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  • 1
    Electronic Resource
    Electronic Resource
    Combined treatment of epidermodysplasia verruciformis with etretinate and α-interferon (1992)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of the European Academy of Dermatology and Venereology 1 (1992), S. 0 
    Details
    ISSN: 1468-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Epidermodysplasia verruciformis (EV) is an uncommon cutaneous disease in which a focal and genetically determined immunological impairment is associated with chronic human papilloma virus (HPV) infection. In sun-exposed areas, when an oncogenic HPV type is the agent, skin cancer may occur. The treatment of EV is difficult and often unsatisfactory; etretinate has been reported in some instances as effective in improving lesions. We report a typical case of EV with pityriasis versicolor-like lesions on the trunk and many flat, erythematous wart-like lesions on the face, dorsal areas of the hands and legs. We performed a treatment with etretinate (1 mg/kg/day for 6 weeks) and subsequently with etretinate and α2r-interferon (3 MU, 3 times per week for 2 weeks, then 6 MU, as above, for 4 weeks). After 4 weeks of therapy with etretinate we observed moderate improvement; we did not observe any further clinical improvement in the final 2 weeks. The subsequent combined treatment with etretinate and a-interferon achieved further improvement. We conclude that the association of etretinate and a-interferon may represent an efficacious treatment of EV.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1468-3083.1992.tb00700.x
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  • 2
    Electronic Resource
    Electronic Resource
    Granulomatous slack skin: report of a case associated with Hodgkin's disease and a review of the literature (1994)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 131 (1994), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We report a case of granulomatous slack skin (GSS) associated with Hodgkin's disease, and review the literature on this entity. GSS, a variant of cutaneous T-cell lymphoma, clinically presents with erythematous patches in the flexures, which gradually transform into bulky, pendulous areas of skin. Histology shows an elastolytic granulomatous infiltrate, with atypical lymphoid cells, and occasional epidermotropism. As far as we are aware, 10 cases of GSS, including our patient, have been reported in detail. The male: female ratio of these cases is 9:1, and the age range 15–51 years. Five cases were associated with Hodgkin's disease, one with small lymphocytic lymphoma, and one developed cutaneous T-cell lymphoma. The axillae, abdomen and groins were the most frequently affected areas. No definitive management for GSS has been established. Surgery has been performed in localized forms, and systemic treatments have included corticosteroids, dapsone, chlorambucil, nitrogen mustard, and radiotherapy. Our patient was treated with chemotherapy for his Hodgkin's disease, and this resulted in complete remission of the lymphoma. Subsequent maintenance therapy with interferon-alpha produced good control of the cutaneous lesions.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1994.tb08505.x
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  • 3
    Electronic Resource
    Electronic Resource
    HIV-Specific IgG3 in Cord Blood: Predictive Value for Seroreversion (1993)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 693 (1993), S. 0 
    Details
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1749-6632.1993.tb26277.x
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  • 4
    Electronic Resource
    Electronic Resource
    Indeterminate Western Blot in Children Who Serorevert for HIV (1993)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 693 (1993), S. 0 
    Details
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1749-6632.1993.tb26281.x
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  • 5
    Electronic Resource
    Electronic Resource
    Transfusion-associated graft-versus-host disease—report of two further cases with an immunohistochemical analysis (1994)
    Oxford, UK : Blackwell Publishing Ltd
    Clinical and experimental dermatology 19 (1994), S. 0 
    Details
    ISSN: 1365-2230
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Transfusion-associated graft-vs.-host disease (tGVHD) is a severe disease usually affecting immunocompromised hosts with haematological neoplasia. Two patients with acute leukaemia are reported, who developed fatal tGVHD after blood transfusions. Intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule 1 (VCAM-1) and endothelial leucocyte adhesion molecule 1 (ELAM-1) expression and the CD4/CD8 ratio were assessed in lesional skin. ICAM-1 was strongly expressed on epidermal keratinocytes and endothelial cells (EC) and correlated with HLA-DR staining. VCAM-1 was strongly expressed on EC in the superficial dermal vessels. ELAM-1 stained weakly on EC in some of the superficial vessels. CD8+ lymphocytes showed prominent epidermotropism; the CD4/CD8 ratio was 0.8 in case 1 and 1.2 in case 2. Infiltrating cells were positive for CD3, CD11a, and CD18. Langcrhans1 cells were almost completely absent. The dermatologist must be aware of the importance of such a rare, unexpected and almost always fatal complication of blood transfusion, in order to make an early diagnosis. Irradiation of blood products is the only effective way to prevent tGVHD in all subjects at risk.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2230.1994.tb01112.x
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  • 6
    Electronic Resource
    Electronic Resource
    Localized crusted scabies in the acquired immunodeficiency syndrome (1992)
    Oxford, UK : Blackwell Publishing Ltd
