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”.
