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Biomedical subjects

T M Starink

Publications and source records attributed to T M Starink.

At least 55 records · Page 3Linked to original sources

Multiple angiomatous proliferations of ileal stoma following Campylobacter enteritis. Effect of laser photocoagulation.

Multiple polypoid sessile tumors arose at the tip of the ileal stoma of a 37-year-old man, who previously underwent proctocolectomy for ulcerative colitis. The lesions developed shortly after a well-established gastrointestinal infection with Campylobacter jejuni with mucosal ulceration. The histological and ultrastructural examination showed angiomatous proliferations, reminiscent of pyogenic granuloma. The destruction of the tumors by laser photocoagulation was followed by superficial ulcerations of the mucosa, which healed without scarring. Small recurrences, however, could not be prevented.

Adult↗

Generalized trichoepitheliomas with alopecia and myasthenia gravis: clinicopathologic and immunohistochemical study and comparison with classic and desmoplastic trichoepithelioma.

We report on a patient with a syndrome characterized by smooth facial papules and nodules; alopecia of the eyebrows, eyelashes, and most body hair; mild alopecia of scalp hair; possibly hypohidrosis; and myasthenia gravis. The clinical, histologic, and immunohistochemical findings are compared with classic and desmoplastic trichoepitheliomas. All fourteen biopsy specimens of the patient showed a lacelike network of basaloid cells with follicular differentiation and a prominent stroma with focal alkaline phosphatase activity, as in fourteen comparison specimens of classic trichoepithelioma. Studies with antikeratin antibodies of various specificities also revealed similar patterns. The tumor cells showed a keratin phenotype characteristic of cells of the infrainfundibular outer root sheath, that is, positive staining with basal keratinocyte markers, negative staining with suprabasal keratinocyte markers, and patchy staining with LP2K (against keratin 19). beta 2-Microglobulin expression was partially or totally absent. It is concluded that the lesions in our patient actually are trichoepitheliomas. The condition may represent a new syndrome that either is closely related to the generalized hair follicle hamartoma (basaloid follicular hamartoma) or is a variant. The finding of generalized trichoepitheliomas with clinical and microscopic alopecia should alert one to the possibility of myasthenia gravis.

Adult↗

The Cowden syndrome: a clinical and genetic study in 21 patients.

An analysis of the findings in 21 patients with the Cowden syndrome or the multiple hamartoma syndrome is presented. The Cowden syndrome is a cancer-associated genodermatosis with characteristic mucocutaneous findings and a wide array of associated abnormalities including a high incidence of breast cancer in female patients. Genetic studies confirmed autosomal dominant inheritance with a high penetrance in both sexes and moderate interfamilial and intrafamilial differences in the expressivity of a number of symptoms. Familial occurrence was present in 4 of the 7 families. There was a strong predominance of female patients (6:1), which may be fortuitous. Mucocutaneous changes were the most constant (100% incidence) and characteristic findings; they almost invariably became manifest in the second decade. Four of our 18 female patients (22%) were treated for breast cancer, a lower incidence than reported previously. No increased incidence of other types of malignancies was found. Craniomegaly (high head circumference) was found to be the most common extracutaneous manifestation (80% incidence); craniomegaly appears to be an important early marker. We also found high incidences of gastrointestinal polyps (approximately 60%) and cutaneous fibromas (76%), while the incidence of thyroid abnormalities, thus far regarded as the most common extracutaneous finding, was similar to that reported previously (62%). G-banded karyotype and preliminary DNA-repair studies revealed no clear abnormalities. No linkage with the loci of HLA, and immunoglobulin haplotypes was found.

Abnormalities, Multiple↗

The cutaneous pathology of Cowden's disease: new findings.

Histological, histochemical and immunohistochemical findings were studied in 40 cutaneous biopsies from 7 patients with Cowden's disease. Most facial biopsies showed a spectrum of trichilemmomas and related follicular malformations, including cylindrical trichilemmomas, lobulate trichilemmomas (14 lesions), and a keratinizing type sharing features with inverted follicular keratosis. One facial growth showed trichilemmomal changes without apparent follicular origin. Studies for common papilloma virus structural antigens were negative, apart from a typical common wart in one patient. Immunohistochemical studies in 6 facial trichilemmomas and acral keratoses, using a panel of anti-keratin antibodies, disclosed only abnormal differentiation with lack of large keratins in the lobulate trichilemmomas. Nine biopsies revealed a distinctive type of fibroma characterized by an organized pattern of interwoven fascicles of collagen bundles with a laminated or tortuous appearance, embedded in abundant mucin. A number of fibromas showed striking hyalinization; these may represent a second microscopic hallmark of Cowden's disease in addition to facial trichilemmomas.

