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Biomedical subjects

S A Muller

Publications and source records attributed to S A Muller.

At least 55 records · Page 3Linked to original sources
PubMed1989

Necrobiosis lipoidica diabeticorum: platelet survival and response to platelet inhibitors.

Results of an open trial of platelet inhibitor treatment for necrobiosis lipoidica diabeticorum suggest the possible importance of abnormal platelet function in this disease. In ten female patients with necrobiosis lipoidica diabeticorum (six who were diabetic and four who were not) platelet survival times were measured before and after treatment with aspirin and dipyridamole. Pretreatment platelet survival time was considerably shortened in 50 percent of the diabetic and nondiabetic patients. Platelet-inhibitor treatment prolonged platelet survival time toward normal in most of these patients. The clinical response to treatment varied from healing to no noticeable effect.

Adult↗
PubMed1988

Prospective study of cutaneous phototoxicity after systemic hematoporphyrin derivative.

Hematoporphyrin derivative (HpD) is a photoactive, oncophilic substance that produces cutaneous photosensitivity as its only significant side effect. Twenty-three patients who received systemic HpD and the usual light-avoidance precautions were studied prospectively to determine the incidence and severity of cutaneous phototoxicity (CP). Seventeen of the 23 patients (74%) reported CP, including three patients (18%) who experienced blister formation. Symptoms of CP occurred for a mean duration of 6 weeks (range 5-23 weeks). Lack of compliance with restrictive photoprotective measures was felt to be a major contributing factor. Other HpD-related complications included skin hyperpigmentation, ocular discomfort, pruritus, pain at injection site, and urticaria. CP and the restrictive measures to avoid it represent major disadvantages of the clinical use of HpD.

Adult↗
PubMed1988

Photopatch testing: a six-year experience.

The results of photopatch testing performed at the Mayo Clinic from 1980 through 1985 were analyzed. During the 6 years, 70 patients underwent photopatch testing. Of the 70 patients, 27 (38.5%) had positive photopatch test reactions and 22 (31%) had ordinary contact reactions. Of the 27 patients with photocontact reactions, 14 had relevant reactions. The most frequent positive reactions were to chlorpromazine (13 patients), musk ambrette (nine patients), and promethazine (eight patients). Photopatch testing is an essential part of the evaluation of patients who are suspected of having photosensitive dermatitis. In some patients with recalcitrant eruptions, testing may help in excluding photosensitivity as a possible contributing factor.

Adolescent↗
PubMed1988

Follicular mucinosis of childhood and adolescence.

We cared for nine patients who were less than 21 years old at the time of diagnosis of follicular mucinosis. Four had clearing of the lesions, four did not, and one was lost to follow-up. Follicular mucinosis, even in childhood, may be persistent and be associated with lymphoma, which can result in death. Because there is no clinical pattern or single histopathologic criterion that correlates with the outcome, clinical follow-up and assessment of several histopathologic criteria must be relied on when evaluating this condition in children.

Adolescent↗
PubMed1988

The cutaneous immunopathology of necrobiosis lipoidica diabeticorum.

Twelve female patients with necrobiosis lipoidica diabeticorum (six with diabetes and six without) had a 5-mm punch biopsy of the skin lesion performed. The tissue was processed for dermatopathologic examination in 12 cases and for direct immunofluorescence in 11. Vasculopathy with inflammation and thickening of vessel walls, at times leading to occlusion, was found in lesional skin in all 12 cases. Vessels contained deposits of immunoreactants in the involved skin in 11 cases. This included IgM in six, C3 in nine, fibrin in ten, IgG in one, and IgA in two. Vessels contained deposits of immunoreactants in uninvolved skin in seven patients (C3 in four, IgM in three, fibrin in three, C4 in one, and IgA in one), three of whom had type I diabetes.

Adult↗
PubMed1988

Aggressive intranasal carcinoma mimicking infection or inflammation.

In a twenty-year period we have treated five patients with intranasal squamous cell carcinomas who initially were thought to have benign cutaneous disorders of the nasal skin. Advanced squamous cell carcinoma requiring total rhinectomy, often with extensive contiguous midface excision, was found in all patients. Three of these five patients died of their disease. In one patient, a red nose resembling acne rosacea was present. In the four others, chronic cellulitic or ulcerative cutaneous involvement was caused by squamous cell carcinoma. These cases emphasize the need for intranasal examination and appropriate radiologic studies to exclude intranasal carcinoma in patients with apparently unresponsive cutaneous nasal disease.

Adult↗
PubMed1988

3 percent topical minoxidil compared with placebo for the treatment of chronic severe alopecia areata.

