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)

Many immunodeficiency states create an imbalance in the ratio of T cells to B cells or in the subsets of lymphocytes found within these two major classes. A 30-d period of suboptimal intake of zinc often caused a 50% decrease in the total number of splenic lymphocytes in young adult mice. One- and two-color flow cytometric analysis was made of the residual splenocytes of the zinc-deficient mice to determine whether the deficiency had also altered the composition or phenotypic distribution of any of the major subsets of T cells or B cells. Marginally zinc-deficient mice had a normal ratio of T cells to B cells with no notable change in the subsets of lymphocytes making up these two classes. Severely zinc-deficient mice that exhibited a significant degree of parakeratosis demonstrated a 20% increase in the overall ratio of T helper to T suppressor/cytotoxic cells as well as a modest decline in the percentage of B cells (5-8%). The latter decrease was not in the B cells bearing immunoglobulin M or D but in the minor subsets bearing other immunoglobulin isotypes. Thus it seems that, whereas zinc deficiency caused significant reductions in the total numbers of splenic lymphocytes, it caused no significant change in the composition of the splenic lymphocytes except in the most severe cases.
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PMID:Flow cytometric analysis of the phenotypic distribution of splenic lymphocytes in zinc-deficient adult mice. 188 Jun 21

Oral hairy leukoplakia (HL) was studied in 32 patients infected with the human immunodeficiency virus (HIV). Features most often found were parakeratosis of variable thickness, acanthosis, little or absent submucosal chronic inflammatory cell infiltrate, association with candidiasis in 71.8%, and intranuclear herpetic type inclusions in keratinocytes, associated with ballooned or ground-glass cytoplasm. The last feature was found in all biopsies and was most striking at the top of the stratum spinosum, just beneath the parakeratotic surface. We consider that intranuclear inclusions are the histologic hallmark of HL, which, together with other features described, make it possible to diagnose HL specifically by light microscopic examination. Ultrastructural studies were carried out in 20 cases. Viral particles of the human herpesvirus group were detected in 17 cases. Particles of papillomavirus (HPV) were not found in any of the specimens. Immunohistochemical studies were made in 26 cases using a rabbit antiserum to papillomavirus. Nuclear positivity was observed in 25 cases. Despite such apparently contradictory results, we think that a cross reaction may exist between the antiserum used against HPV and some antigens of the Epstein-Barr virus (EBV).
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PMID:Oral hairy leukoplakia: a histopathologic study of 32 cases. 217 38

Oral hairy leukoplakia (HL) is a newly described lesion (1984) in human immunodeficiency virus infected patients. Patients with HL show a high probability of developing an acquired immunodeficiency syndrome (AIDS). The results of histopathological, microbiological, immunological and ultrastructural studies in five patients with HL and AIDS are reported. The histopathological exam revealed in all cases acanthosis, parakeratosis, koilocytosis and keratin projections on the surface. Dermis was normal. Herpes type virus were seen in four cases and in one of them papilloma virus was also present in electron microscopic examination. There was immunocytochemical evidence of papilloma virus in one lesion. Candida albicans was found in 5 lesions by culture but only in two ones by periodic acid Schiff stain. Virus cultures for herpes virus were negative. Immunocytochemical search of Langerhans cells (S-100, HLA-DR, OKT4, OKT6) showed nearly absence of these cells in HL lesions. These results favour the viral etiology hypothesis of hairy leukoplakia.
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PMID:[Oral hairy leukoplakia. A study of 5 cases]. 269 22

In 23 out of 227 patients with positive serological tests for the human immunodeficiency virus (10%), seen between June 1987 and May 1988, lingual lesions of hairy leukoplakia (HL) were found. HL was present in 16/188 drug abusers (9%) and in 7/19 homosexuals (32%). In 3 cases HL was the only clinical manifestation of disease, in 11 it was associated with other symptoms of acquired immunodeficiency syndrome related complex (ARC), and in 9 it was found in patients with a previous or concomitant diagnosis of acquired immunodeficiency syndrome. The mean count of CD4 lymphocytes in the 23 patients was 0.22 X 10(9)/l. The diagnosis was made on the basis of the characteristic clinical features. In 3 cases biopsy was carried out, and parakeratosis and vacuolization of the spinous layer cells were found. Remarkably, particles of the herpesvirus group were also found. The lesions oscillated in size or even spontaneously disappeared, at least transiently; however, in the patients treated with zidovudine the improvement appeared to be more significant. The detection of HL discloses a likely infection by the HIV; it is usually associated with other features of ARC and/or severe immune depression, and it requires the institution of antiretroviral treatment.
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PMID:[Hairy leukoplakia: a new disease of the oral mucosa associated with infection by the human immunodeficiency virus]. 274 11

