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[Successfully treated case with microscopic polyangiitis complicated severe varicella zoster virus infection including encephalitis and disseminated varicella zoster].

We report a case with microscopic polyangiitis (MPA) complicated by varicella zoster encephalitis. A 60-year-old woman caught a common cold and had acute otitis media in April 1998. Proteinuria and hematuria with hyaline cast were noted at the routine medical check in May, and she was referred to our hospital because of high fever and chest pain. MPA was diagnosed with acute progressive renal failure, pleuritis and elevated anti-neutrophil cytoplasmic myeloperoxidase antibody (MPO-ANCA). Corticosteroid therapy was administered under hemodialysis but MPA was flared several times with various symptoms including interstitial pneumonitis, alveolar hemorrhage and erythema multiforme exudativum. During the course of the disease she developed disseminated varicella zoster and encephalitis. Positive polymerase chain reaction to varicella zoster in cerebrospinal fluid helped to differentiate her encephalitis from central nervous system symptoms due to microscopic angiitis and herpes simplex encephalitis. Combination of corticosteroid and acyclovir therapies for MPA and varicella zoster encephalitis under hemodialysis were successful. The diagnostic process and therapies to these complicated contexts were thought to be very important.

Acute Kidney Injury↗

Clinical manifestations of parainfluenza infection in children.

Parainfluenza viruses are major pathogens causing respiratory illness, manifesting from mild upper respiratory tract infection to bronchiolitis and pneumonia. This retrospective study aimed at providing clinical and epidemiologic data addressing the parainfluenza virus infection in Taiwan. A total of 39 patients were enrolled in this study from March 1999 to December 2000. Infants and young children were the major susceptible population, with 87.2% of them younger than 3 years. No seasonal trend was noted for parainfluenza type 1 and type 2 infections. One clustering of parainfluenza virus type 3 infections occurred in late spring of 2000 based on collected results. Parainfluenza type 1 viral isolates accounted for all of the cases of croup. Most isolates of parainfluenza virus type 3 were associated with upper and/or lower respiratory tract infections. A substantial proportion of the patients had skin involvement; the identification of one case of possible parainfluenza virus-related erythema multiforme is particularly interesting, especially because the chances of a causal relation between viral infection and skin symptoms are formerly thought to be slight. The identification of parainfluenza virus in illnesses classically considered to be due to other viruses is intriguing and may have important implications in the management of childhood illness clinically.

Adolescent↗

Cutaneous manifestations of systemic lupus erythematosus in Pakistani patients.

OBJECTIVE: Systemic Lupus Erythematosus (SLE) is an autoimmune process in which cutaneous lesions occur in majority of patients. This study from Karachi, Pakistan was conducted to determine the pattern and prevalence of such lesions in SLE in Pakistani patients. METHODS: One hundred ninety eight patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatology Association were examined between 1986 and 2001 for the presence of cutaneous manifestations. RESULTS: Skin changes noted were: noncicatricial diffuse alopecia (22%), malar rash (31%), mucosal lesions (20%), discoid eruptions (15%), photosensitivity (33%), vascular lesions (20%), pruritus (17%), and pigmentary changes (22%). Peripheral gangrene, chronic ulcers, Raynauds phenomenon, urticaria, chilblains, thrombophlebitis, palmar erythema, and erythema multiform were rare. Anti ANA and anti dsDNA were positive in 93% and 83% patients respectively. CONCLUSION: A different clinical pattern was noted in our patients than reported previously.

Adult↗

Antihistaminic drugs in dermatology.

From the results of treatment of 1,770 patients with dermatologic diseases (1,458 as reported in the literature and 312 observed by the author) it is concluded that antihistaminic preparations are of great value in allergic diseases where it is hypothecated that liberated histamine is the offender, as in acute edematous types of urticaria, erythema multiforme, and some cases of allergic pruritus. The indiscriminate use of these drugs is to be avoided. Antihistaminic drugs are palliative-they do not cure. They often prolong the disease. They give temporary relief from swelling and pruritus. They develop no specific immunity and do not replace immunizing efforts. They do not replace or interfere with epinephrine or ephedrine.

