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Biomedical subjects

H Trau

Publications and source records attributed to H Trau.

At least 19 recordsLinked to original sources

Necrobiosis lipoidica and diabetic control revisited.

Necrobiosis lipoidica diabeticorum is a rare skin disorder, usually considered a marker for diabetes mellitus. More than half of the patients with necrobiosis lipoidica diabeticorum have diabetes mellitus, but less than one per cent of diabetes mellitus patients have necrobiosis lipoidica diabeticorum. In the diabetes and dermatology literature, we find the position that there is no effect of glucose control on either the appearance of necrobiosis lipoidica diabeticorum or the clinical course of the lesion. We base our challenge to this position on a critical review of the original data. And conclude on the contrary, that necrobiosis lipoidica diabeticorum is usually associated with poor glucose control and that tighter glucose control, as currently practised, might improve or prevent the disorder.

Blood Glucose

Necrobiotic xanthogranuloma with paraproteinemia.

Necrobiotic xanthogranuloma (NXG) is a rare skin disease mimicking necrobiosis lipoidica diabeticorum clinically and histologically. However, its association with paraproteinemia, and the evolution of hematologic malignancies in some cases, established NXG as a separate clinicopathologic entity. A typical case with indolent course is described.

Aged

Familial cutaneous malignant melanoma and tumors of the nervous system. A hereditary cancer syndrome.

BACKGROUND: Excessive risk of cutaneous melanoma as a second cancer has been associated with benign or malignant tumors of the nervous system. Cutaneous melanoma and nervous system tumors may independently aggregate in families. There are, however, no previous reports of increased likelihood of tumors of the nervous system in families of patients with cutaneous melanoma or--of cutaneous melanoma in families with tumors of the nervous system. METHODS: The occurrence of nervous system tumors as second cancers was examined in a series of 904 patients with cutaneous melanoma and/or their family members. RESULTS: Fifteen families with 17 members with cutaneous melanoma from this series had one or more additional relatives with tumors of the nervous system, including astrocytoma, medulloblastoma, glioblastoma multiforme, ependymoma, glioma, meningioma, and acoustic neurilemmoma. Another subgroup of 10 patients with cutaneous melanoma had either a meningioma (n = 9) or acoustic neurilemmoma (n = 1) as a second tumor. The pattern of atypical melanocytic nevi occurring in the majority (20/25) of cutaneous melanoma patients in our series and in additional first degree relatives of 9 of 11 of the affected families, has been previously associated with the Familial Atypical Multiple Mole-Melanoma syndrome. CONCLUSIONS: This unusual familial and personal occurrence of tumors, showing differentiation toward tissues of the neural crest, neuroepithelium, and/or mesenchymal derivation, supports a putative association with a hereditary cancer susceptibility trait.

Adolescent

The pattern of HMB-45 antibody staining in compound Spitz nevi.

We studied the staining pattern of HMB-45 antibody in 29 compound Spitz nevi (SNs) of the epithelioid cell variety, 17 of which showed extension of nevus cells into the reticular dermis (i.e., "deep"); 20 ordinary compound nevi (CNs), all with a deep dermal component; and 22 primary cutaneous invasive malignant melanomas (MMs) (excluding the desmoplastic and spindle cell types), 12 of which extended into Clark level IV or V. Of the 29 SNs, eight (28%) stained negatively; five (17%), including two deep SNs, stained in the epidermal component only; and 16 (55%), including 10 deep SNs, stained in both the epidermal and dermal components. Of the latter 10 deep SNs, eight stained in the upper dermis only, and in the remaining two lesions, a smaller number of positively stained nevus cells were detectable in the lower dermis as well; these two SNs were not atypical histologically. Of the 20 CNs, four (20%) stained negatively, two (10%) stained in the epidermal component only, and 14 (70%) stained in the epidermal component and the upper dermis only. Of the 22 MMs, one stained negatively, and 21 (95%) stained positively in both the epidermal and dermal components. The pattern was variable in frequency of both staining and distribution, but showed no stratification. We conclude that the majority of our positively stained deep compound SNs showed a stratified pattern of HMB-45 staining, similar to ordinary CNs and different from MMs, and that this pattern might be used as an adjunct in the histopathologic differential diagnosis of compound SN and MM, in the proper clinicopathological context.