The efficacy of a 3 percent topical minoxidil solution (2,4-diamino-6-piperidinopyrimidine-3-oxide) in a propylene glycol-water-ethanol base applied twice daily for one year to half the scalp was evaluated in patients with severe chronic alopecia areata. A randomized, double-blind, bilateral comparison-controlled study design was used. Of the twenty-one patients, thirteen were women and eight were men; their ages ranged from nineteen to fifty-five years. All the patients had alopecia totalis or alopecia universalis, except for two who had lost two-thirds of their scalp hair. The mean disease duration was 11.5 years (range, one to forty years). Transient regrowth of sparse vellus hair occurred in twelve patients, bilaterally in eight, but it was not significant in any. No cosmetically acceptable results were achieved. No significant side effects were noted, except for a moderately severe bilateral dermatitis in one patient. The results indicate that 3 percent topical minoxidil solution is ineffective as treatment for severe chronic alopecia areata.

Administration, Topical↗
PubMed1987

Treatment of psoriasis with etretin: a preliminary report.

Eight patients with psoriasis (seven with plaque-type and one with palmoplantar pustular psoriasis) were treated with the synthetic retinoid etretin, the active metabolite of etretinate. An initial 8-week double-blind phase of the study with dosages of 0, 25, or 50 mg/day was followed by an open phase in which variable dosages of 25, 50, or 75 mg/day were used to achieve an optimal clinical response. All patients completed a minimum of 6 months of therapy. A good or an excellent response (at least 50% clearing) after 8 months of treatment was noted in six of the eight patients. One patient had a poor response (less than 50% clearing), and one patient continued to have worsening of psoriatic involvement during treatment. The best response occurred in those patients with the most extensive initial plaque involvement or palmoplantar pustulosis. The clinical side effects were similar to those reported with use of etretinate and seemed to be related to the dose. The laboratory abnormalities--primarily mild intermittent elevations of liver enzymes and elevations in serum lipids--were similar to those described in previous reports about etretinate. Both etretin and etretinate are potent teratogens. Because of its shorter half-life, etretin will likely be preferred, especially in female patients of childbearing potential.

Acitretin↗
PubMed1987

Nasal septal carcinoma masquerading as acne rosacea.

A 57-year-old woman developed diffuse erythema of the nose that had been diagnosed as acne rosacea for 18 months. Biopsy showed infiltration deep in the nasal skin of a squamous cell carcinoma that extended from the nasal septum. Nasal septal tumors are rare; the most common is squamous cell carcinoma.

Carcinoma, Squamous Cell↗
PubMed1987

Polymorphonuclear leukocyte chemotaxis in generalized pustular psoriasis.

We studied polymorphonuclear leukocyte (PMNL) chemotaxis in 3 patients with generalized pustular psoriasis using 3 chemoattractants: Zymosan-activated serum from both the patients and normal individuals, and n-formyl-methionyl-leucyl-phenylalanine (FMLP). These attractants were assayed against both patient-derived and normal PMNLs. All patients had quiescent skin disease at the time of assay. We found that all patients had decreased PMNL chemotaxis to autologous Zymosan-activated serum, but not to FMLP. PMNLs from normal individuals responded normally to serum from the patients. We conclude that patients with generalized pustular psoriasis, when free of symptoms, may have an isolated PMNL chemotactic defect that is restricted to serum-derived attractants.

Adult↗
PubMed1987

Dermatologic disorders in patients with thymoma.

We describe the cutaneous disorders in 172 patients with thymoma. Myasthenia gravis was the most common additional disease. Cutaneous disorders were found in 34 patients; 19 had skin disorders at the time of diagnosis of thymoma and 15 developed skin disorders after diagnosis of thymoma. Cutaneous fungal diseases were found in fewer than 10% of the patients (tinea pedis excluded). One patient with chronic mucocutaneous candidiasis was included in this group. Two patients with lichen planus, 2 with pemphigus, 2 with myositis, and 1 with lupus-like disease were also included in this study. Patients with skin disorders were no different than patients without these disorders with regard to thymoma histology, age at diagnosis, sex, or presence of myasthenia gravis. There were no associations between these variables and the patients who developed these skin disorders after diagnosis of thymoma. Thymectomy did not alter the clinical course with respect to cutaneous diseases.

Adult↗
PubMed1987

Quantitative and morphologic changes of Langerhans' cells after ultraviolet A irradiation of human epidermis.

