We describe two patients with unusual asymptomatic, papular lesions on the vulva, clinically resembling lichen planus, the histology of which revealed unexpected findings of suprabasilar clefting, acantholysis and dyskeratotic cells giving rise to corps ronds and grains together with hyperkeratosis and parakeratosis, features originally associated with a diagnosis of Darier's disease. Focal acantholytic dyskeratosis has been described in a wide variety of inflammatory and neoplastic processes including those involving mucous membranes and has been attributed various diagnostic labels. We feel that the findings in our patients are consistent with a diagnosis of papular acantholytic dyskeratosis of the vulva, a rare condition, which was first described in 1984.
We report three infants with a rare syndrome of restrictive dermopathy, in which rigidity of the skin at birth is associated with characteristic facial anomalies, generalised arthrogryposis, bony abnormalities, and lung hypoplasia. The skin has a distinctive pathology with compaction of the dermal coliagen and fibrosis of the subcutaneous tissue. The inheritance is likely to be autosomal recessive and the condition appears to be fatal in the early neonatal period.Several cases of the fetal akinesia/hypokinesia deformation syndrome have been described, where facial anomalies, arthrogryposis, and lung hypoplasia are associated. Examples include Pena-Shokier, NeuLaxova, cerebro-oculo-facio-skeletal, and lethal
Pseudoainhum is a term used to describe the presence of constricting bands of the extremities due to a variety of underlying causes. Progression of the lesions can cause irreversible damage and autoamputation of the affected digit. This report documents a rare association of pseudoainhum and psoriasis and emphasizes the importance of recognizing this condition.
The cell surface expression of beta-2-microglobulin (beta 2 M) was investigated in 33 keratoacanthomas (KA) and 58 squamous cell carcinomas (SCC) to determine whether this antigen was expressed to a different extent in these two conditions and, thus, whether this constitutes a reliable and practical test for distinguishing them. Loss of beta 2 M expression was not a reliable feature for distinguishing between KA and SCC and seemed to be related more to the degree of cellular differentiation and maturation, than to malignancy as such.
Dicaprylyl maleate (DCM) has been reported rarely as a cause of allergic contact dermatitis. The objectives of this study were to identify patients from multiple centres with allergy to DCM in cosmetic products confirmed by patch testing and, in addition, to investigate the effect of testing with aged DCM. This is an international multicentre study of 22 patients with 26 reactions to products containing DCM. Patch testing was carried out to ingredients including DCM obtained from the manufacturer. Further testing was carried out with deliberately aged DCM in a sample of patients. 22 patients had clinical and positive patch test reactions at 4 days to a total of 26 cosmetic products containing DCM. 5 patients did not react to DCM prepared by the manufacturer from concurrent factory stock but did have positive reactions to a deliberately aged batch of DCM. DCM is an emerging cosmetic allergen. Testing with aged material yields a greater number of positive results. Co-operation between cosmetics manufacturers and clinicians is important in the identification of new allergens.
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