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Biomedical subjects

D L Tuffanelli

Publications and source records attributed to D L Tuffanelli.

14 recordsLinked to original sources

Systemic scleroderma.

Scleroderma encompasses a wide variety of diseases, including localized scleroderma, overlap syndromes, sclerodermoid conditions, and systemic scleroderma. This article emphasizes the systemic scleroderma.

Humans

Speckled (particulate) epidermal nuclear IgG deposition in normal skin. Correlation of clinical features and laboratory findings in 46 patients with a subset of connective tissue disease characterized by antibody to extractable nuclear antigen.

Clinical and laboratory findings were correlated from 46 patients with IgG localization in epidermal nuclei in a speckled (particulate) pattern on direct immunofluorescence of normal skin. Cutaneous manifestations included lupus erythematosus (LE), swollen hands or sclerodactyly, alopecia, vasculitis, and dyspigmentation. Systemic manifestations included arthritis or arthralgia, Raynaud's phenomenon, serositis, vascular headaches, mild renal disease, myositis, and sicca syndrome. High titer (mean = 1:142, 800) serum antibody to extractable nuclear antigen (ENA) was found in 81%. Eighty-six percent had antibody to an RNase-sensitive antigenic component of ENA (ribonucleoprotein or RNP); 14% had antibody to an RNase-resistant ENA termed Sm. Deposition of IgG in a speckled pattern in epidermal nuclei is an immunopathologic marker for a subset of connective tissue disease characterized by antibody to ENA. Those with Sm specificity had systemic LE (SLE); Those with RNP specificity had Raynaud's phenomenon usually associated with overlapping features of SLE, scleroderma, and/or dermatomyositis.

Adolescent

Porphyria-like cutaneous changes induced by tetracycline hydrochloride photosensitization.

Five patients manifested cutaneous changes indistinguishable from those noted in some porphyric disorders, consisting of fragility, denudation, and blister formation of sun-exposed skin. Microscopical examination showed subepidermal bulla formation and the desposition of PAS-positive, diastase-resistant material and IgG in or around the upper dermal blood vessel walls. There was also electron microscopical evidence of vascular basal lamina reduplication and the deposition of a fine fibrillar material in and around these vessels. However, no abnormal porphyrin formation was noted. All five patients had been receiving 250 mg of tetracycline hydrochloride twice a day for at least six months and had had extensive sun exposure prior to the onset of the condition. For four patients, discontinuing the medication led to complete remission, despite subsequent sun exposure; the fifth patient was much improved, but her skin was still somewhat fragile seven months later. We concluded that these cutaneous changes resulted from a low-grade photosensitization by tetracycline hydrochloride.

Acne Vulgaris

Cutaneous immunopathology: recent observations.

Immunofluorescent studies are currently being done on patients with pemphigus, pemphigoid, dermatitis, lupus erythematosus and its variants, the cutaneous prophyrias, scarring alopecia, erosive mouth lesions, light-sensitive disorders, and cutaneous vasculitis. In this paper I shall review some of the recent advances in immunopathology and report the results that have been obtained in our laboratory.

Alopecia

Incontinentia pigmenti and defective neutrophil chemotaxis.

A child with incontinentia pigmenti and chronic erythema multiforme had recurrent bacterial infections. Greatly elevated serum IgE was found. In addition, the patient's neutrophils showed essentially no chemotaxis toward Staphylococcus aureus, Escherichia coli, or Pseudomonas aeruginosa in either patient or control serum. Neutrophil phagocytosis and killing function were normal.

Adult