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Biomedical subjects

J C Maize

Publications and source records attributed to J C Maize.

13 recordsLinked to original sources

A histopathologic comparison of Shulman's syndrome (diffuse fasciitis with eosinophilia) and the fasciitis associated with the eosinophilia-myalgia syndrome.

A comparison of the histopathologic features of Shulman's syndrome (diffuse fasciitis with eosinophilia) and the fasciitis associated with the eosinophilia-myalgia syndrome is presented. The study population consisted of eight biopsy specimens of seven patients with Shulman's syndrome and 11 specimens from 10 patients with eosinophilia-myalgia syndrome. Both groups exhibited inflammatory changes in the subcutaneous fat, septa, and fascia; cutaneous changes were more prominent in cases of eosinophilia-myalgia syndrome. Eosinophils and plasma cells were not consistently present in either condition; mast cells and factor XIIIa-positive cells were consistently present in the inflammatory infiltrates. Although there was overlap in the histopathologic findings, Shulman's syndrome tends to involve the subcutis alone and the eosinophilia-myalgia syndrome tends to be a pancutaneous-subcutaneous process.

Eosinophilia

Morsicatio buccarum et labiorum (excessive cheek and lip biting).

In some individuals habitual cheek and lip biting becomes a fixed neurosis. Pieces of oral mucosa are actually torn free from the surface, producing a distinctive clinical appearance termed morsicatio buccarum et labiorum. Sometimes it may be confused with other dermatological disorders involving the oral mucosa, and can lead to misdiagnosis. Most patients with this condition are unaware of their habit and will not aid in the diagnosis. We suggest that the histopathological features of this condition are distinctive, if not pathognomonic, and that an accurate diagnosis should be rendered if a biopsy sample is taken. Two cases are reported herein that were originally misdiagnosed as pemphigus, one of which resulted in complications of improper oral corticosteroid use.

Adult

Scleroderma, fasciitis, and eosinophilia associated with the ingestion of tryptophan.

An association between the ingestion tryptophan and a syndrome characterized by scleroderma-like skin abnormalities, fasciitis, and eosinophilia has recently been recognized in the United States. We report the clinical and histopathological findings in nine patients and the results of biochemical analyses of tryptophan metabolism in seven patients with this syndrome. Edema of the extremities, frequently accompanied by pruritus, paresthesia, and myalgia, developed in the nine patients (six women and three men; age range, 30 to 66 years) 1 to 18 months after the start of therapy with tryptophan (1.5 to 3.0 g daily) for insomnia, depression, or obesity. Five patients were taking drugs (benzodiazepines) known to inhibit hypothalamic-pituitary-adrenal function, and one had adrenal insufficiency. All had blood eosinophilia in the acute phase of their illness (mean eosinophil count [+/- SD], 3.62 +/- 2.87 X 10(9) cells per liter). All had histopathological changes in the dermis and subcutaneous tissue typical of scleroderma, and seven patients had eosinophils. The fascia was inflamed and fibrotic, and adjacent skeletal muscle often showed perifascicular inflammation. Tryptophan was discontinued in all patients, and eight received prednisone. The cutaneous symptoms improved, but only two patients had complete resolution of their illness. The patients had plasma levels of tryptophan before and after an oral dose of tryptophan that were similar to those in normal subjects. Plasma levels of L-kynurenine and quinolinic acid, which are metabolites of tryptophan, were significantly higher in four patients with active disease than in three patients studied after eosinophilia had resolved or in five normal subjects (P less than 0.001)--findings consistent with the activation of the enzyme indoleamine-2,3-dioxygenase. This illness resembles eosinophilic fasciitis and probably represents one aspect of the recently reported eosinophilia-myalgia syndrome. The development of the syndrome may result from a confluence of several factors, including the ingestion of tryptophan, exposure to agents that activate indoleamine-2,3-dioxygenase, and possibly, impaired function of the hypothalamic-pituitary-adrenal axis.

Adult

Unusual subepidermal bullous diseases with immunologic features of bullous pemphigoid.

Sixty-seven patients with histologic and immunologic features of bullous pemphigold (BP) were evaluated. Eleven patients had a localized blistering disease that was predominantly confined to one area of the body, most commonly the lower extremities. Two patients displayed a dapsone-responsive blistering disease that was characterized by a flexural distribution of ten to 20 1-cm or less, intensely pruitic, subepidermal bullae and linear IgA basement membrane zone deposition. Two patients had a chronic recalcitrant generalized scarring, hyperkeratotic, subepidermal blistering eruption that demonstrated serologic and direct immunofluorescence (IF) findings of BP. One patient displayed grouped small vesicles surmounted on an erythematous base; the clinical diagnosis was dermatitis herpetiformis, but direct IF examination of the biopsy specimen showed features of BP. One patient with epidermolysis bullosa acquisita had serologic and direct IF features suggestive of BP.

Adult

Nevus of large spindle and/or epithelioid cells (Spitz's nevus).

By now it is well recognized that there is a benign melanocytic nevus, common in the young and common enough in adults, that has histological features that are confusable with those of malignant melanoma. The anomaly is usually referred to as benign juvenile melanoma, sometimes as Spitz's nevus, and, by some histopathologists, as spindle and epithelioid cell nevus. All the histological subtleties and variations of the condition are still not fully appreciated and some of them are still being misinterpreted as those of malignant melanoma. We herewith present a study designed to clarify the issue and offer firm criteria for histological differentiation of the nevus in point from malignant melanoma. We also suggest a new name for it and supporting arguments therefor.

Adolescent

Bullous pemphigoid. Clinical and immunologic follow-up after successful therapy.

Thirty-six patients with bullous pemphigoid (BP) have been periodically evaluated for four years. This study demonstrates that BP may occur in a transient predominantly localized form that remits spontaneously, and most BP patients after successful therapy remain in prolonged clinical remission. In this study, all patients with active or recurrent disease had IgG and/or C3 basement membrane zone (BMZ) deposition. Serum anti-BMZ antibodies was an inconstant feature. In most instances, clinical remission of BP was associated with disappearance of BMZ ig and C3 deposition and serum BMZ antibodies. Fluorescein-conjugated, antihuman C3 appears to be a more sensitive immunoreagent than antihuman, class specific, immunoglobulin antisera in detecting positive BMZ staining in BP. Combined therapy with azathioprine plus prednisone appears to be superior to prednisone alone in the treatment of BP.

Adult

Atopic dermatitis.

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Antibody Formation

Pemphigus and myasthenia gravis.

Various forms of true pemphigus have been reported to occur with myasthenia gravis, with and without thymoma, more frequently than can be ascribed to chance. A57-year-old woman with myasthenia gravis in remission developed pemphigus foliaceus. No roentgenologic evidence of a thymoma was present. Antinuclear antibodies caused interference with indirect immunofluorescent determination of pemphigus antibody. Indirect immunofluorescent studies showed no evidence for cross-reactivity between intercellular substance antibody and thymic components, as is found in the case of antiskeletal muscle antibodies with thymic myoid cells. The concurrence of these two diseases may involve the failure of a subgroup of thymic-dependent lymphocytes to suppress underlying autoimmune phenomena.

Animals