Anti-Allergic Agents↗

Histologic features of paraneoplastic pemphigus.

BACKGROUND: We describe the histopathologic features of paraneoplastic pemphigus, a recently described autoimmune mucocutaneous disease associated with neoplasia. Complete evaluation for paraneoplastic pemphigus requires identification of the characteristic mucocutaneous eruption, tissue specimens for routine histologic and direct immunofluorescence evaluation, and identification of circulating autoantibodies with a unique specificity. Immunoprecipitation from keratinocytes reveals a characteristic complex of four proteins with the circulating antibodies. Various neoplasms have been identified in patients with paraneoplastic pemphigus. OBSERVATIONS: We reviewed 16 skin and oral mucous membrane biopsy specimens from six patients with paraneoplastic pemphigus confirmed by fulfillment of all criteria. Major features include epidermal acantholysis, suprabasal cleft formation, dyskeratotic keratinocytes, vacuolar change of the basilar epidermis, and epidermal exocytosis of inflammatory cells. Seven (44%) of 16 specimens displayed a unique combination of suprabasal acantholysis and dyskeratotic keratinocytes throughout the epidermis. These histologic findings correspond to those of the characteristic clinical lesions that are described as having features of pemphigus and erythema multiforme. CONCLUSIONS: Paraneoplastic pemphigus represents a unique clinical, histologic, and immunologic disease characterized by autoantibody production to desmoplakin I and desmoplakin II, bullous pemphigoid antigen, and, possibly, other antigens in the desmosomal complex. Recognition of the histologic features should prompt immunopathologic confirmation and evaluation for an occult neoplasm.

Aged↗

[A clinical analysis of 50 cases of medicament-like dermatitis due to trichloroethylene].

OBJECTIVE: To investigate the clinical manifestations, complications and treatment of medicament-like dermatitis due to trichloroethylene (TCE), so as to provide basis for studying its etiology and mechanism. METHODS: Fifty patients with dermatitis due to TCE from 1997 to 2000 were analysed retrospectively. RESULTS: The occurrence of the dermatitis was not parallel to TCE exposure levels, without significant dose-effect relationship. This disease could be caused by both inhalation and skin exposure. The latency period of TCE dermatitis ranged from 5 to 66 days, and the average was 31.5 d (Medium). The major clinical manifestations included skin lesions, fever, superficial lymph node swelling and liver dysfunction. Infection was the major complication. Glucocorticoid was effective for treatment of this disease. CONCLUSION: The clinical manifestations due to TCE exposure were similar to dermatitis medicamentosa. The major clinical types of TCE dermatitis included exfoliative dermatitis and erythema multiforme. The dermatitis is considered to be mediated by delayed-type (IV) hypersensitivity. The key factors to treat this disease successfully included the use of glucocorticoid in time with sufficient dose and full course, professional skin care, active treatment to protect the liver and to avoid infection.

Adolescent↗

[Skin diseases with photosensitivity].

Skin diseases associated with photosensitivity are numerous and may be divided into three main groups: photo-aggravated dermatoses, genophotodermatoses and metabolic photodermatoses. Photo-aggravated dermatoses are autonomous skin diseases in which exposure to sunlight may make the disease worse or precipitate its onset and/or its progressiveness; this group includes lupus erythematosus, autoimmune bullous diseases, acantolytic dyskeratoses, acne vulgaris, rosacea and cutaneous lymphoid infiltrates. To these must be added photosensitive forms of autonomous dermatoses such as atopic dermatitis, psoriasis, herpes labialis, erythema multiforme, granuloma and disseminated superficial actinic porokeratosis. Genophotodermatoses are genodermatoses which are made photosensitive by a recognized or as yet unidentified deficiency of the natural photoprotection system. In this group are albinism, vitiligo, xeroderma pigmentosum and poikiloderma. Metabolic photodermatoses are diseases in which photosensitization reactions, often revealing, are due to the accumulation in the skin of an endogenous chromophore as a result of a congenital (porphyria) or acquired (pellagra) enzymatic disorder.