Antigens, Viral↗

Familial multiple trichodiscomas. A clinicopathologic study.

Three patients had hereditary multiple trichodiscomas of early onset and dominant inheritance. This report is an analysis of the histologic, histochemical, immunohistochemical, and electron microscopic findings in 27 trichodiscomas, the largest published series so far, to our knowledge. The tumors of perifollicular connective tissue are briefly reviewed and a classification is proposed. Hereditary multiple trichodiscomas are regarded as a new entity that should be distinguished from the syndrome recently described by Birt and associates that is characterized by autosomal dominant inherited fibrofolliculomas as the hallmark, associated with trichodiscomas and acrochordons.

Adult↗

Cowden's disease: analysis of fourteen new cases.

Fourteen new patients with Cowden's disease from the Netherlands were analyzed and compared with the sixty-nine previously reported patients. The full clinical spectrum of the disease can now be fairly well delineated. Mucocutaneous lesions, especially facial trichilemmomas and other follicular malformations, acral keratoses, and oral papillomas, remain the most constant findings. The incidence of each of these mucocutaneous findings was slightly higher than reported previously, probably reflecting increasing experience with this condition. Also our patients tended to have more extensive acral keratoses and perhaps less extensive facial lesions. In one family there was an extremely high incidence of lipomas. Breast cancer was seen in three of our twelve female patients, reflecting the trend toward a lower incidence of breast cancer in the recent literature. Gastrointestinal polyps in one family were much more common than previously reported, occurring in five of seven patients. Thyroid disease was found in about two thirds of the patients and abnormalities of the female reproductive system in slightly more than half of the patients, both incidences being approximately the same as in the literature. With increased awareness of the condition it is likely that many more cases will be recognized in the future.

Adult↗

The cutaneous pathology of facial lesions in Cowden's disease.

A study of 11 facial lesions from 7 patients with Cowden's disease indicated that most belong to a spectrum of related follicular malformations. The most distinctive lesions included multiple facial trichilemmomas, which were frequently cylindrical, resembling a blown-up hair follicle, but could be lobular. Four of the 5 trichilemmomal lesions were from the centrofacial area. Three periauricular lesions showed the pattern of tumor of follicular infundibulum without trichilemmonal features. Two lesions were epidermal without a follicular component and resembled digitate warts. No evidence of virus infection was found using an antiserum to papilloma virus.

Adult↗

The cutaneous pathology of extrafacial lesions in Cowden's disease.

Twenty extrafacial lesions from 6 patients with Cowden's disease were studied. Most were hyperkeratotic papillomas that resembled verruca vulgaris, acrokeratosis verruciformis, or hyperkeratotic acanthomas. Small hyperkeratotic papillomas frequently showed abnormalities of the follicular infundibulum. Occasionally, follicular abnormalities were seen without concomitant epidermal hyperplasia. It is concluded that some of the extrafacial lesions may originate from the follicular infundibulum. No evidence of a virus infection was found using an antiserum to papilloma virus.

Adult↗

Zinc deficiency syndrome versus glucagonoma syndrome.

In this paper two patients with uncommon syndromes, viz. acrodermatitis enteropathica-like eruption due to acute zinc deficiency, when on long-term intravenous hyperalimentation for Crohn's disease, and necrolytic migratory erythema as a consequence of a malignant glucagon secreting alpha-cell tumour of the pancreas (glucagonoma syndrome) are reported. Attention is paid to the many common features of the skin lesions in both syndromes. This is discussed in detail. Both patients passed through a catabolic stage. Laboratory investigations, however, failed to demonstrate a common biochemical mechanism which might be responsible for the skin lesions. Administration of zinc in the first patient and surgical treatment of the second patient results in rapid clearing of the skin lesions and other symptoms.

Acute Disease↗