Epidermolizele buloase: aspecte clinico-evolutive şi de tratament

Abstract

41 cases of epidermolysis bullosa (EB), simple, jonctional and dystrophic types, were analyzed in this article. It is showed a predominance of male patients originated from rural regions as well as high incidence of dominant type of disease. Bullous manifestaions of EB in about 1/3 of cases were complicated with nail involvement, also hyperhidrosis, palmo-plantar keratoderma, alopecia and ichthyosiform lesions were seen occasionaly. Associated pathology as anemia, pneumonia, hepatitis, pancreatitis, pielonephritis were established in a half of patients. Common signs as dental disorders and mucous membrane involvement were reported in 1/3 of patients, every 10th patient had a mental retardation. 20% of patients with EB have developed complications as secondary pyodermas, acrosclerodactilitis, mutilations, etc. It is mentioned a moderate eficiency of the standard treatment, abcense of prenatal diagnostic, crucial role of sustained administration of non-adherent bandages, and the need of colaboration with patients’ support organisations as „DEBRA-Moldova”.

În articol sunt analizate 41 cazuri de epidermolizele buloase simple, joncţionale şi distrofice. Se constată predominanţa pacienţilor de sex masculin, din mediul rural, precum şi a formelor clinice de EB dominante. Manifestările buloase de epidermoliză au fost completate în o treime din cazuri cu afectare unghială, iar hiperhidroza, keratodermia palmo-plantară, alopecia şi leziunile ichtioziforme au fost semnalate sporadic. La circa o jumătate din pacienţi au fost constatate o serie de maladii concomitente (anemii, pneumonii, hepatite, pancreatite, pielonefrite). Comune au fost anomaliile dentare şi afectarea mucoaselor raportate la o treime din pacienţi, fiecare al zecelea pacient având şi retard mental. La circa 20% bolnavi cu epidermolize buloase au fost observate complicaţii cu agenţi microbieni, acrosclerodactilie, contracturi, mutilaţii, stenoze esofagiene, precum şi un caz de deces. Se accentuieză eficacitatea modestă a tratamentelor de rutină, lipsa diagnosticului prenatal, imperativul de administrare continuă a pansamentelor non-aderente, precum şi importanţa conlucrării cu organizaţiile de suport al pacienţilor cu epidermolize buloase de tip „DEBRA-Moldova”.