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Biomedical subjects

R M Strauss

Publications and source records attributed to R M Strauss.

At least 19 recordsLinked to original sources

A child with laryngo-onychocutaneous syndrome partially responsive to treatment with thalidomide.

Laryngo-onychocutaneous syndrome (LOCS) is a condition characterized by erosive or ulcerative skin lesions associated with excessive granulation tissue, at sites of trauma such as the digits, elbows and knees. Similar lesions can occur within the conjunctival mucosa, leading to corneal scarring and blindness. The main complications, however, occur in the respiratory tract, where a similar process of erosions and subsequent formation of granulation tissue causes airway obstruction which may lead to premature death. LOCS is now believed to be a nonblistering variant of junctional epidermolysis bullosa and to date there are no efficacious treatments available. We report a 16-year-old girl with LOCS who failed to respond to methylprednisolone and cyclophosphamide, but had a partial response to oral thalidomide with marked decrease in granulation tissue and tracheal secretions. Interruption of treatment resulted in prompt resurgence of the granulation tissue which was again controlled by reintroduction of thalidomide. We propose that in the absence of effective therapies for LOCS, a trial of thalidomide in these patients should be considered.

Adolescent↗

Unilateral naevoid trichoepitheliomas on the face of a child.

Trichoepitheliomas are benign epidermal appendage tumours that present most commonly as solitary lesions, and less often as multiple, symmetrically distributed lesions on the face, scalp, neck and trunk. Only a few patients have been reported in whom trichoepitheliomas were found as unusually configured linear or plaque-like confluent lesions, all of which occurred in patients with skin types V or VI. We describe a white girl with a hemifacial plaque of confluent naevoid trichoepitheliomas, the first report of such lesions in a white patient.

Child↗

Skin abnormalities in the National Portrait Gallery.

Pathological changes in the human body have long been recognised in painting. To assess the frequency of potential physical abnormalities reflected in paintings, we reviewed portraits on display at one visit in the National Portrait Gallery in London. We identified a number of possible abnormalities, most of them dermatological, with the commonest finding being small facial papular lesions, most likely representing either common warts or dermal naevi. This exercise however, reflects a subjective interpretation of the different artists's perceptions of their models' physical appearances.

History, 16th Century↗

Mountaineer's heel.

Mountaineers are at risk of skin lesions caused by constant boot friction. This is the case of a 35 year old mountaineer who presented with large and deeply ulcerated lesions over the medial aspects of both heels after a two and a half day climb using crampons. A number of factors such as the length of the climb in cold and thin air and the impossibility of removal of the footwear to allow rapid rewarming may have contributed to a more acute presentation of these lesions.

Adult↗

A case of childhood dermatofibrosarcoma protuberans without detected cytogenetic abnormality.

Dermatofibrosarcoma protuberans (DFSP) is a rare, infiltrative skin tumour of intermediate malignancy, with a limited potential for metastasis but a high rate of recurrence; specific cytogenetic abnormalities are now known. Childhood DFSP has been considered a rarity in the past, but it is now recognized that many cases of childhood DFSP are diagnosed only in adulthood. Despite advances in the understanding of its pathogenesis as well as the development of valuable immunohistochemical and cytogenetic diagnostic techniques, there often remains a significant delay between the initial presentation and diagnosis of DFSP. We report a case of childhood DFSP in which the diagnosis was reached only after a nodular lesion developed in a plaque that was initially present. Causes for delay between initial presentation and diagnosis in childhood DFSP are discussed. Histology and immunostaining in our patient showed the typical features of DFSP, but the G-banded cytogenetic analysis of short-term tissue culture was negative. However, this technique offers only a detection rate between 50% and 80%. Clinicians should be aware of the limitations of newer diagnostic techniques. Increasing recognition amongst paediatricians and paediatric dermatologists that childhood DFSP is not as rare as once believed will probably lead to the use of newer diagnostic methods at an earlier stage, and so reduce the delay between the onset of symptoms and diagnosis.

Biopsy↗

Digital imaging in clinical dermatology across the UK in the year 2001.

Colour photography is an important tool in the daily practice of dermatologists. Digital photography promises quicker acquisition and handling of clinical images. Despite increasing availability and falling costs, dermatologists have been slow in adopting this new technique. To establish to what degree digital photography has found its way into routine practice in dermatology departments in the UK during the year 2001, we conducted a postal survey of all dermatology departments in the UK about usage of digital cameras. A total of 65.4% of the questionnaires were returned. The responses indicated that 71% of departments in teaching hospitals vs. 56% of departments in district general hospitals (DGH) had access to a digital camera. Reasons for not having a digital camera were satisfaction with conventional photography and lack of resources. In 48% of the departments of teaching hospitals or DGH with access to a digital camera, less than 10% of all photos were taken digitally. However, 24% of departments in DGH and 22% in teaching hospitals took more than 80% of their clinical photos digitally, indicating that in many departments digital photography is still in an early phase and only occasionally used, whilst in others a change from conventional photography towards digital photography as the mainly used technique has already taken place. Overall, the main drawbacks of digital photography were poorer resolution in comparison with conventional photography, problems with storage and filing images and time required for downloading and storing images.

Dermatology↗

Occupational contact dermatitis in nurses with hand eczema.

Occupationally related dermatitis is a common problem in nurses, who are exposed to a wide variety of allergenic and irritant substances. In a group of 44 nurses with hand dermatitis (40 female, 4 male), 18 were thought to have a predominantly allergic contact dermatitis, 15 an irritant dermatitis, 7 other form of eczema, 3 atopic dermatitis and one pompholyx. 10 of the 15 irritant cases were diagnosed as occupational. Of the 18 patients with allergic contact dermatitis, the allergens were thought to be occupationally relevant in 8 cases. In 6 of these 8 the dermatitis was due to natural rubber latex (3) or other rubber chemicals (3). 2 had additional evidence of immediate-type hypersensitivity to natural rubber latex (one was patch test allergic to latex, the other to thiuram mix). Natural rubber latex allergy, both delayed and immediate, is a significant problem, and nurses at risk should be tested for both types of hypersensitivity, as well as being patch tested to standard, rubber and medicaments series.

Adult↗