Proliferative lesions were found on the squamous epithelium of the tongue, esophagus, or penis or haired skin of the lip, hand, or thorax of 8 simian immunodeficiency virus-infected rhesus monkeys that died of simian AIDS. The lesions were focal and consisted of hyperkeratosis, parakeratosis, and acanthosis in the skin, with additional ballooning degeneration in the tongue, esophagus, and penis. The epithelial surfaces were frequently colonized by Candida species or gram-positive cocci. Intranuclear inclusion bodies were seen in cells in the middle and superficial layers. Herpesvirus virions were found in inclusion-bearing cells by transmission electron microscopy. An Epstein-Barr-like virus was identified in inclusion-bearing cells by immunohistochemistry and in situ hybridization. No virus was detectable in basal layers of the epithelium. These lesions resemble oral hairy leukoplakia in AIDS patients and may thus provide a useful primate model to study permissive epithelial infection by Epstein-Barr-like viruses.
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PMID:Squamous epithelial proliferative lesions associated with rhesus Epstein-Barr virus in simian immunodeficiency virus-infected rhesus monkeys. 762 99

The clinical differential diagnosis of erythroderma plus immunodeficiency and failure to thrive in neonates includes graft-versus-host-disease (GVHD), Omenn's syndrome (OS), and Netherton's syndrome (NS). In addition to immunological investigations, skin biopsy is an important part of the diagnostic work-up. We reviewed biopsies from 25 patients that were retrieved from the archives of the Department of Histopathology at Great Ormond Street, of which 9 were OS, 11 were GVHD, and 5 were NS. Five patients had two biopsy specimens. Both OS and GVHD show dyskeratosis and basal vacuolation. OS always shows acanthosis and almost always parakeratosis. GVHD shows a flat epidermis and rarely parakeratosis. OS and GVHD can be distinguished after immunohistochemistry for LCA and CD68 by the relative proportions of lymphocytes and macrophages in the dermal infiltrate (predominantly lymphocytes in OS, relatively more macrophages in GVHD). Skin biopsy diagnosis of OS is difficult before 6 weeks of age because the features are poorly developed. NS can be distinguished by psoriasiform acanthosis, thickening of the basement membrane, prominent dermal blood vessels, absence of dyskeratosis, and basal layer vacuolation, and a dermal infiltrate in which lymphocytes and macrophages are equally represented. Thus, the main difference between GVHD and OS is in the proportion of lymphocytes and macrophages in the infiltrate on immunohistochemical staining for LCA and CD68, while OS and NS may be distinguished on H&E morphology alone.
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PMID:Omenn's syndrome: differential diagnosis in infants with erythroderma and immunodeficiency. 1137 Feb 61

