Biomedical subjects
Publications and source records attributed to L C Fuller.
A 4-year-old girl presented with a linear, indurated area of dusky erythema and hyperpigmentation down the left leg, present since birth. Histology suggested syringomata. The clinical course and appearances suggest a novel entity for which we have coined the term 'linear syringomatous hamartoma'.
The aim of this study was to examine trends in the presentation of cutaneous malignant melanoma at King's College Hospital (KCH) over the last three decades (1970-2000). KCH was one of seven centres that participated in the 1987 Cancer Research Campaign (CRC) publicity campaign aimed at promoting earlier self-recognition of melanoma. Data included patient age at presentation, sex, tumour site, Breslow thickness and histological subtype. The late 1980s saw a threefold increase in the annual number of melanomas and an eightfold increase in thin melanomas compared to the 1970s. The increase occurred in both sexes and was particularly marked after the CRC campaign but numbers had already begun to increase prior to this. The increase has predominantly been thin (Breslow < 1.5 mm) tumours of the superficial spreading variety with a resultant fall in mean Breslow thickness. There has been a decline in the annual number of melanomas since the peak in 1992 which is not explained by increased proportion of in situ tumours. The CRC campaign may have contributed to the documented increase in thin tumours but this trend had begun prior to 1987 suggesting factors other than public awareness and earlier presentation are important. It is encouraging that the number of melanomas has declined over the last 5 years at KCH but it is yet to be seen whether this reflects a real decrease in the incidence of melanoma.
Germline mutations of the fumarate hydratase (FH, fumarase) gene are found in the recessive FH deficiency syndrome and in dominantly inherited susceptibility to multiple cutaneous and uterine leiomyomatosis (MCUL). We have previously reported a number of germline FH mutations from MCUL patients. In this study, we report additional FH mutations in MCUL and FH deficiency patients. Mutations can readily be found in about 75% of MCUL cases and most cases of FH deficiency. Some of the more common FH mutations are probably derived from founding individuals. Protein-truncating FH mutations are functionally null alleles. Disease-associated missense FH changes map to highly conserved residues, mostly in or around the enzyme's active site or activation site; we predict that these mutations severely compromise enzyme function. The mutation spectra in FH deficiency and MCUL are similar, although in the latter mutations tend to occur earlier in the gene and, perhaps, are more likely to result in a truncated or absent protein. We have found that not all mutation-carrier parents of FH deficiency children have a strong predisposition to leiomyomata. We have confirmed that renal carcinoma is sometimes part of MCUL, as part of the variant hereditary leiomyomatosis and renal cancer (HLRCC) syndrome, and have shown that these cancers may have either type II papillary or collecting duct morphology. We have found no association between the type or site of FH mutation and any aspect of the MCUL phenotype. Biochemical assay for reduced FH functional activity in the germline of MCUL patients can indicate carriers of FH mutations with high sensitivity and specificity, and can detect reduced FH activity in some patients without detectable FH mutations. We conclude that MCUL is probably a genetically homogeneous tumour predisposition syndrome, primarily resulting from absent or severely reduced fumarase activity, with currently unknown functional consequences for the smooth muscle or kidney cell.
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BACKGROUND: Scalp ringworm or tinea capitis has become an increasingly important public health issue in the past decade in Great Britain. Recently, certain dermatology departments in London have seen a large increase in tinea capitis in all its forms. OBJECTIVES: The aim of this paper is to present the detailed analysis of a cohort of 277 patients with tinea capitis seen during a 2-year period together with the latest local figures of tinea capitis cases from an inner city paediatric dermatology service. Methods Demographic, clinical and laboratory data were collected prospectively over 2 years from all cases of ringworm in patients seen in a paediatric clinic specially set up for scalp problems. RESULTS: Sixty-two per cent of 277 cases of scalp ringworm were caused by Trichophyton tonsurans, occurring mainly (91%) in patients with Afro-Caribbean hair type, more often in boys (68%), and in the 3-8 year olds (70%). Only 7% of the patients had received appropriate treatment with oral griseofulvin. An additional 156 cases from the general paediatric dermatology clinic showed 91%T. tonsurans infections. CONCLUSIONS: The prevalence of scalp ringworm appears to be reaching epidemic proportions in certain areas that include south-east London. The clinical problem is not yet well recognized by local general practitioners.
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Fixed drug eruption is characterized by recurrent well-defined lesions appearing in the same location each time the drug responsible is taken. A number of agents have been implicated. Metronidazole, a nitroimidazole agent widely used for its antibacterial and antiprotozoal activity, has been reported only rarely as the causative agent. We describe a patient with FDE due to metronidazole in whom we were able to induce the clinical and histological features of FDE by topical provocation testing. In agreement with the published literature we commend the use of topical provocation testing as a possible first-line investigation in the diagnosis of FDE. This may avoid the need for subsequent oral provocation testing and therefore the prevention of possible adverse sequelae.
