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N Saxe

Publications and source records attributed to N Saxe.

At least 19 recordsLinked to original sources

Validation of the U.K. Working Party diagnostic criteria for atopic eczema in a Xhosa-speaking African population.

BACKGROUND: Reliable diagnostic criteria for eczema are important for epidemiological comparisons. Although the U.K. diagnostic criteria for atopic eczema have performed well in an English language setting, limited data are available from other countries where cultural and linguistic factors may affect their validity. OBJECTIVES: We sought to determine the validity of the U.K. criteria for eczema in relation to clinical assessment by a dermatologist in a Xhosa-speaking South African population. METHODS: A cross-sectional survey of 3067 children aged 3-11 years was conducted in rural, peri-urban and urban settings in South Africa. The prevalence of atopic eczema was determined using the U.K. diagnostic criteria and a clinical assessment by a dermatologist. Questions were translated into the local language (Xhosa). Trained researchers administered the questions to the children's parents or carers. The validity of the U.K. criteria was then determined by calculating the sensitivity, specificity, positive and negative predictive values, and Youden's Index in relation to the dermatologist's examination. RESULTS: The point prevalence of atopic eczema according to a dermatologist was 1.0% [95% confidence interval (CI) 0.6-1.4], while the prevalence of visible flexural eczema according to the U.K. protocol was 1.8% (95% CI 1.3-2.2). The sensitivity and specificity of the U.K. criteria in this setting was 43.7% (95% CI 26.3-62.3) and 97.9% (97.3-98.4), respectively. The positive and negative predictive values of the U.K. criteria were 18.4% (95% CI 10.4-28.9) and 99.4% (95% CI 99.0-99.6), respectively. The presence of visible flexural eczema according to the U.K. photographic protocol was the best predictor of atopic eczema, with a sensitivity and specificity of 81.2% (95% CI 63.5-92.7) and 99.0% (95% CI 98.6-99.3), respectively, and a positive and negative predictive value of 48.1% (95% CI 34.3-62.1) and 99.8% (95% CI 99.5-99.9), respectively. CONCLUSIONS: The validity of the full question-based version of the U.K. diagnostic criteria for atopic eczema in this South African setting is low, which may be due to a combination of translational and cultural issues. However, the one physical sign of visible flexural eczema performed well, suggesting that it alone might be a useful tool for future international comparative prevalence studies.

Child↗

Poikiloderma, tendon contracture and pulmonary fibrosis: a new autosomal dominant syndrome?

Members of two generations of a South African family have a unique syndrome comprising poikiloderma, tendon contractures and progressive pulmonary fibrosis. The condition is clinically important as the skin changes, which involve the face, have considerable cosmetic impact, while lung involvement is potentially lethal in adulthood. Skin manifestations which facilitate diagnosis include facial telangiectasia, mottled hypo- and hyperpigmentation, papules and epidermal atrophy. The scalp, facial and body hair are fine and scanty. The tendon contractures lead to progressive digital flexion deformities and abnormalities of the ankles and feet, with disturbance of gait. Pulmonary involvement manifests as progressive dyspnoea. Pedigree data are compatible with an autosomal dominant mode of transmission. Poikiloderma of Weary is characterized by linear sclerotic and fibrous bands and not tendon contractures and is not associated with potentially lethal pulmonary fibrosis. Rather than name this disorder a variant of Weary syndrome, it might be prudent to use as an umbrella title one composed by Weary himself: 'hereditary sclerosing poikiloderma' (HSP), under which variants such as HSP Weary type, HSP with cardiac involvement (aortic stenosis described as inconsistently associated with Weary syndrome) and HSP with tendon/pulmonary involvement (current family) may be classified. The manifestations in this family differ from other poikilodermata and, to the best of our knowledge, have not been previously documented.

Adult↗

Malignant melanoma in Cape Town, South Africa.

There is a world-wide increase in the incidence of cutaneous malignant melanoma among white people. Absence of accurate population-based data on the incidence of melanoma in South Africa prompted a study to determine the incidence, anatomical sites and pathological details of melanoma in Cape Town. In a prospective study from 1 January 1990 to 31 December 1995, all the histopathology reports of melanoma presenting in a geographically defined area of Cape Town, were actively retrieved from every pathologist practising in this area. The data evaluated included information on age, sex, ethnic group and location of residence. Details of melanoma comprised body site, Clark level of invasion, Breslow thickness in millimetres and histogenetic type. The histology slides were reviewed by a panel in those cases where the recorded information was ambiguous or incomplete. A final number of 595 reports of primary invasive cutaneous melanomas in white people was analysed. Of these 50.3% were men and 49.7% women. The overall age-standardized incidence rate was 24.4 per 100,000 per annum (27.5 for men and 22.2 for women). There was no change in the incidence rate over the study period. Most melanomas in both sexes (74% of women and 71% of men) were < 1.5 mm Breslow thickness. Results of this study indicate a high incidence rate of melanoma in white South Africans, comparable with that in Australia, which demands urgent preventive health measures.