A 45-year-old previously healthy man presented with minimally itchy spiny papular lesions of 3 years' duration and discharging nodular cystic lesions for the past 2 years. Initially, lesions appeared on his ears, followed by the gradual appearance of similar lesions over his face, back, and extremities. The lesions were not associated with photosensitivity. Over the years, the patient continued to have similar lesions without any significant response to various topical medications and oral antibiotics. There was no history of fever or any other systemic complaints and the patient denied any other significant medical problems in the past. Cutaneous examination revealed multiple, grouped, spiny papular lesions coalescing to form plaques at places over the ears, extremities, and trunk (Figure 1). Elongated, horny, follicular spires were noted on the top of the papular lesions. Multiple discharging nodular lesions with crusting were seen predominantly over the chest, abdomen, and back (Figure 2), and closed comedonal lesions were noted on the face and trunk. Multiple orange-brown scaly plaques were seen over the extensor aspect of the patient's thighs (Figure 1, inset). A few nails showed wedge-shaped thickening without subungual hyperkeratosis or other nail changes. Palms, soles, and oral mucosa were normal. Considering the atypical cutaneous findings, the clinical possibility of pityriasis rubra pilaris (human immunodeficiency virus [HIV]-associated type 6 PRP) was considered. Hematoxylin and eosin stain of skin biopsy specimens taken from the spiny papular and plaque lesions revealed marked hyperkeratosis with alternating orthokeratosis and parakeratosis with follicular keratotic plugging (Figure 3; Figure 3, inset). Irregular broad acanthosis was also detected in the epidermis. Dermis showed moderate perivascular lymphomononuclear inflammatory infiltrate. Thus, the skin biopsy was consistent with PRP. HIV serology by enzyme-linked immunosorbent assay (ELISA) with 3 different kits was found to be positive for HIV-1. The patient denied any risk factors associated with acquiring HIV infection, and laboratory values for complete blood cell count, routine serum biochemical profile, and lipid profile were normal. The patient was prescribed oral isotretinoin and asked to come for follow-up for CD4 T-cell count, but did not return.
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PMID:Pityriasis rubra pilaris in a HIV-positive patient (Type 6 PRP). 1817 6

A 32-year-old man was referred for acute onset of pruritic scaly eruptions in the axillae of 8 days' duration, which was unresponsive to topical clotrimazole. The lesions consisted of multiple, coalescent oval plaques of 1 cm to 6 cm in longest diameter (Figure 1 and Figure 2) with central clearing and typical collarette scales at the periphery (Figure 3). Other skin areas and mucosal surfaces were unaffected. His general and systemic examinations were normal. Family and past histories were unremarkable except for a "ring worm-like patch" on his lower aspect of the abdomen 4 months ago, which rapidly regressed. On further inquiry, he gave a history of an episode of fever, coryza, and headache 3 weeks earlier to his eruption on the abdomen, which resolved with conservative remedies and one paracetamol tablet. He remained asymptomatic until axillary lesions appeared. We made a provisional diagnosis of pityriasis rosea (PR). Investigations including scrapings for potassium hydroxide examination, complete blood cell counts, urinalysis, blood sugar, VDRL test, and human immunodeficiency virus antibodies were all normal or non-reactive. As cutaneous biopsy revealed parakeratosis, epidermal spongiosis, dermal inflammatory cells, and extravasated red blood cells (Figure 4). The eruptions cleared within 8 days, following treatment with mometasone furoate cream and oral desloratidine 5 mg/d, leaving post-inflammatory hyperpigmentation. There was no recurrence for the next 6 months of observation. He was later lost to follow-up.
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PMID:Bilateral scaly plaques in axillae: pityriasis rosea of Vidal. 2300 48

Proximal subungual onychomycosis (PSO), which predominantly involves the nail plate from the proximal nail fold, is the rarest form of onychomycosis. Classically associated with an immunocompromised state, PSO is an uncommon diagnosis in individuals without immunodeficiency. We present a case of a healthy 51-year-old man, who presented with a three-month history of white discoloration of multiple toenails. Physical examination revealed white, opaque patches on the proximal third nail plates of multiple toenails. The affected digits also demonstrated proximal onycholysis, subungual debris, and mild paronychia. Laboratory examinations, including routine serologic studies as well as human immunodeficiency virus and antinuclear antibodies, were within normal limits. Proximal nail fragments of the left hallux showed sections of dystrophic nail plate with mounds of parakeratosis, collections of neutrophils, and hyphae that highlighted with periodic acid-Schiff staining. The patient was diagnosed with PSO and tinea pedis bilaterally and treated with oral fluconazole with gradual improvement. This case of PSO highlights the potential for its rare occurrence in a healthy host. However, the clinical presentation of PSO should trigger an evaluation for possible immunodeficiency. <p><em>J Drugs Dermatol. 2018;17(4):475-478.</em></p>.
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PMID:Proximal Subungual Onychomycosis in the Immunocompetent: A Case Report and Review of the Literature. 2960 25