operational setting. 875 10

Papular urticaria is the result of hypersensitivity (id-reaction) to bites from certain insects such as mosquitoes gnats, fleas, mites, and bedbugs. Papular urticaria is common in childhood and is characterized by symmetrically distributed pruritic papules and papulovesicles. Scratching causes erosions and ulcerations. Pyoderma is common. Lesions occur in crops. The histopathologic features of papular urticaria are inadequately documented. In a prospective study we recorded the histopathologic features of 30 patients (female, 18; male, 12) with papular urticaria. Their ages ranged from 6-343 months (median = 21 months, mean = 37.73 months). Features that presented in more than 50% of cases included mild acanthosis, mild spongiosis, exocytosis of lymphocytes, mild subepidermal edema, extravasation of erythrocytes, a superficial and deep mixed inflammatory cell infiltrate of moderate density, and interstitial eosinophils. We recognized lymphocytic (n = 4), eosinophilic (n = 9), neutrophilic (n = 7), and mixed (n = 9) subtypes. Immunohistochemistry was performed on formalin-fixed, paraffin-embedded sections from 10 cases and revealed abundant T-lymphocytes (CD45RO, CD3) and macrophages (CD68) in all cases. B-lymphocytes (CD20) and dendritic antigen-presenting cells (S100) were absent. Direct immunofluorescence staining was conducted on cryostat-prepared sections from 26 specimens. Deposition of IgA, IgG, IgM, C3, and fibrin could not be demonstrated. The histopathologic differential diagnosis of papular urticaria includes other spongiotic dermatitides, pityriasis lichenoides et varioliformis acuta, the pruritic papular eruption of human immunodeficiency virus disease, and papulonecrotic tuberculid. Papular urticaria with marked spongiosis and a dense inflammatory cell infiltrate cannot be reliably distinguished from arthropod bites on clinical and histopathologic grounds. The present study provides morphologic and immunohistochemical evidence that a type I hypersensitivity reaction plays a central role in the pathogenesis of papular urticaria. The putative antigen remains undetermined.
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PMID:Papular urticaria: a histopathologic study of 30 patients. 912 95

Twelve German shepherd dogs, each diagnosed as having a recurrent or refractory deep pyoderma (i.e., German shepherd dog pyoderma [GSP]), were evaluated for several parameters over a six-year period. Results indicated that GSP could be associated with flea allergy dermatitis, atopic dermatitis, food allergy, cell-mediated immunodeficiency, or hypothyroidism, or could be an idiopathic disease. The combination of diseases present for a given dog varied from case to case. Adequate control of the pyoderma was achieved only after each identified underlying disease was treated specifically, along with aggressive concurrent medical therapy using systemic antibiotics and medicated baths.
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PMID:German shepherd dog pyoderma: a prospective study of 12 dogs. 920 74

Chronic, recurrent disease involving the alimentary tract, joints, skin and peripheral lymph nodes, central nervous system and conjunctivae was recorded over a 229 day period in a young Weimaraner dog, first presented at 15 weeks of age. The dog had a left shift neutrophilia during periods of active disease and persistently subnormal levels of serum immunoglobulin G (IgG; 2.8 to 9.2 mg/ml) and IgA (< 0.1 to 0.26 mg/ml) in the absence of circulating immune complexes. One littermate died suddenly at 27 weeks of age and a second littermate had an episode of pyoderma. Both of these dogs had low serum IgG (3.5 to 7.2 mg/ml) and the second littermate also had reduced serum IgA (< 0.1 to 0.15 mg/ml). The dam of the litter and three other related dogs had reduced serum IgA (0.22 to 0.31 mg/ml); circulating immune complexes were not recorded in any of the related dogs. This case is the first of putative immunodeficiency of Weimaraners in the UK. Inability to synthesise adequate concentrations of serum immunoglobulins should be considered a primary defect in this disorder.
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PMID:Low serum immunoglobulin concentrations in related Weimaraner dogs. 923 35


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