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Biomedical subjects

R L Rietschel

Publications and source records attributed to R L Rietschel.

At least 19 recordsLinked to original sources
PubMed1992

Treatment of systemic sclerosis with extracorporeal photochemotherapy. Results of a multicenter trial.

BACKGROUND AND DESIGN: In a pilot study of extracorporeal photochemotherapy, two patients with systemic sclerosis who received this therapy experienced significant clinical improvement. These results prompted the development of a multicenter trial to examine the benefit of extracorporeal photochemotherapy in the treatment of systemic sclerosis. Seventy-nine patients with systemic sclerosis of recent onset (mean symptom duration, 1.83 years) and progressive skin involvement during the preceding 6 months entered a randomized, parallel-group, single-blinded clinical trial comparing extracorporeal photochemotherapy treatments given on 2 consecutive days monthly with treatment with D-penicillamine at a maximum dose of 750 mg/d. Blinded clinical examiners evaluated skin severity score (thickness), percent surface area involvement, oral aperture, and hand closure. Serial skin biopsies and pulmonary function studies were also performed. RESULTS: Following 6 months of treatment, significant improvement in skin severity score occurred in 21 (68%) of 31 patients receiving photochemotherapy and in eight (32%) of 25 receiving D-penicillamine treatment, while significant worsening occurred in three (10%) of 31 receiving photochemotherapy and in eight (32%) of 25 receiving penicillamine treatment, thus indicating a significantly higher response rate for individuals who received photochemotherapy (P = .02). At both the 6- and 10-month evaluation points, the mean skin severity score, mean percent skin involvement, and mean oral aperture measurements were significantly improved from baseline among those who received photochemotherapy. Mean right and left hand closure measurements had also improved significantly by 10 months of therapy. By comparison, among the patients treated with D-penicillamine, none of the parameters of cutaneous disease had improved significantly after 6 months of therapy, although for those individuals in whom treatment was continued, the mean skin severity score and mean percent skin involvement had improved by 10 months. Skin biopsy studies revealed a correlation between clinical improvement and decreased thickness of the dermal layer. Adverse effects of extracorporeal photochemotherapy were minimal and did not require discontinuation of treatment in any of the patients receiving this therapy; six patients permanently discontinued the use of D-penicillamine treatment due to adverse effects. CONCLUSIONS: For patients with systemic sclerosis of recent onset, extracorporeal photochemotherapy is a well-tolerated treatment that may partially reverse the process that results in cutaneous sclerosis.

Adult↗
PubMed1991

Alpha 1-antitrypsin deficiency-associated panniculitis: case report and review of the literature.

Alpha 1-antitrypsin is the primary serum proteinase inhibitor. Alpha 1-antitrypsin deficiency, especially the ZZ genotype, has been linked mainly to emphysema and cirrhosis; it is also associated with paniculitis. A case of alpha 1-AT-associated panniculitis was documented in a 13-year-old girl in whom a deficiency of the enzyme was known to be present from infancy. This is unusual, since alpha 1-AT panniculitis previously was described in older patients without prior knowledge of the duration of the deficiency. Our patient developed erythematous, subcutaneous nodules subsequent to trauma, which later developed into deep, painless ulcers. We report this case so that the condition may be suspected in patients with panniculitis. The diagnosis may be confirmed by measuring quantitative alpha 1-AT serum levels and by enzyme genotyping. The treatment of choice is dapsone.

Adolescent↗
PubMed1979

Histopathogenesis of skin and subcutaneous injury induced by adriamycin.

Extravasation of Adriamycin from an intravenous needle or catheter can produce a progressive skin necrosis and deep painful ulceration. The pathological changes which result in this ulceration were studied in a rabbit model. The earliest changes include vascular obliteration and necrobiosis of collagen. At no point were inflammatory cells found to play a primary role.

Animals↗
PubMed1978

A method to evaluate skin moisturizers in vivo.

