The frequency of osteoporosis in patients with pemphigus vulgaris on treatment.

BACKGROUND: Pemphigus vulgaris was almost fatal before the advent of glucocorticoids. Unfortunately, the high doses and prolonged administration of glucocorticoids, which often needed to control the disease, result in numerous adverse effects many of which are serious. AIMS: To evaluate the patients with pemphigus vulgaris on treatment in respect of osteoporosis and to compare the frequency of osteoporosis in these patients with the healthy ones. METHODS: The study consisted of 40 patients with pemphigus vulgaris and 34 healthy controls. Bone mineral density measurements were obtained by dual- energy X-ray absorptiometry. Blood serum, bone parameters, and biochemical hormonal measurements were examined in both groups. RESULTS: When the bone mineral density values of patients with pemphigus vulgaris were compared with those of the control group, there was no significant difference between hip bone mineral density values, while lumbar region T and Z scores were found significantly low in the patient group (p = 0.034 and p = 0.006, respectively). Osteoporosis, osteopenia, and normal dual-energy X-ray absorptiometry rates in the patient group were found to be 32.5%, 32.5%, and 35%, respectively. These rates were found to be 18%, 23%, and 59% in control group, respectively. There were more fractures in the patient group and the difference was statistically significant (p = 0.004). CONCLUSION: An increase in osteoporosis frequency and secondary fracture to osteoporosis in the patients with pemphigus vulgaris was detected.

Pemphigus in India.

Pemphigus is a chronic epidermal immunobullous disease with potentially fatal outcome. The journey of literature on pemphigus in India has come a long way in last five decades. Pemphigus in Indian patients has unique genetic, clinical, and epidemiological differences from those in the Western countries. Corticosteroids remain the mainstay of treatment for pemphigus. Dexamethasone-cyclophosphamide pulse therapy has revolutionized the management of pemphigus in India and abroad for nearly 3 decades now. Corticosteroid-based treatment, along with adjuvants, has significantly brought down the high mortality rates that had been observed in precorticosteroid era. Present day research is largely based on elucidating the pathogenesis beyond the antidesmoglein antibodies, and newer diagnostic and treatment approaches. In this article, we review various aspects of literature on pemphigus in India, on Indians abroad, or literature from other countries that are considered relevant to the topic.

Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: a systematic review.

BACKGROUND: Pemphigus, bullous pemphigoid, and epidermolysis bullosa acquisita are autoimmune diseases of skin associated with considerable morbidity and sometimes mortality. There is no cure for these diseases. AIMS: To summarize evidence-based treatments for these diseases by performing a systematic review. METHODS: The research protocol included the following steps: identification of databases to be searched, defining search strategy, searching the databases for references, first-stage screening of the abstracts, second-stage screening of full texts of articles identified after the first-stage screening, data extraction from the identified articles after second-stage screening, quality appraisal of the studies using the Delphi list, and summarizing the findings. RESULTS: No randomized controlled trials of interventions in pemphigus vegetans, pemphigus erythematosus, and epidermolysis bullosa acquisita could be found. After the second-stage screening, 12 randomized controlled trials were analyzed, which included patients with pemphigus vulgaris or pemphigus vulgaris and pemphigus foliaceus, and 7 which included patients with bullous pemphigoid. CONCLUSIONS: Number of high-quality randomized controlled trials conducted on pemphigus and bullous pemphigoid is small. Oral corticosteroid along with a steroid-sparing agent appears to be the most effective treatment for pemphigus. Azathioprine may be most effective as a steroid-sparing agent. Topical corticosteroid therapy (as studied) is effective for bullous pemphigoid and appears to be superior to oral corticosteroid for extensive disease. Some suggestions about future research are made.

Associação terapêutica de glicocorticoide e ácidos graxos w-3 em doentes com pênfigo. Estudo metabólico-nutricional / Therapeutic association of glucocorticoid and w-3 PUFA in pemphigus patients. Metabolic and nutritional status

Para verificar o efeito da associacao terapeutica de glicocorticoide e acidos graxos w-3 (AGw-3) de oleo de peixe sobre indicadores clinicos, metabolicos e nutricionais de doentes com diagnostico de Penfigo, foram estudados 17 doentes de ambos os sexos (17 a 67 anos). Formaram-se 2 grupos: GI (glicocorticoide) e GII(glicocorticoide + AGw-3) em estudos retrospectivo e prospectivo, respectivamente. As doses de prednisona variaram de 5 a 60mg/d e a dose de AGw-3 foi de 2,1g/d. Foram colhidas amostras de sangue a cada 4 semanas no decorrer de 3 meses, para dosagens bioquimicas e de imunofluorescencia indireta (IFI), alem das avaliacoes clinica e antropometrica. A amostra estudada foi predominantemente masclina, na 4 decada de vida, de ocupacao agricola com residencia urbana no centro-oeste paulista.

Um método terapêutico adotado no tratamento do pênfigo foliáceo / A therapeutic method adopted in the treatment of pemphigus foliaceus

Une mêthode thérapeutique adoptée dans le traitement du pemphigus foliacé. L'auteur, médecin du Ministére de la Santé du Brésil se trouvait à la tête de la Délégation Fédérale de Santé de la 8éme Région (États du Mato Grosso et Goías) lorsqu'il eut l'occasion de vérifier une méthode thé-rapeutique adoptée pour le traitement du Pemphigus Foliacé. A l'hôpital Mato-grossense du Pemphigus à Campo Grande (sud de l'État du Mato Grosso) instituti-on qui appartient à "l'Union Sud-Brésilienne de l'Eglise Adventiste du 7éme Jo-ur", il observa quelques centaines de malades pemphiguex, soumis à une thérapeu-tique empirique: le corps entier du malade est enduit d'une pâte de brai de gou-dron dissout dans l'huile de cacahuéte ou de coton, associé à d'autres ingrédi-ents. Le grand intérêt de l'auter pour ce traitement se justifiait bien, de-vant l'ignorance de la Science Médicale au sujet du Pemphigus Foliacé pour le-quel on multiplie, encore aujourd'hui, des recherches sur son épidemiologie, sa thérapeutique et sa prophylaxie