    Clinical and experimental dermatology 17 (1992), S. 0 
    Details
    ISSN: 1365-2230
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Crusted scabies (CrS) is an uncommon occurrence among patients with AIDS. Indeed to date only five cases have been described, all with widespread lesions. A case of localized CrS appearing as a yellowish and crusted plaque on the second right toe is reported in a woman with AIDS. Scraping off the verrucous surface, as well as punch-biopsy revealed many mites within the horny layer. The infestation is related to the cutaneous immune response and thus CrS should be considered an opportunistic infestation in AIDS. The importance of the early diagnosis of CrS in order to prevent disseminated lesions and involvement of other health-care workers is underlined.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2230.1992.tb00225.x
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  • 7
    Electronic Resource
    Electronic Resource
    Reply (1994)
    Oxford, UK : Blackwell Publishing Ltd
    Clinical and experimental dermatology 19 (1994), S. 0 
    Details
    ISSN: 1365-2230
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2230.1994.tb02714.x
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  • 8
    Electronic Resource
    Electronic Resource
    A novel mutation of the extracellular matrix protein 1 gene (ECM1) in a patient with lipoid proteinosis (Urbach–Wiethe disease) from Sicily (2005)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 153 (2005), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background  Lipoid proteinosis (LP), also known as Urbach–Wiethe disease, is a rare autosomal recessive disorder characterized by a hoarse voice, warty skin infiltration and scarring. Mutations within the extracellular matrix protein 1 (ECM1) gene cause LP.Objectives  We report the molecular analysis of the ECM1 gene in a Sicilian patient with LP in order to extend the mutation spectrum of this genodermatosis.Methods  We studied a 32-year-old female born from consanguineous parents who was diagnosed at the age of 11 years as having LP. She has a clinical phenotype corresponding to Urbach–Wiethe disease characterized by papules/nodules, indurated plaques and sometimes ulcerated lesions primarily involving the skin and mucous membranes, and extracutaneous features such as epilepsy, hoarseness of the voice and neuropsychiatric abnormalities. Samples of clinically affected skin obtained by biopsies were analysed after staining with haematoxylin and eosin, periodic acid–Schiff (PAS), and PAS–diastase. The whole ECM1 gene was analysed by direct sequencing.Results  We identified a homozygous nonsense mutation in exon 6 of the ECM1 gene, C589T (Q197Ter).Conclusions  Over 60% of mutations occur in exons 6 and 7. Exon 7 is alternatively spliced and frameshift mutations in exon 7 lead to ablation of the ECM1a transcript, but not the shorter ECM1b transcript that normally lacks this exon. Homozygous nonsense or frameshift mutations in exon 6 are predicted to affect both full-length ECM1a and ECM1b transcripts, whereas ECM1b should be unaffected for similar types of mutation in exon 7. It has been suggested that individuals with mutations in exon 7 have a slightly milder phenotype than those with exon 6 mutations. This is the first report with respect to a novel mutation of the ECM1 gene responsible for recessive LP in Sicily.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.2005.06842.x
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  • 9
    Electronic Resource
    Electronic Resource
    The behaviour of Bcl-2, Bax and Bcl-x in Darier's disease (2002)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 147 (2002), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary Background Darier's disease (DD) is a rare autosomal dominant disorder of keratinization caused by a mutation of the ATP2A2 gene. There is little information on the behaviour of Bcl-2, Bax and Bcl-x in DD. Objectives To investigate the dynamic control and the behaviour of Bax, Bcl-2 and Bcl-x in DD. We asked whether members of the Bcl-2 family might manifest their effects through modulation of intracellular calcium signalling or whether the gene that encodes the sarco/endoplasmic reticulum Ca2+ ATPase isoform 2 (SERCA2) modulates the Bcl-2 family in the regulation of apoptosis in DD. Methods Immunohistochemical methods were used. Results There was no immunoreactivity for Bcl-2 and Bcl-x in epidermal keratinocytes in lesional epidermis. Staining for Bax was evident in the cells of the perilesional uninvolved skin, but decreased in the epidermal cells of lesional involved skin. Conclusions The decrease or absence of Bcl-2 and Bcl-x and the imbalance of Bax in the epithelial cells of affected DD skin is likely to be an important control point determined by the genetic mutation of SERCA2, which modifies the programme of the antiapoptotic proteins. The consequent imbalance of the factors controlling apoptosis in keratinocytes underlines another apoptotic pathway responsible for the dyskeratotic cells in DD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2002.04963.x
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  • 10
    Electronic Resource
    Electronic Resource
    Proliferating tricholemmal tumour with lymph node metastases (1989)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 121 (1989), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: A 42-year-old woman with a proliferating tricholemmal tumour (PTT) with regional lymph node metastases is reported. Histochemical studies showed evidence of tricholemmal keratinization. There was recurrence following excision and subsequently the patient developed lymph node metastases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1989.tb08224.x
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