Clinical and experimental dermatology
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Proteus syndrome (PS) is a complex hamartomatous disorder defined by local overgrowth (macrodactyly or hemihypertrophy), subcutaneous tumours and various bone, cutaneous and/or vascular anomalies (VA). VA are manifold in PS, but their prevalence is unknown so far. In order to further characterize PS, we studied the prevalence of VA in 22 PS patients presenting to our outpatient clinic and reviewed 100 PS patients previously reported between 1983 and 2001. ⋯ Unlike Klippel-Trenaunay syndrome, where VA are mostly confined to the hypertrophic limb, major arteriovenous anomalies are rare, and - similar to the other hamartomas and naevi observed in PS (pigmentary naevi, epidermal naevi, subcutaneous tumours, exostoses) - VA appear to be distributed at random sites on the body. We conclude that VA are among the most common findings in PS. Their varying type and distribution lend further support to the concept of somatic mosaicism.
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Clin. Exp. Dermatol. · May 2003
ReviewThe clinical spectrum of nonbullous congenital ichthyosiform erythroderma and lamellar ichthyosis.
Until about 20 years ago, the term lamellar ichthyosis (LI) represented all nonbullous autosomal recessive ichthyoses except for harlequin ichthyosis and ichthyosis syndromes. Since the 1980s, nonbullous autosomal recessive ichthyoses have been divided into two major clinical entities, nonbullous congenital ichthyosiform erythroderma (NBCIE) and LI. The nature of scaling and intensity of erythroderma are important clinical features that distinguish between NBCIE and LI. ⋯ Mutations in any of the three known causative genes, TGM1, ALOXE3 or ALOX12B, can lead either to NBCIE or LI. Candidate genes specific to either NBCIE or LI alone have not been identified. Based on these facts, it might be better to consider NBCIE and LI as variations of a single keratinization disorder, although the classification of these autosomal recessive congenital ichthyosis patients into NBCIE or LI depending on their clinical features is still useful for practical patient management.
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Rubber is an important industrial and consumer product encountered in many household items and medical devices. Whereas the chemical additives used in its manufacture are well recognized as a cause of delayed-type hypersensitivity (allergic contact dermatitis), it is only during the past decade that immediate-type allergy to natural rubber latex (NRL) proteins (latex allergy) has emerged as a serious health issue. ⋯ Undiagnosed latex allergy is potentially very serious for patients. This article briefly overviews latex allergy with emphasis on the practical issues of diagnosis and management.
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Clin. Exp. Dermatol. · Mar 1998
Review Case ReportsA case of atrophoderma vermiculatum responding to isotretinoin.
Atrophoderma vermiculatum (AV) is a rare disorder leading to reticular or honeycomb scarring of the face and responding poorly to treatment. A case is now presented of the successful induction of remission in the inflammatory component of the disease following a prolonged course of isotretinoin; improvement was then maintained after cessation of the treatment. In severe cases of AV with significant scarring, a trial of isotretinoin therapy is thus worthwhile in an attempt to stop progression of the disease and improve its cosmetic appearance.
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Flagellate erythema, described by Moulin in 1970 as reminiscent of the lesions produced by flogging and by other authors as zebra-like lesions, was noted first in association with bleomycin treatment, but later occasionally reported in relationship with dermatomyositis, mostly from Japan. It is characterized by linear, erythematous, cutaneous streaks, pruriginous or painful, arising on an oedematous background and disappearing spontaneously over several weeks. So far, fewer than five cases have been reported in association with dermatomyositis, but we now present a further one.