Three different surface markers (OKT6, HLA-DR, and adenosinetriphosphatase) were compared to identify Langerhans' cells, and the changes in number and morphology of these cells were studied at different intervals after irradiation of human skin by a 2.5-fold minimal erythema dose of ultraviolet A. Morphologic alteration and decreased surface-marker reactivity became evident on day 2 and were most pronounced on day 3 or day 4 (injury phase). The recovery phase started between day 4 and 1 week and was complete by 3 weeks. HLA-DR+/OKT6- (DR+T6-) cells were present at all time intervals. The ratio of these cells to the sum of DR+T6- and DR+T6+ cells was 0.3% before irradiation, reached a peak of 65.4% at day 4, and decreased to 0.6% by 3 weeks.

Adenosine Triphosphatases↗
PubMed1987

Trichotillomania.

Trichotillomania is more common than is generally appreciated, especially in children. At the Mayo Clinic, we saw 145 patients in a ten-year period (1968-1977), and we have observed approximately 15 to 20 such patients per year since that time. Trichotillomania can be a mild or major cosmetic impairment and can be a minor neurotic trait or a sign of serious psychiatric disorder. Although the clinical presentation is characteristic, it can be confused with many different types of alopecia but particularly alopecia areata. Punch biopsy of the affected scalp may be very useful in confirming the clinical diagnosis of trichotillomania, particularly if catagen hairs, melanin casts, and signs of follicular tear or avulsion are present. Trichophagy and trichobezoars are rare associated disorders that should be sought. A common sense approach with a kind and honest explanation to the patient or the parents may be sufficient to terminate the hair-pulling. It is the responsibility of the physician to determine whether psychiatric consultation is desirable in the assessment and treatment of associated psychiatric disorders. Patients in whom the trichotillomania is resistant to simple reinforcement and explanation should have psychiatric consultation. Intensive psychotherapy may be worthwhile in selected patients.

Adolescent↗
PubMed1985

Cutaneous extraskeletal Ewing's sarcoma.

Extraskeletal Ewing's sarcoma has been recognized as being histologically indistinguishable from Ewing's sarcoma of bone. Although the occurrence of extraosseous Ewing's sarcoma in deep soft tissues has been well described, such cutaneous lesions are rare. We report the clinical history, histopathologic findings, and results of ultrastructural study of this rare variant occurring in a 12-year-old girl. She presented with a dermal and subcutaneous tumor of the left third toe, without osseous involvement. The lesion was composed of masses of round-oval cells, with perilobular fibrosis, focal hemorrhage, ill-defined pale cytoplasm containing glycogen, and absent pericellular reticulin. Ultrastructural study showed a monotonous cell population, with focal thickening of apposing membranes, high nuclear/cytoplasmic ratio, copious intracytoplasmic glycogen, and paucity of organelles except mitochondria. We present this case along with discussion of the literature and problems concerning differential diagnosis of small cell malignancies in the skin.

Bone Neoplasms↗
PubMed1985

Clinical and histologic findings in Degos' syndrome (malignant atrophic papulosis).

Records of nine patients (aged ten to sixty-four years) seen at the Mayo Clinic with the typical cutaneous lesions of Degos' syndrome were reviewed. The three patients who died all had central nervous system involvement. The six patients now alive have been evaluated for two to fourteen years and have had their disease for four to fourteen years. Histopathologic examination was performed in all nine cases (total of 27 skin biopsy specimens). Wedge-shaped infarction in the dermis and subcutaneous tissue was observed only in one biopsy specimen from each of three patients, and the infarction occurred in older, well-formed skin lesions. Thrombosis of the arterioles was found in two patients. Hyperkeratosis and dermal acid mucopolysaccharide deposits were common. The most consistent histopathologic finding was lymphocytic infiltrate around and in the walls of venules and arterioles. Various degrees of lymphocyte-mediated necrotizing vasculitis were present in all patients.

Adolescent↗
PubMed1985

Netherton's syndrome. Report of a case and review of the literature.

A patient with Netherton's syndrome who was followed since birth had previously been diagnosed as having Leiner's disease and acrodermatitis enteropathica; the disorder was recognized clinically when the patient was 20 years of age. Therapy with 13-cis-retinoic acid significantly aggravated the ichthyosis and induced increased fragility of the skin. This patient also had an intermittent aminoaciduria with clinical investigations that showed normal renal function. Furthermore, the aminoaciduria resolved spontaneously after the discontinuance of topically applied corticosteroids. The normal results of clinical studies and a review of the literature suggest that the aminoaciduria may have been artifactual because excess absorption of topically applied corticosteroids directly affects the renal tubules and impairs renal reabsorption or enhances the free excretion of amino acids. We review forty-two other cases of Netherton's syndrome in the literature and reaffirm the significance of associated ichthyosis, atopy, trichorrhexis invaginata, and other findings in this unique syndrome.

Adult↗