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N P Smith

Publications and source records attributed to N P Smith.

At least 19 recordsLinked to original sources
PubMed1992

Coexistent cutaneous T-cell lymphoma and B-cell chronic lymphocytic leukaemia.

Three patients with cutaneous T-cell lymphoma (CTCL) are reported who had concurrent B-cell chronic lymphocytic leukaemia (B-CLL). The separate lineage and clonal nature of the individual lymphoid malignancies were confirmed by gene-rearrangement analysis. The chronology of the illnesses did not support the hypothesis that CTCL predisposes to the development of B-cell proliferative disorders. There was no clear association with immunosuppressive therapy, and HTLV-1 studies were negative in all patients. Consequently, we speculate that a lymphoid stem cell defect, which may lead to the development of either aberrant B- or T-cell clones, may be responsible for such cases of dual lymphoid neoplasia.

Aged↗
PubMed1992

Erythema elevatum diutinum: a clinicopathological study.

Erythema elevatum diutinum is a syndrome of vasculitis in which lesions, typically over the extensor surfaces, showed a mixed inflammatory infiltrate on biopsy. We describe a series of 13 patients. The most common association in our series was with hypergammaglobulinemia; both mono and polyclonal. Chronic infection, not streptococcal, was a less frequent finding although two of three patients had a positive reaction to the intradermal injection of streptococcal antigen. Dapsone remains the initial treatment of choice.

Adolescent↗
PubMed1992

The clinicopathological features of familial cylindromas and trichoepitheliomas (Brooke-Spiegler syndrome): a report of two families.

We have studied four patients from two families and obtained the clinical data from four other affected family members who demonstrated the coexistence of multiple cylindromas and trichoepitheliomas. An autosomal dominant pattern of inheritance with variable penetrance was exhibited in both families, with a female to male ratio of 3:1. The clinical and pathological features of this rare association are discussed.

Adult↗
PubMed1992

Hypopigmented mycosis fungoides.

We report the case of a 25-year-old Jamaican woman with hypopigmented mycosis fungoides. She first developed a hypopigmented patch on her arm at the age of 11 years. Further lesions developed on the trunk and limbs over a period of 10 years. The lesions were completely impalpable. Skin biopsy showed an infiltrate of atypical lymphocytes, some with cerebriform nuclei, suggesting a diagnosis of mycosis fungoides. The lesions cleared with PUVA therapy.

Adult↗
PubMed1992

Diagnosis of lichen planus pemphigoides in the absence of bullae on normal-appearing skin.

A case of lichen planus pemphigoides is described in which bullae and erosions affected the areas of lichen planus only. The diagnosis was confirmed by the finding of linear C3 and IgG deposition in the basement membrane zone (BMZ) of perilesional skin. Indirect immunofluorescence revealed circulating IgG anti-BMZ antibodies and a lamina lucida split was demonstrated by electron microscopy. This case challenges the clinical dogma that the occurrence of bullae on normal-appearing skin is essential for the diagnosis of lichen planus pemphigoides.

Humans↗
PubMed1992

Recalcitrant pyoderma gangrenosum--two cases successfully treated with cyclosporin A.

The successful use of cyclosporin A (CSA) in organ transplantation is now well established. In recent years its usefulness has extended to the treatment of cutaneous autoimmune disorders, including pyoderma gangrenosum (P.G.). We report two further cases of recalcitrant P.G., both associated with rheumatoid arthritis (R.A.) which responded to low dose CSA.

Aged↗
PubMed1992

Cellular 'neurothekeoma': an epithelioid variant of pilar leiomyoma? Morphological and immunohistochemical analysis of a series.

Cellular neurothekeoma is a recently recognized benign cutaneous neoplasm, which is currently regarded as being of nerve sheath origin and is thought to represent a variant of conventional neurothekeoma (dermal nerve sheath myxoma). Nine new cases presenting predominantly in adolescents or young adults are described. Morphologically they were characterized by short fascicles or small nests of palely eosinophilic epithelioid or spindle-shaped cells which ramified in an ill-defined manner between dermal collagen bundles. Myxoid matrix was absent or sparse. Scattered normal mitoses and multinucleate giant cells were often present. Immunohistochemically all nine cases were strongly NK1/C3 positive, seven were weakly NSE positive and three were smooth muscle actin positive. Staining for S-100 protein, PGP 9.5, epithelial membrane antigen and desmin was negative in all cases. In view of its distinctive architecture and immunophenotype, both of which are totally different from conventional neurothekeoma, it is proposed that cellular 'neurothekeoma' is a separate discrete entity which may represent an epithelioid variant of pilar leiomyoma.

Adolescent↗
PubMed1992

Unusual variants of pemphigoid: from pruritus to pemphigoid nodularis.