Adolescent

Immunohistochemical study of p53 protein expression in Spitz nevus as compared with other melanocytic lesions.

The accumulation of p53 protein was studied immunohistochemically on paraffin-embedded sections of 26 Spitz nevi (SNs), 26 primary invasive cutaneous malignant melanomas (MMs), 20 metastases of MM, and 17 ordinary compound nevi (CNs), using monoclonal antibody BP53-12. Positive reactivity was detected in some of the tumor cells in seven (35%) metastatic MMs, all exhibiting strong nuclear staining; eight (31%) primary MMs, of which seven showed strong nuclear staining; two (7%) SNs, of which only one showed strong nuclear staining; and none of the CNs. The frequencies of the positively stained lesions in general, and the strongly positively stained lesions in particular, in the MM and metastatic MM groups were each statistically significantly higher than the respective frequencies in the SN and CN groups. We believe that the immunohistochemical detection of p53 protein with the use of monoclonal antibodies such as BP53-12 on paraffin sections, especially when strong nuclear reactivity is demonstrated, may prove to be an adjunctive tool in the histopathologic differentiation of MM from SN.

Adolescent

Generalized pustular eruption associated with converting enzyme inhibitor therapy.

A 67-year-old man presented with a high fever and a generalized rash. His extended hospital stay was characterized by fever with repeated staphylococcal bacteremia and the appearance of axillary lymphadenopathy and splenomegaly. Skin lesions became hyperpigmented, dry, and atrophic with areas of exfoliation and uclers. Examination of skin and lymph node biopsy specimens showed findings consistent with mycosis fungoides. The patient unexpectedly recovered on discontinuation of captopril. A positive macrophage inhibiting factor response for both captopril and enalapril indicated that the non-sulfhydryl moiety was the antigenic stimulant for the lesion resembling mycosis fungoides.

Aged

[Porokeratosis of the skin in an immunosuppressed patient].

Porokeratosis is a chronic, progressive disease of hereditary origin, characterized by the formation of annular, flat lesions with elevated scaly borders. It appears as a few clinical variants that differ mainly in the mode of spread of the lesions and age of onset. In recent years immunosuppression has been found to initiate appearance of the disease or cause its exacerbation. We present a 39-year-old man who developed porokeratosis a year after commencing immunosuppressive treatment following a heart transplantation. There is a relationship between the immune system, malignant transformation and porokeratosis.

Adult

Huge cutaneous plasmacytomas complicating multiple myeloma.

The case of a 46-year-old woman with multiple myeloma and extramedullary dissemination to the urinary bladder and skin is described. Cutaneous plasmacytomas were scattered as firm, violaceous-red, lesions of variable diameter (2-13 cm) which were extremely tender. Despite combined chemotherapy and half-body irradiation, the lesions progressed until the patient died 8 months following diagnosis.

Arm

[Fabry disease: systemic deposition of a glycolipid].

A 20-year-old man was admitted with telangiectatic skin lesions over the lower abdomen, buttocks and genitals and also hypohidrosis and heat intolerance. Fabry disease was diagnosed on the basis of biochemical and histopathological analyses. The concentration of urinary trihexosyl-ceramide was increased but the activity of galactosidase in serum, urine and lymphocytes was marginal. Typical lipid inclusions, showing a concentrically arranged, lamellar osmiophilic structure were identified by electron microscopy in the cytoplasm of endothelial cells from affected skin.

Adult

[Cutaneous larva migrans].

Larva migrans is a rare cutaneous parasitic infestation, whose incidence in Israel has increased over the past few years. This is mainly due to the growing number of Israelis traveling to the Far East and to Central and South America. We present 4 such cases. The treatment of choice for this parasite is thiabendazole, an imidazole with anthelminthic properties.

Adult

[Trimethylaminuria: fish-odor syndrome].

Fish-odor syndrome is due to a rare metabolic defect in which there is massive excretion of the volatile tertiary amine, trimethylamine (TMA) in the urine. The typical fish-like odor of TMA also appears on the breath or in the sweat of affected individuals. In normal man TMA is oxidized to its odorless N-oxide derivative. TMA is of dietary origin, formed by intestinal bacterial degradation of the choline in egg yolk, liver, soybeans, etc., or by reduction of TMA-oxide present in high concentrations in marine fish. 2 sisters with the tentative diagnosis of trimethylaminuria are presented.

Adult