A study was undertaken of new referrals by GPs to a dermatology clinic in a district general hospital over a 6-month period. Six hundred and eighty-six consecutive referrals to one consultant were analysed for diagnostic accuracy and requirement for referral. Only 47% of referral letters contained the correct diagnosis. Viral warts and psoriasis were best diagnosed (82 and 78%, respectively), but seborrhoeic warts and dermatofibromas caused difficulty (22 and 19%, respectively). Cutaneous malignancy was correctly diagnosed in 45% of referrals, and eczema, the commonest condition referred, in 54% of cases. Sixty-eight percent of referrals required hospital-based facilities for diagnosis (31%) or treatment/management (37%). Twenty-one per cent of patients referred attended for once-only visits, requiring no specialized diagnostic or therapeutic procedures. Such referrals should decrease with improved GP education. Eleven percent of referrals were for minor surgical procedures such as curettage, shave biopsy, or cryotherapy and would become unnecessary if such facilities were available in the community. Our data demonstrate the potential for management of up to one-third of current dermatological referrals within the community by improving education of GPs and providing appropriate facilities within the community. However, over two-thirds of patients required hospital facilities, a finding of considerable relevance to the future location of dermatological services.
We report a large family with ichthyosis bullosa of Siemens (IBS) including eight affected members spanning three generations. The classical features of the disease were consistently observed with blistering, superficial peeling of the skin, and localized lichenified hyperkeratosis mainly confined to the limbs. Phenotypic variation, however, was also observed with some individuals exhibiting unusual clinical features. Specifically, the index patient was erythrodermic at birth; she subsequently developed a widespread pustular eruption. Erythroderma is classically absent in IBS and pustulation is very unusual. She also had hypertrichosis of the limbs, as did an affected female first cousin. This has not previously been reported in IBS. Electron microscopy showed complex aggregates of keratin in the spinous and granular layers associated, in places, with remarkably little cell lysis. Sequencing of genomic DNA revealed a mutation (E493K) in keratin 2e. A review of the literature on IBS indicates that E493K is the most commonly reported mutation to date and might represent a mutational hotspot for this disease.
We report a case of bullosis diabeticorum with blisters confined to the lower legs and feet. Histology of a lesion demonstrated a bulla at the dermo-epidermal junction, and ultrastructural studies confirmed the split to be at the level of the lamina lucida which we propose is the site of the pathology in this condition. Immunofluorescence studies were negative, excluding an immunobullous disease. We postulate that in our patient a combination of increased venous pressure and poor vascular supply in diabetic skin, led to the blister formation. The literature on this condition is reviewed.
Fifteen unequivocal basal cell carcinomas (BCC) and ten unequivocal trichoepitheliomas (TE) were studied using the lectin peanut agglutinin (PNA), and the monoclonal antibodies Q bend 10 and bcl-2 oncoprotein directed against the antigens CD34 and bcl-2, respectively, to see whether these markers could be used to differentiate between the two tumors. Ten percent of TE demonstrated a continuous band-like peritumorous staining with PNA and 80% demonstrated a discontinuous band-like peritumorous staining with PNA, with the comparable figures for BCC being 40% and 20%, respectively. In addition, 40% of BCC showed focal areas of pemphigus-like staining in contrast with only 10% of TE. Using the antibody directed against bcl-2, TE demonstrated weak staining mainly confined to the basal layer of tumor cells in 20% of cases and staining of the cells throughout the tumor in 30% of cases. Similarly, BCC also showed staining of the basal layer of tumor cells in 7% of specimens and staining of cells throughout the tumor mass in 40% of specimens studied. Finally, with the antibody Q bend 10 directed against CD34, staining of the immediate peritumoral spindle-shaped cells was observed in 20% of TE compared with 7% of BCC. Despite reports in the literature, we found that none of these three markers can be reliably used to differentiate between TE and BCC.
Although pellagra is a recognized complication of isoniazid therapy, the diagnosis may be overlooked or delayed--sometimes with life-threatening consequences. We report a case of isoniazid-induced pellagra which occurred despite pyridoxine supplementation. Drug withdrawal and supplementation with niacin led to a rapid and sustained clinical improvement. The possible mechanisms of isoniazid induced pellagra are discussed.
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