Tikhonovskaya I.V.*, Lesnichaya O.V.*, Pushkarskaya I.A.**
Junctional localized epidermolysis bullosa: a case of late diagnosis
*Education Establishment "Vitebsk State Order of Peoples’ Friendship Medical University", Republic of Belarus
**Healthcare Establishment "Vitebsk regional dermatovenerologic dispensary", Republic of Belarus
Abstract.
A patient from the mycological department of the Vitebsk regional clinical dermatovenerologic dispensary was observed. The onset of the disease occurred in his childhood. Similar eruptions were observed in his mother’s once removed grandfather. The patient has two dermatologically healthy daughters. His dermatologic status is as follows: on the skin of the hands, legs isolated bullae, recovery erosions, superficial atrophy foci, hyperpigmentation are determined. On the IV finger of the left hand the bulla with hemorrhagic content is seen. This bulla appeared after the application of a cotton ball in place of blood sampling. Nails and hair are not changed. Urine levels of porphyrins were negative. His general health is good. Pathomorphologic data: subepidermal blisters with hemorrhagic or fibrin contents are seen. The bland perivascular infiltrate in the dermis was found. The inflammatory infiltration is represented mainly by lymphocytes and fibroblasts. Considering the medical history, clinical manifestations, pathomorphologic data the diagnosis of junctional localized epidermolysis bullosa was made. Light, immunofluorescence and electron microscopy are necessary for the diagnosis and differential diagnosis of various forms of epidermolysis bullosa and bullous skin diseases.
Key words: epidermolysis bullosa, pathomorphology, diagnosis.
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