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Simultaneous occurrence of two rare cutaneous markers of poor prognosis in myelodysplastic syndrome: erythema elevatum diutinum and specific lesions.

We report the concomitant occurrence of erythema elevatum diutinum and specific skin lesions in a patient with a myelodysplastic syndrome (MDS). This patient's course, and review of other reported cases, support the opinion that neutrophilic dermatoses are associated with a poor prognosis of MDS. The simultaneous appearance of these manifestations could be the consequence of a particular chemotactism of myeloid cells, expressed after acute transformation.

Aged↗

Prevalence of dermatophyte onychomycosis in Spain: a cross-sectional study.

To evaluate the prevalence of dermatophyte onychomycosis in Spain, a cross-sectional study was conducted between 1992 and 1993. A total of 10,007 subjects over the age of 15 years were interviewed (using the computer-assisted telephone interview system), completed a directed questionnaire, and reviewed a series of photographs of diverse nail disorders. The period prevalence of onychomycosis was 2.6% and the point prevalence 1.7%. The prevalence of onychomycosis was higher in women (1.8%) than in men (0.8%). Age group distribution showed a higher onychomycosis prevalence (1.2%) in the oldest age group (> 55 years). With regard to localization, the prevalence of toenail onychomycosis was higher than that of fingernail onychomycosis and of concurrent infection in both sites. The results of this study suggest that 802,893 inhabitants of Spain have, or have previously suffered from dermatophyte onychomycosis. Only 38.6% have sought medical advice, and only 14% of those who did so consulted a dermatologist.

Adolescent↗

Detection of human papillomaviruses and eccrine ducts in palmoplantar epidermoid cysts.

Although epidermoid cysts of the palms and soles have long been assumed to develop following implantation of an epidermal fragment as a result of a penetrating injury, the pathogenic mechanism is still controversial, and the discovery of a more common aetiological agent is awaited. Clinical, histological, immunohistochemical and molecular biological studies were performed on 119 epidermoid cysts of palmoplantar location, in order to examine the role of the eccrine ducts, and human papillomavirus (HPV), in the pathogenesis of this disorder. Characteristic histological features were found, including intracytoplasmic eosinophilic bodies (ICB: in 14 cases, 12%) in the cyst wall, vacuolar structures (V: in 28 cases, 24%), or parakeratotic nuclei (P: in 85 cases, 71%) within the keratinous mass in the cyst cavity. Ductal structures suggesting eccrine ducts (E: in 63 cases, 53%) were also found in the cyst wall or in the cyst cavity. Either ductal structures or carcinoembryonic antigen expression (66 cases, 55%) were noted in a total of 73 cases (61%). Papillomavirus common antigens were detected in 36 cases (30%) showing one or more of the three distinct histological features, i.e. ICB, V and P. Subsequently, hybridization experiments to detect HPV DNA were performed in 47 cases, revealing an association between cysts showing ICB or V and the presence of HPV 60 DNA sequences. On the basis of our results, we propose that epidermoid cysts in the palmoplantar regions may develop from eccrine ducts, and that HPV and injury may play a role in their pathogenesis.

Adolescent↗

Successful treatment of acrodermatitis continua suppurativa with topical tacrolimus 0.1% ointment.

Acrodermatitis continua suppurativa of Hallopeau (ACS) is a rare pustular variant of psoriasis in which numerous treatment modalities have been used without any consistent long-term effect. We report for the first time two patients with ACS which was successfully treated with topical tacrolimus 0.1% ointment. Our observations raise hopes that this new treatment strategy for ACS may constitute a novel effective therapeutic option for this recalcitrant condition.

Acrodermatitis↗

A Japanese infant with localized ichthyosis linearis circumflexa on the palms and soles harbouring a compound heterozygous mutation in the SPINK5 gene.

We report a 6-month-old Japanese boy showing ichthyosis linearis circumflexa localized on the palms and soles. He showed bamboo hairs and aminoaciduria, and was positive for cow's milk and egg IgE antibodies by radioallergosorbent tests. Trypsin-like hydrolytic activity in the patient's lesional stratum corneum showed an activity seven times higher than that in age-matched controls. DNA analysis showed that the patient harboured the compound heterozygous mutations R790X and 1220+1 G-->C in the SPINK5 gene, compatible with the diagnosis of Netherton syndrome (NS). As the genotype/phenotype correlations in NS have not yet been fully clarified, the position of the premature termination codon in the SPINK5 gene may contribute to explain such a mild form of NS in our patient.

Asian People↗

A family with palmoplantar epidermolytic hyperkeratosis.

Familial epidermolytic hyperkeratosis confined to the palms and soles was first characterized by Klaus and Weinstein in 1970. This entity has been the subject of only four subsequent reports. We report a family previously diagnosed as suffering from tylosis (Thost Unna syndrome), in which eleven members have been affected, and review the literature on this disease. It would seem possible that this entity may be more common than the sparse literature would indicate, and that other cases may currently be masquerading as tylosis.

Adolescent↗

Epidermolysis bullosa acquisita occurring in association with systemic lupus erythematosus.

A 77-year-old retired male physician with a 6-year history of systemic lupus erythematosus (SLE) developed a mechanobullous eruption, the features of which were clinically and immunopathologically consistent with a diagnosis of 'classical' epidermolysis bullosa acquisita (EBA). As EBA shares immunopathological findings with a number of cases reported as the 'bullous eruption of SLE', the clinical findings commonly recognized as 'classical EBA' may, in patients with SLE, represent a specific subset of the bullous eruption of SLE rather than a separate diagnostic entity. There are few reports in the literature describing classical EBA in patients with SLE. Findings in this patient add further support to the suggestion that EBA occurring in association with SLE, represents a subset of the bullous eruption of SLE, the clinical features of which may be modified by genetic susceptibility or disease activity.

Aged↗