Blank demonstrated that water is the only plasticizer of human stratum corneum. Moisturizers attempt to add and hold water within the horny layer, and have previously been evaluated subjectively or in vitro. By passing a stream of dry nitrogen over skin, then through an electrolytic moisture analyzer, moisture present at the skin surface will be detected. This technique has been utilized to measure transepidermal water loss and the inhibition of water loss from skin by various investigators. However, when this same procedure is done on normal human skin, higher values indicate the presence of more moisture. The more moisture detectable at the surface, the more moisture available for keratin to absorb. By applying the moisturizers to plastic film and the skin, it can be demonstrated that water is not detectable after a few minutes on the plastic, but is detectable on skin at higher than control (transepidermal water loss) values for several hours. The technique can demonstrate enhanced moisturization from plastic occlusion and wet dressings as well as the drying effect of benzoyl peroxide gels. It allows objective rank ordering of moisturizers by monitoring moisture enhancement rather than occlusivity of applied substances.

Benzoyl Peroxide↗
PubMed1978

Heat loss in anhidrotic ectodermal dysplasia.

The dynamics of heat loss by 2 patients with classic anhidrotic ectodermal dysplasia were studied. Both were active in high school athletics and avoided heat injuries by various forms of behavior modification. Elevated core and skin temperature measurements were found at rest in comfortable environments. In a warm environment 35-45% of the heat generated was lost by radiation, 44-52% by conduction and convection, and only 4-6% by evaporation. Heat loss in control subjects was 9% by radiation, 17% by conduction/convection, and 67% by evaporation. The dry routes of heat dissipation used by the anhidrotic patients were inadequate to prevent a rise in core temperature.

Adolescent↗
PubMed1978

Symmetrical lividity of the palms and soles.

Symmetrical lividity (SL) was the term coined by Pernet in 1925 for symmetrical, bluish-red plaques on the soles of the feet, accompanied by hyperhidrosis and not corresponding to areas of pressure or patterns of innervation. We report two patients with a persistent eruption of the palms analogous to that described by Pernet on the feet. Unlike most reported cases of SL, our patients did not respond to topical drying treatments, but one patient partially responded to tretinoin. There appear to be two forms of SL: transient, which responds to drying; and persistent which does not respond to drying. The hyperhidrosis studied in one of our two patients was significantly greater within the plaques of SL than the normal palm. While we could suppress the hyperhidrosis with topical therapy, this failed to clear his hyperkeratosis or eliminate the livid color.

Adult↗
PubMed1978

Photocontact dermatitis to hydrocortisone.

A patient was found to have a delayed positive patch test to hydrocortisone on multiple testing. This proved to be due to a photoallergy with an action spectrum in the UV-A range. The patient coincidently had a polymorphous light eruption in the UV-B range.

Administration, Topical↗
PubMed1978

Skin lesions in paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is an uncommon hemolytic anemia that rarely manifests skin lesions. Leg ulcers and purpura similar to the manifestation of thrombotic thrombocytopenic purpura (TTP); disseminated intravascular coagulation, and Henoch-Schönlein purpura do occur. This is the second known case report of PNH with histopathologic features of TTP. Clinically, our patient's condition resembled TTP with disseminated intravascular platelet aggregation, and she followed a nonfulminant course. She improved on a regimen of systemic steroids, dipyridamole, and cessation of antibiotics.

Adult↗
PubMed1978

Contact dermatitis to homomenthyl salicylate.

Two patients with follicular dermatitis were found to have a contact sensitivity to homomenthyl salicylate, a sunscreening chemical present in a commercially available suntan lotion. One patient did not use the product, but her boyfriend did, and contact between the two individuals resulted in a follicular dermatitis developing in her. A second patient with contact dermatitis to homomenthyl salicylate also had a follicular eruption. Both patients appear to represent true allergic sensitivities.

Adult↗
PubMed1978

Invisible dermatoses.

An invisible dermatosis is defined as a skin disease manifesting no clinically apparent lesion but histologic changes of a characteristic nature. An example of an invisible form of transient acantholytic dermatosis is presented. Invisible forms of pseudoxanthoma elasticum, sarcoidosis, lepromatous leprosy, and lichen planus are reviewed. Dermal deposits may also be found on biopsy of clinically normal skin in amyloidosis, Hunter's and Hurler's syndromes, and thyroid disease.

Acantholysis↗