Familial juvenile elastoma.
Two cases of familial juvenile elastoma are reported with a review of the literature concerning juvenile elastoma and the Buschke-Ollendorff syndrome of connective tissue naevi in association with osteopoikilosis.
Biomedical subjects
Publications and source records attributed to W A Griffiths.
Two cases of familial juvenile elastoma are reported with a review of the literature concerning juvenile elastoma and the Buschke-Ollendorff syndrome of connective tissue naevi in association with osteopoikilosis.
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Short-contact treatment with dithranol (anthralin) is a widely used treatment for chronic plaque psoriasis. Although effective, it causes staining and irritation, and is therefore inconvenient. Calcipotriol is a recently developed vitamin D analogue which is effective and easy to use. To evaluate the relative efficacy, safety and acceptability of these treatments a multicentre, open, randomized, parallel-group comparison was performed. Four hundred and seventy-eight patients with chronic plaque psoriasis were randomized to use one of the two treatments for 8 weeks. One group applied calcipotriol ointment (50 micrograms/g) twice daily. The other used a single application for 30 min each day of Dithrocream in the highest concentration tolerated. Severity of psoriasis was assessed by modified PASI score at baseline, and after 2, 4, and 8 weeks of treatment. A five-point scale was used by subjects and by investigators as an additional assessment of overall response, and a similar scale was used by subjects to grade acceptability. Total serum calcium was monitored at baseline and after 2 and 8 weeks on treatment. The mean PASI score fell from 9.1 to 4.7 after 8 weeks on dithranol (P < 0.001), and from 9.4 to 3.4 on calcipotriol (P < 0.001). The difference between the two treatments was significant in favour of calcipotriol at 2 weeks (P < 0.001), and remained so at subsequent assessments. At 8 weeks the difference between mean improvements in scores for the two groups was 1.6 (95% confidence interval 0.5-2.7). Efficacy grading by subjects and investigators, and acceptability grading by subjects, were all significantly better for calcipotriol.(ABSTRACT TRUNCATED AT 250 WORDS)
We report three patients with pemphigoid nodularis. Patients were females aged 76, 71 and 50 years, and all had features of bullous pemphigoid together with prurigo-like lesions at some stage of their illness. In two cases, nodular lesions preceded the onset of blistering by some months. Blisters arose on normal skin and in one patients also at sites of prurigo lesions. Routine histology of bullous lesions revealed the presence of subepidermal blisters. Electron microscopy performed in two cases confirmed the level of split to be through the lamina lucida. Direct immunofluorescence in all cases was positive, with linear basement membrane zone deposition of IgG and C3. Circulating IgG anti-basement membrane antibody was also detected in all patients, and in two, immunoblotting revealed a single antigen of 220 kD.
The nomenclature relating to structures at the distal part of the nail is confusing. A distal yellow line traversing the nail, described by Pinkus, has been relatively ignored in the literature and remains unnamed. Clinical and histological studies presented in this article show that this band is present in more than 90% of normal adult fingernails and represents the most proximal point of attachment of the fingertip stratum corneum to the nail plate. Therefore, it should be referred to as the onychocorneal band or junction. This region has distinctive histological features and is the first major barrier to material passing proximally beneath the nail plate. It is possible that abnormalities of this structure may result in onycholysis, pachyonychia congenita, and pterygium inversum unguis.
Subcorneal pustular dermatosis (SCPD) (Sneddon-Wilkinson disease) is a rare disorder. We present a case with classical clinical manifestations, and discuss the management of this disorder.
A patient with Darier's disease was discovered to have persistent, asymptomatic cervical and thoracic spinal hyperostoses after receiving isotretinoin for 7 years. The spinal abnormalities have remained asymptomatic but have now progressed following 4 years of etretinate therapy. The development of skeletal abnormalities, in particular spinal hyperostosis, is well-documented in patients receiving the synthetic retinoid, isotretinoin (Accutane, Roaccutane). The occurrence of extraspinal tendon and ligament calcification has been emphasized following long-term therapy with etretinate (Tegison, Tigason), but the relationship between etretinate and spinal hyperostosis is less certain, there being a need for a long-term, prospective, appropriately controlled investigation of patients receiving etretinate. We report a patient with Darier's disease who was discovered to have prominent, asymptomatic cervical and thoracic spinal hyperostoses after receiving isotretinoin for 7 years. Subsequent treatment with etretinate for 4 years did not prevent progression of the spinal abnormalities.
Aphthous ulcers are notoriously difficult to treat. We report repeated remission of recurrent aphthous ulceration in a female while taking etretinate for plantar pustular psoriasis. Although systemic retinoids are potent teratogens and are generally inappropriate for women of childbearing potential, this case report suggests further assessment of retinoids in severe aphthosis is warranted in selected patients.
Two cases are presented of congenital linear and whorled hypermelanosis. Hyperpigmented macules in streaky configurations along Blaschko's lines appeared gradually after birth. Histologic examination revealed prominent epidermal melanocytes and irregular basal layer hyperpigmentation with normal melanosomes. This condition must be differentiated from incontinentia pigmenti, early systematized epidermal nevus, extensive hypomelanosis of Ito, and chimerism. Other similar case reports from the literature suggest that incidence is sporadic and may be associated with more serious congenital anomalies. The patterning is the inverse to that found in hypomelanosis of Ito. Developmental somatic mosaicism may be responsible for this patterned hypermelanosis.
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We describe a patient with Klinefelter's syndrome complicated by recalcitrant leg ulcers, in whom marked platelet hyperaggregability was demonstrated and venous disease excluded. Androgen replacement therapy appeared to bring about healing of the patient's ulcers and was associated with reversal of the platelet abnormality. The possible role of androgen deficiency in the pathogenesis of leg ulceration is discussed.
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