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[Dermatological manifestations of Sjögren's syndrome].

In primary Sjögren's syndrome, various cutaneous manifestations may become apparent: annular erythema or eruptions reminiscent of erythema multiforme, lichen planus or erythema nodosum (dermo-panniculitis), chilblain-like erythema, purpura, cheilitis, and xerosis, are some of them. In the clinical diagnosis, the following laboratory tests are of value: Schirmer test, rose bengal test, fluorescence staining techniques, gum test, sialography, sialoscintigraphy, lip biopsy. In some cases, dryness can be absent though the above-mentioned skin manifestations are observed. Mothers with Sjögren's syndrome can deliver babies with neonatal lupus erythematosus. Serological testing almost always reveals anti-Ro/SSA-autoantibody and the more specific anti-La/SSB in the sera of patients. The sensitivity of detection can be enhanced by using the immunoblotting technique and the two-dimensional immunoblotting method. In some cases, a new antibody against carbonic anhydrase has been detected.

Adult↗

Cutaneous drug reactions: pathogenesis and clinical classification.

Cutaneous drug reactions may be classified with respect to pathogenesis and clinical morphology. They may be mediated by immunologic and nonimmunologic mechanisms. Immunologic reactions require host immune response and may result from IgE-dependent, immune complex-initiated, cytotoxic, or cellular immune mechanisms. Nonimmunologic reactions may result from nonimmunologic activation of effector pathways, overdosage, cumulative toxicity, side effects, ecologic disturbance, interactions between drugs, metabolic alterations, or exacerbation of preexisting dermatologic conditions. Certain defined, cutaneous, morphologic patterns are frequently associated with cutaneous drug reactions. These include urticaria, photosensitivity eruptions, erythema multiforme, disturbance of pigmentation, morbilliform reactions, fixed drug reactions, erythema nodosum, toxic epidermal necrolysis, lichenoid eruptions, and bullous reactions. In addition, certain drugs cause defined cutaneous syndromes. These include iodides and bromides, hydantoins, corticosteroids, antimalarial agents, gold, cancer chemotherapeutic agents, tetracyclines, thiazides and sulfonamides, nonsteroidal anti-inflammatory agents, and coumarin. The criteria for evaluation of possible drug reactions are presented and reviewed.

Adrenal Cortex Hormones↗

Photodermatitis multiformis acuta.

A condition precipitated by acute sun exposure, occurring primarily in women, and having all the characteristics of erythema multiforme is reported.

Acute Disease↗

Inflammatory diseases of the mucous membranes. Part 2. Immunopathologic ulcerative and desquamative diseases.

Bullous and desquamative diseases of the oral mucosa are not common, with the exception of ulcerative lichen planus. All produce mild to severe mucosal pain and some can be life-threatening. Recent studies have helped elucidate the pathogenesis of immunopathologic diseases, although the precise etiology for most of them remains unknown. Minor and major aphthae, lichen planus, pemphigoid, pemphigus and erythema multiforme are discussed.

Diagnosis, Differential↗

Management of non-genital herpes simplex virus infections in immunocompetent patients.

Non-genital herpes simplex virus in immunocompetent hosts causes a variety of primary infections--gingivostomatitis, keratoconjunctivitis, herpetic whitlow, and encephalomyelitis. Recurrent infections with orolabialis are very common, but are usually mild and self-limiting. Cutaneous complications of herpes simplex virus infections include eczema herpeticum and erytherma multiforme. Systemic treatment with acyclovir is indicated in encephalomyelitis, progressive eczema herpeticum, and frequent severe erythema multiforme. Chronic, suppressive acyclovir treatment may be helpful in severe recurrent infections or those complicated by erythema multiforme/dissemination. Many primary and recurrent infections can be treated with simple topical therapy to control secondary infection. There is no evidence that systemic treatment affects viral latency or recurrent infections following discontinuation of treatment.

Acyclovir↗

Histopathologic pattern analysis of human intracutaneous tuberculin reaction.

The involvement of cellular immune reactions in the pathogenesis of many inflammatory dermatoses can be postulated from their histopathologic features, e.g., those dermatoses that show a perivascular lymphohistiocytic infiltration in the dermis in addition to their own characteristic epidermal changes. Several granulomatous dermatoses further show an additional infiltration of epithelioid cells. However, experimental production of similar changes is usually not feasible in animals. A human model is needed of delayed-hypersensitivity skin reaction induced by a definite antigen. After closely observing the histopathologic changes of the skin reactions to the purified protein derivative (PPD) of tuberculin, we noted that all patients did not show uniform histologic patterns at intradermal PPD injection sites. Therefore, we have tentatively analyzed histopathologically the pattern of tuberculin skin reactions at injection sites 48 h to 1 year after injection in eight healthy volunteers and 63 patients with various dermatoses. We classified the reactions at 48 h into three types, based on the extent of the tissue damage (i.e., their resemblance to features noted in corresponding inflammatory dermatoses): (a) the perivascular dermatitis type, (b) the basal spongiotic dermatitis type, and (c) the erythema multiforme type. There was a clear correlation between the magnitude of macroscopic skin reactions and these histopathologic patterns. Even those with clinically negative reactions showed a reaction pattern of perivascular dermatitis, whereas those who clinically developed prominent inflammatory reactions with blister formation always showed a erythema multiforme-like histologic pattern. Interestingly, in more than half of the cases, the dermal cellular infiltrate had a mixture of various numbers of neutrophils in addition to mononuclear cells.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Immunopathology of oral mucosal ulcerative, desquamative, and bullous diseases. Selective review of the literature.

Cell/cell and cell/matrix adhesion proteins are responsible for maintaining the integrity of the mucosal lining of the oral cavity. Disease processes that destroy keratinocytes or adversely affect their adhesion to one another or to the subjacent basement membrane will result in erosions, ulcerations, and desquamations. Immunologic processes that have a deleterious effect on the integrity of the epithelial/basement membrane/submucosa complex are reviewed, and current research findings with respect to pathogenesis are discussed. In particular, T-cell-mediated hypersensitivity is involved in recurrent aphthous stomatitis and lichen planus; humoral-mediated immunity to cadherin intercellular adhesion molecules is important in the process of acantholysis in pemphigus vulgaris, and genetic defects and antibody-mediated processes give rise to junctional separation in epidermolysis bullosa and mucous membrane pemphigoid, respectively. An immune complex mechanism appears to underlie the pathogenesis of erythema multiforme.

Autoantibodies↗

[Rowell's syndrome].

Two cases with this rare syndrome are described, with a combination of lupus erythematosus and erythema multiforme exudativum.

Adolescent↗

Blistering disorders in childhood.

Many conditions present in childhood with vesicles and bullae, ranging from benign conditions to life-threatening diseases. This article reviews selected blistering disorders, with emphasis on new information that is helpful to the practicing physician. Entities that are discussed in this article include childhood zoster and fetal varicella syndrome, urticaria pigmentosa, scabies, bullous bug bites, lichen sclerosus et atrophicus, epidermolysis bullosa, and erythema multiforme.

Child↗

Rowell's syndrome. Report of a case.

We describe a patient with discoid lupus erythematosus who developed annular lesions of the thigh and chilblainlike lesions of the fingers matching those described in the original reports of Rowell's syndrome. The patient also had circulating anti-Ro(SS-A) antibodies whose similarity to the anti-Sj-T antibodies found in the original Rowell's syndrome cases has been recently claimed. A review of the literature suggests that most of the cases of Rowell's syndrome described thus far in fact may be cases of coincidental association of lupus erythematosus and erythema multiforme.

Antibodies, Antinuclear↗