Pemphigus Benign Familial Chronic

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Benign familial chronic pemphigus is a rare self-limiting skin disease from the group of epidermolysis bullosa.

During the chronic course of the disease, vascular damage, loss of elastic fibers, swelling and inflammation, as well as epitheliolysis occur. Mesenchymal tissues also undergo changes, while the epidermis remains intact, and ciliary damage to the hair follicles and nail plates is not observed. As a rule, a bilateral symmetrical process occurs. Patients develop elements, usually multiple, sometimes merging, with a diameter of several millimeters to several centimeters. They are localized mainly on the lower extremities, especially in the joint area, and on the forearms, mainly on the back side. A polymorphic vesicular-bullous rash is observed. The vesicles are large, up to 5–10 mm, with transparent contents and a tense cap; the elements of the rash tend to merge; erosions covered with a crust may appear along the periphery. The color of the elements is yellowish, pale pink or white and differs from the color of the skin. There are no reasons for adding fresh elements. At least 20% of patients may have a family history of the disease (usually close relatives show signs of hereditary transmission of the disease). Men get sick more often than women. Changes in the umbilical area and palms may indicate the possibility of developing benign familial chronic pemphigus. However, they are more often premorbid signs. Detection of these elements should prompt a skin biopsy if present. There are no pronounced changes in the internal organs. Complications are rare but can be fatal.