Adolescent↗

Idiopathic phalangeal osteolysis.

BACKGROUND: The acro-osteolytic diseases are a heterogeneous group of bone disorders characterized by bone resorption. Acquired and genetic forms have been well described. As hand abnormalities are frequently encountered in dermatologic practice, bone resorptive states, with particular reference to the idiopathic osteolytic disorders, are discussed. OBSERVATIONS: Asymptomatic terminal phalangeal osteolysis has been investigated in two unrelated young adults with no other bony or systemic abnormalities. The nails of the attenuated bulbous fingers were dystrophic, and pigmentary changes were present in the affected areas. The skin was otherwise normal, and there was minimal soft-tissue loss. Roentgenograms of the hands and feet showed varying stages of phalangeal osteolysis. CONCLUSIONS: Both cases represent primary idiopathic acro-osteolysis, as the bony changes are limited to the distal phalanges of the hands and feet. There were no features in the history or clinical evaluation to support a diagnosis of secondary acro-osteolysis.

Adult↗

Dermal and intravascular fasciitis. Unusual variants of nodular fasciitis.

Three cases with characteristic features of nodular (pseudosarcomatous) fasciitis arising in the dermis are described. This is only the second report documenting a dermal location for this condition. One of the three cases also showed features of intravascular fasciitis. A fourth case of intravascular fasciitis arising in periocular tissue is included. The differential diagnosis of dermal fasciitis includes benign fibrous histiocytoma, pyogenic granuloma, peripheral nerve tumors, cutaneous smooth muscle tumours, spindle cell carcinoma, spindle cell melanoma, dermatofibrosarcoma protuberans and atypical fibroxanthoma.

Adolescent↗

Bullous secondary syphilis.

An earlier standard syphilology textbook states that 'If vesicles are an essential part of an eruption in an adult, the lesions are not due to secondary syphilis'. However, vesicular and bullous eruptions do occur in congenital syphilis and rare reports of both vesicular and pustular eruptions in adults with secondary syphilis have been published. We describe a patient with a bullous-pemphigoid-like eruption and a positive VDRL in whom treatment with a course of procaine penicillin resulted in rapid permanent resolution of the eruption.

Adult↗

Leg ulcers in hereditary spherocytosis.

Refractory leg ulcers in two patients healed following splenectomy. Neither patient had the ethnic background or family history of haematological abnormalities to suggest a haemoglobinopathy.

Adult↗

Benign and malignant cellular blue nevus. A clinicopathological study of 30 cases.

The clinical and pathological features of 29 cellular blue nevi (CBN) and one malignant cellular blue nevus from our hospital files were reviewed. Although the sacrococcygeal region/buttock was the commonest single site, the majority of CBN occurred on the limb peripheries. Two-thirds of patients were under the age of 40 years. Follow-up of a mean of 7 years did not reveal any evidence of malignancy. Four histological subtypes were recognized: mixed biphasic, alveolar, fascicular or neuronevoid, and atypical varieties. One case developed a benign nodal metastasis. In one case, malignancy arose within a CBN. The importance of recognizing the variety of patterns, the benign behavior of the atypical variety, and the criteria for malignancy are herein discussed.

Adolescent↗

Cutaneous manifestations of the Proteus syndrome.

The Proteus syndrome is a rare disorder in which the major manifestations are skeletal overgrowth, digital hypertrophy, exostoses of the skull, and hamartomatous tumors. Numerous skin lesions also occur. We treated six individuals, all of whom had the features unique to this syndrome of marked hypertrophy of the skin of the soles. The palms were similarly involved in two patients. Light microscopy of biopsy material from thickened areas of the soles showed elongation of the cytoplasm of the basal cells. Large epidermal nevi were present in three persons, as were linear macular lesions with areas of depigmentation and hyperpigmentation.

Abnormalities, Multiple↗

Macromelanosomes in the early diagnosis of neurofibromatosis.

Skin biopsies of café-au-lait macules from 34 patients with a clinical diagnosis of classical neurofibromatosis were examined histologically and ultrastructurally to determine the presence or absence of macromelanosomes in the epidermal melanocytes and keratinocytes. Sixteen of the 34 patients had macromelanosomes. The presence of macromelanosomes varied with age and ethnic background; they were detected in nine of 12 Whites, six of 10 persons of mixed ancestry, and one of two Blacks. In these populations skin biopsy is useful in early diagnosis of neurofibromatosis. However, none of 10 persons of Indian stock had macromelanosomes. Their total absence in this group may be indicative of genetic heterogeneity.

Child↗