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Biomedical subjects

L D Kaufman

Publications and source records attributed to L D Kaufman.

At least 19 recordsLinked to original sources
PubMed1992

Mast cell changes in scleroderma. Presence of MCT cells in the skin and evidence of mast cell activation.

OBJECTIVE: To analyze the concentration and distribution of the MCT (tryptase-positive, chymase-negative) and MCTC (tryptase-positive, chymase-positive) types of mast cell in cutaneous lesions of scleroderma. METHODS: Biopsy specimens were obtained from skin lesions in 24 patients with scleroderma, and subjected to double immunohistochemical analysis using mouse monoclonal anti-tryptase and anti-chymase antibodies. RESULTS: Dermal mast cell concentrations were below the normal range in 12 of the specimens, most of which were obtained between 1 and 4 years after disease onset. All other specimens contained normal concentrations of mast cells. MCT cells were present in 12 specimens and comprised between 8% and 100% of the total mast cells. Extracellular tissue deposits of tryptase-positive and/or chymase-positive granular material were observed in 8 specimens, suggesting possible mast cell degranulation. CONCLUSION: These findings are in contrast to those in normal skin, where MCTC cells are essentially the only type of mast cell present in the dermis. The results suggest that mast cells are involved in the pathogenesis of cutaneous lesions in scleroderma.

Adult↗
PubMed1991

Clinical follow-up and immunogenetic studies of 32 patients with eosinophilia-myalgia syndrome.

31 patients with L-tryptophan-associated eosinophilia-myalgia syndrome (EMS) that developed during the United States outbreak in 1989 were followed up prospectively at a university hospital outpatient rheumatology clinic for 16 to 24 months from the onset of their illness. Another patient with EMS associated with L-tryptophan in 1988 was followed up for 30 months. 93% of the 28 survivors from the 1989 cohort continue to have symptoms affecting 1-4 organ systems (median 3) and 3 have died, so the disorder produces considerable morbidity and mortality. The chronic sequelae most often associated with long-term disability are sclerodermatous skin thickening (54%), sensorimotor polyneuropathy (61%), proximal myopathy (36%), and severe episodic myalgias (64%). Thrombocytopenia developed in 1 patient. HLA-class II typing revealed a non-significant trend towards an association with HLA-DR4. Early therapy with corticosteroids did not seem to prevent the development of chronic manifestations.

Adult↗
PubMed1991

A double-blind randomized controlled trial of ketotifen versus placebo in early diffuse scleroderma.

To determine the efficacy of the mast cell-stabilizing drug ketotifen in scleroderma, we conducted a 6-month, randomized, prospective, double-blind, placebo-controlled trial in 24 patients. No significant improvement in the clinical parameters, pulmonary function, global assessments, and mast cell releasability was noted. Pruritus tended to improve in the group taking the active drug. Six months of treatment with ketotifen (6 mg/day), therefore, produced no apparent benefit in patients with early scleroderma. We were unable to address the role of mast cells in scleroderma since mast cell suppression was not achieved.

Adult↗
PubMed1991

The neuromuscular pathology of the Eosinophilia-Myalgia syndrome.

The Eosinophilia-Myalgia Syndrome (EMS) is a recently reorganized disorder in patients ingesting pharmacologic doses of L-tryptophan. We studied the lesions of skeletal muscle, peripheral nerve and skin in 12 cases of EMS. Perimyositis was severe in four, moderate in two, mild in three and absent in three cases. The lesions contained many eosinophils, T-helper cells, mast cells and activated macrophages. Type 2 myofiber atrophy was present in five cases and in one, this was the only pathologic finding. Severe epineurial inflammation was seen in the three sural nerve biopsies. Indirect evidence for peripheral neurologic involvement in three other cases consisted of inflammation surrounding intramuscular nerve twigs (two cases) and neurogenic atrophy (one case). Phlebitis accompanied the connective tissue inflammation in five cases and endarteritis in one. Fasciitis was present in three of four skin biopsies and dermal fibrosis in one.

Adult↗
PubMed1991

L-tryptophan-associated eosinophilia-myalgia syndrome: perspective of a new illness.

The current knowledge of the eosinophilia-myalgia syndrome as an evolving new disease entity is reviewed in the context of previously described eosinophilic disorders. The acute and chronic manifestations parallel the findings of the toxic oil syndrome of Spain and include scleroderma-like skin disease, neuropathy, and myopathy.

Eosinophilia↗
PubMed1991

The eosinophilia-myalgia syndrome and related disorders.