We report three patients with pemphigoid nodularis. Patients were females aged 76, 71 and 50 years, and all had features of bullous pemphigoid together with prurigo-like lesions at some stage of their illness. In two cases, nodular lesions preceded the onset of blistering by some months. Blisters arose on normal skin and in one patients also at sites of prurigo lesions. Routine histology of bullous lesions revealed the presence of subepidermal blisters. Electron microscopy performed in two cases confirmed the level of split to be through the lamina lucida. Direct immunofluorescence in all cases was positive, with linear basement membrane zone deposition of IgG and C3. Circulating IgG anti-basement membrane antibody was also detected in all patients, and in two, immunoblotting revealed a single antigen of 220 kD.

Aged↗
PubMed1991

Analysis of beta, gamma, and delta T-cell receptor genes in mycosis fungoides and Sezary syndrome.

The authors have analyzed the configuration of immunoglobulin (Ig) and beta, gamma and delta T-cell receptor (TCR) genes in DNA extracted from skin, lymph nodes, and peripheral blood mononuclear cells obtained from 41 patients with mycosis fungoides (MF), 14 patients with Sezary syndrome, and 13 patients with benign inflammatory dermatoses. No discrete rearranged bands (DRB) were detected in patients with inflammatory dermatoses. In tissue DNA from 19 patients with MF DRB were detected with beta and gamma, but not delta TCR probes. Only one patient with MF had a rearrangement of gamma and delta with germ line beta TCR genes. In 13 patients multiple biopsies were analyzed and DRB, when present, were identical in different lesions from individual patients. In three patients analysis of DNA from dermatopathic lymph nodes did not reveal DRB. Analysis of peripheral blood DNA from 24 patients revealed a discrete rearrangement of the gamma TCR gene in four patients and both beta and gamma genes in four additional patients. In MF DRB were detected more frequently with advancing stage of disease in tissues (P less than 0.01) but not in peripheral blood (P equals 0.36). Of 14 patients with Sezary syndrome, eight had DRB in peripheral blood DNA with both beta and gamma probes and in three of these patients identical DRB were also detected in DNA from skin biopsy samples. In contrast, DRB were not detected in the peripheral blood of the other six patients. In both MF and Sezary syndrome there was no restricted usage of particular V gamma genes. These results indicate that in MF (1) T-cell clones can be detected in skin biopsy specimens from the majority of patients with early stage disease, (2) gamma delta T-cell clones are only rarely found, and (3) TCR gene analysis can detect T-cell clones in the peripheral blood with a greater degree of specificity than conventional light microscopic study. In Sezary syndrome these studies also suggest that a subset of patients have a polyclonal population of circulating atypical lymphoid cells. In addition these patients appear to have a better prognosis than those with monoclonal disease.

DNA, Neoplasm↗
PubMed1991

Malignant chondroid syringoma--a rare cause of secondary bone tumour.

Sweat-gland carcinomas are very rare, comprising only 0.01% of primary tumours of the skin. Malignant chondroid syringoma is the least common variety, of which less than twenty cases have been recorded. We report a case in which this tumour presented as an osteolytic secondary deposit.

Adenoma, Sweat Gland↗
PubMed1990

Subcutaneous xanthomatosis.

A patient with multiple subcutaneous xanthomata in whom there were only minor abnormalities of the fasting serum lipid profile is described. To our knowledge, this is only the second published case of subcutaneous xanthomatosis.

Aged↗
PubMed1990

Skin exudate levels of interleukin 6, interleukin 1 and other cytokines in mycosis fungoides.

The role of locally released cytokines in inducing lymphocyte activation and infiltration in the skin lesions of mycosis fungoides has been investigated. The levels of selected cytokines were measured in chamber fluid samples from lesional and control skin. Biologically active interleukin 6 was significantly elevated in lesional samples and a recombinant form of this cytokine was shown to induce lymphocyte migration in an in vitro assay. Biologically active interleukin 1 was detected in all control chamber fluid samples. Significantly reduced levels of this cytokine were present in lesional samples, which may be the result of the release of preformed material. Interleukin 2 and tumour necrosis factor activity, and gamma interferon and granulocyte macrophage colony-stimulating factor immunoreactivity, were not detectable in any of the samples. Interleukins 1 and 6 may play a role in the pathogenesis of the lesional lymphocyte infiltrates in mycosis fungoides.

Adult↗
PubMed1990

Multinucleate cell angiohistiocytoma: an acquired vascular anomaly to be distinguished from Kaposi's sarcoma.

Multinucleate cell angiohistocytoma is a newly described benign vascular condition that usually arises on the extremities of women over the age of 40 as discrete grouped violaceous erythematous papules, often mimicking Kaposi's sarcoma. Of 10 patients, nine were women aged between 37-66 (average 51.5) years at the onset of their condition. The legs, and in particular the calves and thighs, were the commonest sites to be involved. However, in three patients the papules were confined to the back of the hands, and the male patient had lesions across the front of the chest. Bilateral lesions occurred in four patients. Histologically, the salient features were proliferation of capillaries and small venules at the level of the subpapillary plexus and the mid dermis, in association with prominent connective tissue cells and larger angulated multinucleate cells. Detailed histochemical and immunocytochemical studies have not elucidated the histogenesis of the multinucleate cells that seem to be a characteristic feature of this condition.

Adult↗