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)

A 69-year-old woman sought treatment with a history of persistent debilitating intraoral ulcerations and recurrent oral and vaginal candidiasis. The medical history included thymoma, breast cancer, and lip cancer. The oral lesions were consistent with bullous lichen planus. Laboratory studies showed severe hypogammaglobulinemia. The chronic mucocutaneous candidiasis-thymoma syndrome is now recognized as a distinct form of primary immunodeficiency.
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PMID:Chronic mucocutaneous candidiasis-thymoma syndrome. A case report. 279 38

Serum IgG, IgM, IgA, C3, C4 concentrations and circulating immune complexes (CIC) were measured in a series of 46 patients with oral lichen planus (LP). This investigation revealed a significant increase in the level of serum IgG in patients with oral LP as compared with 36 healthy subjects. In erosive LP, serum IgM was also elevated as compared with healthy subjects and patients with the non-erosive type. The results of this study did not support the suggestion that a humoral immunodeficiency underlies oral LP. Elevated serum level of IgM may be considered to represent secondary oral infection during mucosal erosion. The study also demonstrated a significant reduction in serum C4 in both variants of oral LP, but the C3 level was normal. A 3% polyethylene glycol precipitation (PEG-ppt) method was used, no significant amount of CIC could be detected in the patients. It is tempting to speculate that oral LP reflects an immunologic disorder in which serum IgG and C4 is disturbed. However, further investigation is needed to clarify the pathogenesis of oral LP.
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PMID:Serum immunoglobulins, complements and circulating immune complexes in oral lichen planus. 381 55

The exact pathogenic mechanism involved in lichen planus (LP) remains obscure. Two patients who have severe immunodeficiency diseases and who developed LP during the course of their illness are reported here. Both patients had hypogammaglobulinemia and disturbed immune function prior to the development of LP. Although such an association could be coincidental, the development of LP may be related to the underlying immune disturbances. Association of LP with several other disorders of the immune system has been previously observed. Other evidence for the possible involvement of an immunopathogenic mechanism in LP includes (1) deposition of immunoglobulin within the colloid bodies and at the dermoepidermal junction, (2) predominantly T cell dermal infiltrate in LP lesions, and (3) existence of clinical and histologic similarities between graft-versus-host disease and LP.
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PMID:Lichen planus in two immunodeficient hosts. 698 Feb 33

The cutaneous manifestations of human immunodeficiency virus infection include papulosquamous diseases, viral and fungal infections, and neoplastic disorders. Eczematous photosensitivity disorders have been reported in patients infected with the human immunodeficiency virus. We describe a patient with advanced acquired immunodeficiency syndrome who developed photodistributed hypertrophic lichen planus. We believe this is a distinct cutaneous manifestation of human immunodeficiency virus infection.
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PMID:Photodistributed hypertrophic lichen planus in association with acquired immunodeficiency syndrome: a distinct entity. 772 55

It is well known that several dermatoses, such as psoriasis vulgaris and seborrheic dermatitis, present with more extensive and severe disease in patients infected with the human immunodeficiency virus (HIV-1). Except for one report, however, lichen planus (LP) has not been described in patients with HIV infection. In this report we describe the clinical and morphological features of 3 HIV-positive patients who presented with extensive hypertrophic LP. To determine if alteration in the immune status in HIV-positive hosts is reflected in the nature of the infiltrate in LP, we determined the proportion of T-helper and T-suppressor cells in the infiltrate in 1 case. The majority of the infiltrating lymphocytes in the dermis were of the T-helper phenotype. Epidermal lymphocytes, however, were predominantly of the T-suppressor phenotype. We conclude that LP in HIV-positive hosts may present with more extensive disease than in immunocompetent hosts. Based on our immunohistochemical studies, we conclude that, similar to immunocompetent hosts, T-helper cells are the predominant cells in the dermal infiltrate of LP in HIV-positive patients. However, in contrast with reports in the literature on LP in immunocompetent hosts, we found that, in the case studied, the epidermal lymphocytes were predominantly of the T-suppressor phenotype.
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PMID:Hypertrophic lichen planus in three HIV-positive patients: a histologic and immunological study. 818 34

This report describes a case of secondary syphilis in a patient with the human immunodeficiency virus (HIV) in whom the main clinical presentation of the disease was an extensive oral lesion. Few reports in the literature have focused on oral manifestations of syphilis in HIV-infected patients; most of these reports have dealt with a rare manifestation of secondary syphilis, the ulceronodular variety or lues maligna. However, in the case reported here the lesions were painless, smooth, white, oral plaques and papules on an erythematous base with a serpentine and reddish outline corresponding to the description of mucous patches. The differential diagnosis of this lesion may include oral squamous carcinoma, leukoplakia, erythroleukoplakia, candidosis, lichen planus, and granulomatous diseases. This case report emphasizes the importance of considering secondary syphilis in the differential diagnosis of oral lesions in HIV-infected patients, reinforces the importance of follow-up on the patient, and highlights the consideration for routine serologic testing for syphilis in all HIV-infected patients.
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PMID:Oral secondary syphilis in a patient with human immunodeficiency virus infection. 878 96

