Biomedical subjects
Publications and source records attributed to F M Keane.
We report the first case of a pseudolymphomatous skin reaction precipitated by flucloxacillin. Skin histology was suggestive of a cutaneous lymphoma, and DNA analysis by single stranded conformational polymorphism (SSCP) demonstrated T-cell receptor gamma gene monoclonality. Withdrawal of flucloxacillin led to immediate clinical improvement and gradual resolution of skin rash and lymph nodes.
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OBJECTIVE: To determine whether Chinese herbal creams used for the treatment of dermatological conditions contain steroids. DESIGN: 11 herbal creams obtained from patients attending general and paediatric dermatology outpatient clinics were analysed with high resolution gas chromatography and mass spectrometry. SETTING: Departments of dermatology and clinical biochemistry. MAIN OUTCOME MEASURE: Presence of steroid. RESULTS: Eight creams contained dexamethasone at a mean concentration of 456 micrograms/g (range 64 to 1500 micrograms/g). All were applied to areas of sensitive skin such as face and flexures. CONCLUSION: Greater regulation needs to be imposed on Chinese herbalists to prevent illegal and inappropriate prescribing of potent steroids.
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.
Recently, the first example of a human mutation in the gene encoding the desmosomal plaque protein, desmoplakin, has been described in a patient with autosomal dominant striate palmoplantar kerato-derma. We now report a further case of a desmoplakin mutation in a proband with striate palmoplantar keratoderma that also results in a null allele and haploinsufficiency. The mutation was a heterozygous G > A transition at the donor + 1 site of intron 7 of the desmoplakin gene (939 + 1 G > A; Genbank M77830). The aberrant splicing leads to retention of the entire intron 7, which contains a premature termination codon within the N-terminal domain of the peptide. Because the mutant null allele could not be identified on cDNA sequencing, we determined by polymerase chain reaction the exon-intron organization of the desmoplakin gene to facilitate analysis of genomic DNA. The gene spans approximately 45 kb of chromosome 6 and comprises 24 exons ranging in size from 51 bp to 3922 bp. We have also characterized fully the 3'UTR of the desmoplakin cDNA. This study demonstrates the relevance of haploinsufficiency for desmoplakin in the pathogenesis of this genodermatosis. Assessment of family members bearing the mutant allele also emphasizes the significance of an individual's age and exposure to skin trauma in manifesting full phenotypic expression of the disorder.
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