The recognition of the eosinophilia-myalgia syndrome associated with L-tryptophan in the United States during 1989 as a disorder resembling the previously described 1981 toxic oil syndrome of Spain has established an increased level of consciousness regarding drug and toxin associated diseases. Both of these disorders were characterized by the development of acute and chronic multisystem features that parallel many idiopathic connective tissue diseases. Common manifestations have included generalized myalgias, fever, transient pulmonary infiltrates, and xerostomia during the early months followed by late stage neuromuscular and cutaneous disease. The most conspicuous laboratory abnormality was a peripheral eosinophilia. One of the most striking clinical findings has been scleroderma-like skin disease manifesting as diffuse fasciitis or hidebound induration. A sensory neuropathy and proximal myopathy in association with skin thickening have established these syndromes as chronic disabling diseases for many of their victims. Mononuclear perimysial and epineurial infiltrates have been distinctive pathological findings. Although the etiology of the eosinophilia-myalgia syndrome and the toxic oil syndrome are unknown, there is epidemiologic evidence to support the presence of contaminants in L-tryptophan and rapeseed oil, respectively, as the causative agents. No therapy has been demonstrated to arrest the evolution of the chronic sequelae in either disorder.

Eosinophilia↗
PubMed1990

Cutaneous manifestations of the L-tryptophan-associated eosinophilia-myalgia syndrome: a spectrum of sclerodermatous skin disease.

The natural history of the clinical and pathologic features of skin disease was reviewed prospectively in 30 patients with the L-tryptophan-associated eosinophilia-myalgia syndrome. Overall, cutaneous manifestations developed in 26 patients (87%). Early lesions were nonspecific and characterized predominantly by an erythematous macular eruption on the trunk and extremities. The most characteristic abnormality noted was the spectrum of sclerodermatous disease in 15 patients (50%) often after a subacute stage of peripheral or truncal edema. Clinical and/or biopsy evidence of eosinophilic fasciitis was seen in nine patients (30%). Findings consistent with diffuse, limited, or localized scleroderma were subsequently observed in nine patients (33%). Small mucinous papules, similar to those seen in scleromyxedema, were found in five patients (17%). Alopecia, frequently a late sequela, developed in 11 (37%). Common histologic features included papillary dermal fibrosis, dermal and fascial infiltrates consisting of mononuclear cells and eosinophils, deposition of glycosaminoglycans in the dermis, and, in some patients, numerous mast cells.

Adult↗
PubMed1990

L-tryptophan-associated eosinophilic perimyositis, neuritis, and fasciitis. A clinicopathologic and laboratory study of 25 patients.

We have described the spectrum and prevalence of the clinical and laboratory manifestations of a multisystem disorder associated with the ingestion of L-tryptophan. At least 3 subsets of clinical disease have been identified: 1) a neuromuscular disorder which may present with myalgias and mild weakness and then progress to quadriparesis related to an axonal neuropathy and interstitial myositis (perimyositis), 2) a syndrome of eosinophilic fasciitis with characteristic cutaneous induration, and 3) the Löffler syndrome consisting of pulmonary infiltrates with eosinophilia. Corticosteroids may be useful for patients with the Löffler syndrome and offer only a modest benefit in the majority of patients with neuromuscular disease. The clinical course appears to be chronic, and the long-term sequelae of this disorder are unknown. The etiologic agent remains undetermined; however, studies are in progress to examine the mechanism of eosinophilia, appropriate therapeutic intervention, and the long-term outcome of the affected individuals.

Adult↗
PubMed1990

Eosinophilic neuritis, perimyositis, and vasculitis associated with ingestion of L-tryptophan.

Four cases are described of a clinical syndrome which developed in the setting of L-tryptophan ingestion. The major manifestations consisted of myalgias, neuropathy, weakness, and profound eosinophilia. Pathologically a vasculitis involving predominantly small veins was observed along with a mixed cellular infiltrate in the perimysium and epineurium. Clusters of eosinophils were characteristically noted in the tissue specimens. The clinical course appears to be chronic although further longterm followup will be required. One patient pursued a relentless downhill course with progressive neurologic impairment and death. Although the mechanism of tissue injury in these individuals is speculative, the possible association of this widely used nonprescription medication with this syndrome should be recognized.

Administration, Oral↗
PubMed1989

Male lupus: retrospective analysis of the clinical and laboratory features of 52 patients, with a review of the literature.

In conclusion, in a review of 52 male lupus patients we found an increased incidence of thrombocytopenia and renal disease without any other notable differences in clinical, laboratory, or serological parameters. Of further interest was the finding of an early peak in the age of onset of disease resembling that found among female SLE patients, and a generally poor prognosis as has been reported by others.

Adolescent↗