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Enfermedad de Hailey-Hailey en varias generaciones de una familia
López Pupo,Natacha; Tablada Robinet,María Elena; López Osorio,Danisela; Selva López,Varinia; Solá Owen,Angélica;
MEDISAN , 2012,
Abstract: two case reports of adult siblings are described, who presented with benign familial pemphigus or hailey-hailey disease --type of genodermatosis by altered epidermal cohesion-- attended in "dr. juan bruno zayas alfonso" teaching general hospital of santiago de cuba. patients were treated with steroids at anti-inflammatory doses, antibiotics, and topical plant liniment and aloe cream, besides receiving flower therapy. in general, both of them improved the clinical picture during their stay in this institution.
Enfermedad de Hailey-Hailey en varias generaciones de una familia Hailey - Hailey disease in several generations of a family  [cached]
Natacha López Pupo,María Elena Tablada Robinet,Danisela López Osorio,Varinia Selva López
MEDISAN , 2012,
Abstract: Se describen 2 casos clínicos de hermanos adultos que presentaban pénfigo benigno familiar o enfermedad de Hailey-Hailey --tipo de genodermatosis por alteración en la cohesión epidérmica--, atendidos en el Hospital General Docente "Dr. Juan Bruno Zayas Alfonso" de Santiago de Cuba. Los pacientes fueron tratados con esteroides en dosis antiinflamatorias, antibióticos y, de forma tópica, linimento vegetal y crema de aloe, además de recibir terapia floral. De manera general, ambos mejoraron su cuadro clínico durante la estadía en dicha institución hospitalaria. Two case reports of adult siblings are described, who presented with benign familial pemphigus or Hailey-Hailey disease --type of genodermatosis by altered epidermal cohesion-- attended in "Dr. Juan Bruno Zayas Alfonso" Teaching General Hospital of Santiago de Cuba. Patients were treated with steroids at anti-inflammatory doses, antibiotics, and topical plant liniment and aloe cream, besides receiving flower therapy. In general, both of them improved the clinical picture during their stay in this institution.
Hailey Hailey disease: a case report
Iffat Hassan,Abid Keen
Our Dermatology Online , 2012,
Abstract: Hailey Hailey disease or Familial chronic benign pemphigus is a rare autosomal dominant acantholytic disease, clinically characterized by flaccid bullae and erosions in the intertriginous areas, mainly the axillary and inguinal region. We herein report a case of a forty year old female belonging to ethnic Kashmiri population with clinical and histopathological features suggestive of Hailey Hailey disease.
Benign familial pemphigus (Hailey-Hailey disease) responsive to low dose cyclosporine
Nanda Arti,Khawaja Fatima,Harbi Rafat,Nanda Mousmee
Indian Journal of Dermatology, Venereology and Leprology , 2010,
Abstract:
Hailey- Hailey Disease - A Report Of Four Cases
Das Jayasri,Sarkar Jyothirindra Nath,Roy Aloke Kumar
Indian Journal of Dermatology , 2002,
Abstract: Hailey-Hailey disease is a benign familial chronic vesicobullous disease with unique histopathology. Four cases of this rare genodermatosis are reported here. All of them had the history of exacerbation in hot and humid weather.
Immunomodulators in Dermatology  [PDF]
Eda Kumbasar,Gonca G?kdemir,Adem K??lü
Turkderm , 2008,
Abstract: Immunomodulators include both immunostimulatory and immunosuppressive agents. The introduction of this new class of drugs marked the beginning of the post-cortisone era in clinical dermatology. Topical immunotherapy with both immunostimulatory and immunosuppressive agents show potential for effective treatment of inflammatory, infectious and neoplastic skin diseases. These agents have few side effects and have clinical effects similar to corticosteroids. The purpose of this review is to introduce to our collagues potentially useful immunomodulators in clinical dermatology. (Turkderm 2008; 42: 3-8)
Pemphigus
Derya U?mak
Journal of Clinical and Experimental Investigations , 2011,
Abstract: Pemphigus is an uncommon autoimmune mucocutaneousblistering disease, mediated by antibodies directedagainst proteins present on the surface of keratinocytesthat provide mechanical structure to the epidermis. Incidencesof the various forms of pemphigus vary fromcountry to country. Pemphigus vulgaris consists of the80% of pemphigus cases and it occurs 10 times morethan pemphigus foliaceus. There have been several recentadvances in the management of pemphigus. Treatmentof with systemic steroids and cytotoxic immunosuppressantscan be highly effective. This article will discussits epidemiology, pathogenesis, clinical variants and approachesto treatment. J Clin Exp Invest 2011; 2 (4): 468-473
Genital Hailey- Hailey disease: a case report  [cached]
Deeptara Pathak Thapa,Anil Kumar Jha,Sujata Pudasaini,Chandani Kharel
Our Dermatology Online , 2013,
Abstract: Hailey – Hailey disease is a rare autosomal dominant acantholytic disorder, previously not reported from Nepal. We report a case of 30 years old female who presented with pruritic hyperkeratotic papules and plaques on vulva, perianal area and inner left thigh for a period of one year. Biopsy from the lesion showed suprabasal acantholysis with loss of intercellular bridges resulting in a dilapidated brick-wall appearance; characteristic of Hailey – Hailey disease. Treatment of this disease till date is far from satisfactory.
Topical immunomodulators in dermatology  [cached]
Khandpur Sujay,Sharma V,Sumanth K
Journal of Postgraduate Medicine , 2004,
Abstract: Topical immunomodulators are agents that regulate the local immune response of the skin. They are now emerging as the therapy of choice for several immune-mediated dermatoses such as atopic dermatitis, contact allergic dermatitis, alopecia areata, psoriasis, vitiligo, connective tissue disorders such as morphea and lupus erythematosus, disorders of keratinization and several benign and malignant skin tumours, because of their comparable efficacy, ease of application and greater safety than their systemic counterparts. They can be used on a domiciliary basis for longer periods without aggressive monitoring. In this article, we have discussed the mechanism of action, common indications and side-effects of the commonly used topical immunomodulators, excluding topical steroids. Moreover, newer agents, which are still in the experimental stages, have also been described. A MEDLINE search was undertaken using the key words "topical immunomodulators, dermatology" and related articles were also searched. In addition, a manual search for many Indian articles, which are not indexed, was also carried out. Wherever possible, the full article was reviewed. If the full article could not be traced, the abstract was used.
Childhood pemphigus  [cached]
De Dipankar,Kanwar Amrinder
Indian Journal of Dermatology , 2006,
Abstract: Childhood pemphigus is a rare diasease and comprises only a minority of immunobullous diseases seen in children. Almost all variants of pemphigus in childhood have been described in literature. Pemphigus vulagaris is the most common type seen, after excluding endemic foliaceus. The clinical features are essentially similar to that of adults. Special consideration is required while treating children so as to use drugs which are less carcinogenic and do not affect fertility. We present here a review of cases of childhood pemphigus vulgaris, pemphigus foliaceus, pemphigus vegetans, pemphigus erythematosus, paraneoplastic pemphigus, IgA pemphigus and neonatal pemphigus.
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