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BACKGROUND: Tinea capitis is a common childhood infection that has recently become more frequent in urban areas in Europe and the U.S.A. The current licensed treatment in children is griseofulvin 10 mg kg(-1) daily, which is usually given for 6--8 weeks. OBJECTIVES: To compare this treatment with a 4-week course of oral terbinafine. METHODS: Terbinafine was given at the following doses: in children weighing < 20 kg, 62.5 mg daily; 20--40 kg, 125 mg daily; > 40 kg, 250 mg daily. Two hundred and ten children aged 2--16 years, with mycologically confirmed tinea capitis, were randomized to 4 weeks treatment with terbinafine or 8 weeks with griseofulvin, and followed for a total of 24 weeks to determine the difference between treatments with respect to short- and long-term efficacy and tolerability. RESULTS: One hundred and forty-seven patients were evaluable (terbinafine 77, griseofulvin 70). Although the 4-week course of terbinafine resulted in a trend to more rapid clearance of tinea capitis, there were no statistically significant differences between the two drugs in terms of overall outcome or tolerability, apart from in a subgroup of patients with Trichophyton infections, and weighing > 20 kg, who responded better to terbinafine than to griseofulvin at 4 weeks. By contrast, there was a better response to griseofulvin than to terbinafine in patients with Microsporum audouinii infections. CONCLUSIONS: Overall, the study showed that 4 weeks of treatment with oral terbinafine has similar efficacy to 8 weeks of treatment with griseofulvin for the management of tinea capitis in children.
Lipoatrophy semicircularis is thought to be a rare condition characterized by band-like horizontal depressions of the skin typically involving the lower limbs. Previous literature has suggested that repetitive trauma to the lower limbs could explain this condition; however, no direct causal link has ever been clearly established. There have also been several reports where no preceding history of trauma could be found. In our series we report seven cases of lipoatrophy semicircularis representing over one-third of the staff in a particular office. We propose that the most logical explanation for the indentations present in these individuals is repetitive trauma to their thighs by the sharp edge of the desks. The consistent nature of the distance between the floor and the horizontal indentations on the lower limbs despite differences in height weight and body mass index support this theory. It is likely that this condition is more common than initially thought.
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These guidelines for the management of tinea capitis have been prepared for dermatologists on behalf of the British Association of Dermatologists. They present evidence-based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines, and a brief overview of epidemiological aspects, diagnosis and investigation.
Recalcitrant viral warts are a troublesome therapeutic problem. Immunotherapy with the universal allergic contact sensitizer diphencyprone (DCP) has been used successfully in such cases. We have reviewed our experience of the use of DCP in the treatment of resistant hand and foot warts during an 8-year period. Sixty patients were sensitized to DCP during this time; the median duration of warts was 3 years. Twelve patients defaulted from treatment. Of the remaining 48 individuals, 42 (88%) cleared of all warts. The median number of treatments to clear was five (range one to 22) and the median time to clear was 5 months (range 0.5-14). Adverse effects occurred in 27 of 48 patients (56%), most commonly painful local blistering (n = 11), blistering at the sensitization site (n = 9), pompholyx-like reactions (n = 7) and eczematous eruptions (n = 4). Three of those who defaulted did so due to side-effects, one became pregnant and eight dropped out for unknown reasons. Three of the 48 patients who cleared or had at least six treatments also discontinued DCP therapy due to side-effects, but most tolerated treatment well. Twenty-five patients were followed up for periods of 1 month to 8 years (median 2 years) and none had a recurrence. DCP immunotherapy is an effective option for the treatment of recalcitrant viral warts but patients must be motivated to attend for sequential applications and must be warned about potential uncomfortable side-effects.
We recorded the diagnosis made in 461 black patients (187 children and 274 adults) attending a dermatology clinic between January and March 1996. In the childhood population, atopic eczema and tinea capitis were the most frequent dermatoses, comprising 63% of diagnoses recorded. In the adult population, acne and acne keloidalis nuchae were seen most frequently. Other conditions observed commonly were eczema, psoriasis, keloid scarring, pityriasis versicolor and postinflammatory changes. Our study demonstrates a wide spectrum of skin disease and includes disorders more common in black skin, disorders unique to black skin, those which present a greater cosmetic disability, and normal findings which have been mistaken for pathological disease.
We report a patient with purely cutaneous Rosai-Dorfman disease (RDD) who presented with a solitary, asymptomatic plaque on the back of her left thigh, with characteristic, large histiocytoid cells exhibiting emperipolesis histologically. Cutaneous lesions occur in 27% of patients with lymph node involvement in RDD however purely cutaneous disease has only been reported on 18 previous occasions. The aetiology is unknown, although it is though to be a reactive disorder rather than neoplastic, possibly an immunological response to an infectious agent.
BACKGROUND: Skin diseases represent one of the most frequent causes of morbidity in developing countries; however, little is known about the dermatologic needs of the population. The prevalence of skin disease in two different rural communities in southwestern Ethiopia was determined using descriptive epidemiologic techniques. METHODS: A household survey, designed to ascertain demographic information and dermatologic needs, was given to all households in both communities (827). The point prevalence of skin diseases was determined after examination by dermatologists of 768 self-selected individuals (40% of individuals were invited to attend dermatologic examination, either those self-reporting skin disease or identified as positive cases during the household survey); an individual survey form was given to all of these patients. RESULTS: Although 47% (S/UO) and 59% (Kishe) of the households in the two communities reported skin symptoms, the true dermatologic needs of these settlements were far greater than the expressed values, as examination by dermatologists of randomly selected households revealed that 67% of householders not reporting dermatoses had significant skin disease. During the point prevalence study, the commonest complaints were parasitic (scabies, pediculosis, and onchocerciasis) infestations (46% of diagnoses), followed by bacterial and fungal infections (33%); other conditions included endemic nonfilarial elephantiasis. Overcrowding was the main risk factor for infection. Thirty-two per cent (S/UO) and 39% (Kishe) of examined individuals had received previous treatments, which were ineffective in 74% and 63% respectively. CONCLUSIONS: Subsistence farmers spend a high proportion of their limited cash income on ineffective treatment. Simple schemes of management for the most common dermatoses, which local health workers could be trained to recognize and manage, could do much to redress the burden of skin disease in this population.