Humans↗

Paraneoplastic pemphigus with autoantibody deposition in bronchial epithelium after autologous bone marrow transplantation.

Paraneoplastic pemphigus is a newly described syndrome in which patients have a severe mucocutaneous eruption with clinical features reminiscent of both erythema multiforme major (Stevens-Johnson syndrome) and pemphigus vulgaris, in association with non-Hodgkin's lymphomas and other malignant neoplasms. These patients have autoantibodies that bind to a characteristic set of epidermal proteins: desmoplakin I and desmoplakin II (molecular weight equals 250 kd and 210 kd, respectively), both major cytoskeletal structural proteins associated with desmosome cellular junctions within all epithelia, the bullous pemphigoid antigen, a 230 kd protein associated with hemidesmosomes, and a 190,000-d protein that has not been characterized. In this report, we describe a patient with paraneoplastic pemphigus who had (1) non-Hodgkin's lymphoma in apparent complete remission following autologous bone marrow transplantation, (2) very tense blisters reminiscent of bullous pemphigoid, (3) a unique pattern of immune deposits within the skin, and (4) IgG deposits within the epithelium of the pulmonary bronchi associated with respiratory compromise.

Autoantibodies↗

COX-2 selective nonsteroidal anti-inflammatory drugs: current status.

Since, their introduction, COX (cyclooxygenase enzyme)-2 specific inhibitors have become a rapidly growing segment of the prescription drug market. Researchers have recently focused on the potentially lethal side effects associated with their. FDA has banned the use of nimesulide (hepatotoxic) in pediatric patients and rofecoxib (cardiovascular complications) in both adults and children. COX-2 inhibitors may decrease vascular prostacyclin production and may tip the balance in favour of prothrombotic eicosanoids (thromboxane A2) and lead to increased cardiovascular thrombotic events. COX-2 inhibitors can also result into increase blood pressure, macular eruptions, urticaria, pseudoporphyria, erythema multiforme, oedema, worsening of heart failure, fatal allergic vasculitis and aggravation of doxorubicin-mediated cardiac injury. The COX-2 enzyme is also involved in the development of many organ systems, and its inhibition may lead to various congenital defects in neonates. It has been reported that COX-2 inhibitors also interfere with implantation, hence their use should be avoided in sexually active women at risk of pregnancy. However, presently the choice of COX-2 selective inhibitors for a particular patient should be based upon their relative efficacy, toxicity, concomitant drug use, concurrent disease states, hepatic and renal function and relative cost.

Adult↗

Work-related toxic epidermal necrolysis?

A case of toxic epidermal necrolysis is described in an employee of a company that carries out plastic extrusion using various resins, including cellulose acetate, co-polyester, polyvinyl chloride, acrylonitrile-butadienestyrene, and polyethylene. Air sampling during normal operating conditions indicated only trace amounts of hydrogen chloride and the plasticizer diethylphthalate. However, pyrolysis products of resin plugs could include compounds such as formaldehyde, acrylonitrile, and chlorinated hydrocarbons that have been associated with previous case reports of either erythema multiforme, Stevens-Johnson syndrome, or toxic epidermal necrolysis. The development of toxic epidermal necrolysis in this worker was directly preceded by exposure to a vapor from a machine used to dry cellulose acetate. The problems in determining work relatedness and advising about return to work are discussed.

Adult↗

Desquamative vulvitis.

The typical appearance of inflammatory and bullous diseases may be changed when they occur on the vulva. The moist, warm, occluded environment produces a tendency for the thin skin of the vulva to erode and scar, resulting in a common final appearance often characterized by loss of the labia minora and agglutination of the clitoral hood. The most common diseases that may produce desquamative vulvitis include lichen sclerosus, lichen planus, immunobullous diseases, contact dermatitis, erythema multiforme, lupus erythematosus, and squamous cell carcinoma in situ. The differentiating characteristics and treatment of these diseases are discussed in this article.

Adult↗

Surveillance guidelines for smallpox vaccine (vaccinia) adverse reactions.