A study was made of the oral manifestations in 396 patients with human immunodeficiency virus (HIV) infection. The following risk groups were established: intravenous drug users (79.5%), homosexuals (7.8%), homosexual intravenous drug users (3.3%), heterosexuals (8.1%) and hemophiliacs/transfusion patients (1.3%). The oral lesions, in decreasing order of frequency, were: periodontal disease (78.28%), candidiasis (65.65%), hairy leukoplakia (16.16%), herpes simplex virus lesions (5.30%). Kaposi's sarcoma (2.27%), recurrent aphthous ulceration (RAS) (1.01%), lichen planus (0.5%), non-Hodgkin's lymphoma (0.25%), tuberculous lesion of the tongue (0.25%) and ulcerations of uncertain etiology (0.25%). Attention is drawn to the greater predominance of these lesions in patients with CD4 values of less than 200/ mm3, compared with those who have higher lymphocyte counts.
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PMID:Oral manifestations associated with human immunodeficiency virus infection in a Spanish population. 898 62

The treatment of onychomycosis has improved in recent years and many patients can now expect a complete and lasting cure. However, for up to 25% of patients, persistent disease remains a problem, thus presenting a particular challenge to the clinician. For these patients, it is obviously important to ensure that a correct diagnosis of onychomycosis has been made, as misdiagnosis will inevitably jeopardize the perception of therapeutic effectiveness. Although onychomycosis accounts for about 50% of all nail diseases seen by physicians, nonfungal causes of similar symptoms include repeated trauma, psoriasis, lichen planus, local tumours vascular disorders and inflammatory diseases. Predisposing factors that contribute to a poor response to topical and/or oral therapy include the presence of a very thick nail, extensive involvement of the entire nail unit, lateral nail disease and yellow spikes. However, poor penetration of systemic agents to the centre of infection, or the inability of topical agents to diffuse between the surface of the nail plate and the active disease below, probably contributes to this. Other factors contributing to recurrence may be related to the patient's family history, occupation, lifestyle or underlying physiology. In addition, patients with concomitant disease (e.g. peripheral vascular disease, diabetes) or patients who are immunosuppressed (e.g. those with human immunodeficiency virus/acquired immunodeficiency syndrome) are more susceptible to onychomycosis. In the elderly, the prevalence of onychomycosis may be as high as 60%, and increases with age; in this population, physical trauma plays a major role in precipitating recurrence, especially in patients with faulty biomechanics due to underlying arthritis and bone abnormalities. It is also possible that recurrence in some cases is due to early termination of treatment or use of an inappropriate dose, and these possibilities should be eliminated before further investigations are undertaken. There is good evidence to suggest that a combination of oral and topical therapies, when given at the same time, yield excellent clinical outcomes, although there remains a need for more effective topical agents with greater nail penetration and more effective oral antifungal agents.
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PMID:Onychomycosis in clinical practice: factors contributing to recurrence. 1451 Sep 69

A 28-year-old man with a diagnosis of vitiligo universalis for 6 years presented with reddish polygonal papules that had developed on his left hand first and then on his right hand. On dermatologic examination, porcelain-white hypopigmented appearance was observed all over his body, and there were violaceous, flat-topped papules changing from 2 to 5 mm in diameter localized on the hypopigmented areas of the dorsum of both hands and flexor sites (Figure 1). The physical examination and laboratory investigations including hemogram, erythrocyte sedimentation rate, serum biochemistry, and urinanalysis were normal. Antibodies to thyroid tissue, hepatitis viruses, human immunodeficiency virus, nuclear, and streptolysine were negative. Repeated IgA levels in serum were found to be decreased; however, the other immunoglobulins (IgG, IgM) and C3, C4 and cryoglobulins were in normal ranges. Histopathologic examination of the polygonal papules revealed hyperkeratosis, focal thickening of the granular layer, and irregular acanthosis in triangular saw-tooth pattern. The basal layer was invaded by the lymphocytic inflammatory infiltrate and had numerous necrotic keratinocytes. The infiltrate in the upper dermis was band-like and sharply demarcated at its lower border. There were also a few melanophages in the upper dermis. Melanocytes were decreased in number, and in some areas they were absent at the basal layer of epidermis. Clinical and histopathologic diagnosis were consistent with lichen planus and vitiligo (Figure 2).
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PMID:Colocalization of lichen planus and vitiligo associated with selective IgA deficiency. 1761 76

Vulvovaginal gingival lichen planus (VVG LP) is a distinct variant of LP frequently associated with mucocutaneous scarring and vaginal stricture formation. Good's Syndrome (thymoma with hypogammaglobulinemia) is a rare cause of immunodeficiency in adults. The clinical features, investigation findings, and challenges in the management of a patient presenting with VVG LP and Good's Syndrome are discussed.
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PMID:A case of vulvovaginal gingival lichen planus in association with Good's syndrome. 1828 Sep 48


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