CDC and the U.S. Food and Drug Administration rely on state and local health departments, health-care providers, and the public to report the occurrence of adverse events after vaccination to the Vaccine Adverse Event Reporting System. With such data, trends can be accurately monitored, unusual occurrences of adverse events can be detected, and the safety of vaccination intervention activities can be evaluated. On January 24, 2003, the U.S. Department of Health and Human Services (DHHS) implemented a preparedness program in which smallpox (vaccinia) vaccine was administered to federal, state, and local volunteers who might be first responders during a biologic terrorism event. As part of the DHHS Smallpox Preparedness and Response Program, CDC in consultation with experts, established surveillance case definitions for adverse events after smallpox vaccination. Adverse reactions after smallpox vaccination identified during the 1960s surveillance activities were classified on the basis of clinical description and included eczema vaccinatum; fetal vaccinia; generalized vaccinia; accidental autoinoculation, nonocular; ocular vaccinia; progressive vaccinia; erythema multiforme major; postvaccinial encephalitis or encephalomyelitis; and pyogenic infection of the vaccination site. This report provides uniform criteria used for the surveillance case definition and classification for these previously recognized adverse reactions used during the DHHS Smallpox Preparedness and Response Program. Inadvertent inoculation was changed to more precisely describe this event as inadvertent autoinoculation and contact transmission, nonocular and ocular vaccinia. Pyogenic infection also was renamed superinfection of the vaccination site or regional lymph nodes. Finally, case definitions were developed for a new cardiac adverse reaction (myo/pericarditis) and for a cardiac adverse event (dilated cardiomyopathy) and are included in this report. The smallpox vaccine surveillance case definitions presented in the report can be used in future vaccination programs to ensure uniform reporting guidelines and case classification.

Adverse Drug Reaction Reporting Systems↗

[Diseases that affect both skin and oral mucosa].

BACKGROUND: Several diseases may affect both skin and oral mucosa. This article presents hereditary, autoimmune and other inflammatory conditions within this group. MATERIAL AND METHODS: Review of the literature as well as the authors' own experience in the field. RESULTS AND INTERPRETATION: Lichen planus, erythema multiforme and lupus erythematosus are relatively common disorders with generally favourable prognosis. The much less frequent bullous diseases pemphigoid and pemphigus are characterized by a high morbidity, often requiring a deliberate use of immunosuppressants. Stevens-Johnson syndrome, toxic epidermal necrolysis and the more severe forms of epidermolysis bullosa are potentially life-threatening conditions. It is recommendable that medical doctors and dentists are familiar with the clinical presentations and diagnostic procedures of diseases affecting skin and oral mucosa.

Autoimmune Diseases↗

[Adverse drug effects in the oral region].

Syphilis was previously termed "the great imitator" because so many of the signs and symptoms are indistinguishable from those of other diseases. This role has been taken over by drugs, and this also applies to adverse drug reactions in the oral region. Accordingly, a careful drug history, including identification of any prescription, over-the-counter, or herbal medicines used, may give an important clue to the differential diagnosis of oral diseases when the aetiology is not apparent. Virtually all drugs have the potential to cause oral adverse reactions, but some have a greater ability to do so than others. Among the numerous adverse oral manifestations are xerostomia, taste disturbances and ulceration. The reactions are often non-specific, but they may mimic specific disease states such as erythema multiforme, lichen planus and pemphigus. Drug-induced gingival hyperplasia is an example of a quite characteristic and easily recognisable oral side effect. This article briefly describes some of the presentations and mechanisms of oral manifestations of drug therapy and the drugs that most commonly are responsible. Just like approved pharmaceuticals, herbal medicines are also associated with adverse oral manifestations. Finally we comment on some of the more recent reports on osteonecrosis of the jaws associated with the use of bisphosphonates.

Diphosphonates↗

[A case of follicular lymphoma complicated with lethal pemphigus].

Paraneoplastic pemphigus (PNP) is a rare autoimmune bullous disease associated with neoplasm, which is clinically characterized by mucocutaneous lesions resembling pemphigus vulgaris or erythema multiforme. A case presented with PNP refractory to chemotherapy including rituximab, predonisolone and cyclophosphamide (RCHOP regimen). A 36-year-old man, who had been diagnosed as extended follicular lymphoma, presented with a polymorphous skin eruption of the trunk, sclera conjunctivitis, and severe mucosal erosions of the lips and oral cavity. He was diagnosed as pemphigus pathologically by a biopsy of the oral mucosa. However, 3 courses of rituximab and CHOP therapy, which exert a partial response with lymphoma lesions, did not prove effective for oral stomatitis due to pemphigus. He received corticosteroid therapy (prednisolone 40 mg/day) and went into a state of temporally remission regarding pemphigus. However, the mucosal lesions were again exacerbated despite control of the lymphoma status after chemotherapy. Oral stomatitis extended to the upper respiratory system through the larynx and resulted in bronchiolitis obliterance clinically presented likely as severe chronic obstructive pulmonary disease (COPD). Because it is known that PNP refractory to long-term steroid and cytoreductive therapy has a progressive character and poor prognosis, supportive care would be warranted for these patients.

Adult↗

[The immune system of the skin and stereotyped reaction patterns in inflammatory skin diseases].

The clinical and histological presentation of inflammatory disease in the skin is exceedingly heterogeneous. Nonetheless, most inflammatory dermatoses can be classified according to five stereotypical tissue-reaction patterns: the spongiotic, the lichenoid, the psoriasiform, the vesiculo-bullous and the vasculopathic. By means of potent antigen-presenting cells, cytokine and chemokine cascades and a skin-specific cutaneous lymphocyte antigen (CLA)-positive lymphocyte population, the skin is able to respond very efficiently to pathogens that threaten the individual. Inflammatory skin diseases follow the rules and routes of the physiological reaction to inflammation but however for various reasons the immune response may be inadequate, enhanced or chronic. In allergic contact dermatitis, which is a prototype of the spongiotic reaction pattern, the inflammation is directed against an otherwise harmless antigen, for which the body is sensibilized. Lichenoid dermatitis (like erythema multiforme, graft versus host disease or lupus erythematodes) is based on a primary cytotoxic reaction against the basal epithelial cell, due to alterations in its antigenic make-up or due to an altered immune response. In psoriasis, an example of psoriasiform dermatitis, the interaction between inflammatory cells, antigen presenting cells and epithelial cells is disturbed.

Antigen-Presenting Cells↗

Adverse events following pertussis and rubella vaccines. Summary of a report of the Institute of Medicine.

In August 1991, the Institute of Medicine released a report entitled Adverse Effects of Pertussis and Rubella Vaccines, which examined 18 adverse events in relation to diphtheria-tetanus-pertussis (DTP) vaccine and four adverse events in relation to the currently used rubella vaccine strain, RA 27/3. The committee spent 20 months reviewing a wide range of information sources, including case series and individual case reports, both published and unpublished, epidemiologic studies, studies in animals, and other laboratory studies. The committee found that the evidence indicates a causal relation between DTP vaccine and anaphylaxis and between the pertussis component of DTP vaccine and extended periods of inconsolable crying or screaming. The committee also reported that the evidence indicates a causal relation between the rubella vaccine and acute arthritis in adult women. The committee found the available evidence weaker but still consistent with a causal relation between DTP vaccine and two conditions--acute encephalopathy and hypotonic, hyporesponsive episodes--and between rubella vaccine and chronic arthritis in adult women. Estimated incidence rates of these adverse events following vaccination are provided, where possible. The committee found that the evidence does not indicate a causal relation between the DTP vaccine and infantile spasms, hypsarrhythmia, Reye's syndrome, and sudden infant death syndrome. The committee found insufficient evidence to indicate either the presence or absence of a causal relation between DTP vaccine and chronic neurologic damage, aseptic meningitis, erythema multiforme or other rash, Guillain-Barré syndrome, hemolytic anemia, juvenile diabetes, learning disabilities and attention-deficit disorder, peripheral mononeuropathy, or thrombocytopenia, and between rubella vaccine and radiculoneuritis and other neuropathies or thrombocytopenic purpura. The committee's evaluative methods are briefly described and a summary of research needs is provided.

Acute Disease↗