The International Day of Persons with Disabilities is celebrated worldwide every year on December 3rd, in accordance with Resolution 47/3 of the General Assembly dated October 14, 1992. Its purpose is to promote the rights of persons with disabilities in all areas of social life and to raise public awareness of their issues.

On December 3rd, special events are held around the world to remind people of the many challenges faced by individuals with disabilities. However, the main goal of this holiday is not to evoke pity for these people, but rather to remind everyone that persons with disabilities are equal to all other members of society. At the same time, it is important to remember that people with limited abilities require social protection and assistance.

On December 3rd, special events are held around the world to remind people of the many issues faced by individuals with disabilities. However, the main goal of this day is not to evoke pity for these people, but rather to remind everyone that persons with disabilities are equal to all other members of society. At the same time, it is important to remember that people with limited abilities require social protection and assistance.

Congenital bullous epidermolysis (CBE) is a group of genetically and clinically heterogeneous diseases characterized by the formation of blisters and erosions on the skin and mucous membranes as a result of minor mechanical trauma or spontaneously. The severity of this genodermatosis depends on its clinical form, ranging from mild CBE to a disabling, recessive, dystrophic form of CBE.

How CBE is related to disability:

Increased susceptibility to injury: Any mechanical impact on the skin and mucous membranes causes blistering and painful wounds.
Severe healing issues: The wounds become infected and heal poorly, requiring constant and long-term care.
Limitations in daily life: These symptoms seriously restrict everyday activities, leading to the development of disability.

This day has special significance:

Raising awareness: Events held on December 3rd help spread information about this rare (orphan) genetic disease, which often leads to disability due to the constant formation of blisters and wounds on the skin and mucous membranes.
Drawing attention to issues: On this day, the challenges faced by people with CBE are highlighted, including the need for expensive specialized dressings and medications, the necessity of symptomatic therapy, and social adaptation.
Social support: The date serves as a reminder of the importance of ensuring a decent quality of life, access to high-quality medical care, education, and employment opportunities for people with CBE and other forms of disability.

In addition to the International Day of Persons with Disabilities, there is also the International Bullous Epidermolysis Week, which is held annually from October 25 to 31. This week focuses exclusively on "butterfly children" (a term used for people with CBE due to the fragility of their skin) and aims to raise awareness specifically about this disease worldwide.

Both of these days play an important role in mobilizing support and resources for people with congenital bullous epidermolysis.
The presence of long-lasting non-healing erosions leads to a significant decline in quality of life, both in childhood and adulthood. For a person suffering from CBE, the most important thing is to be able to rely on themselves and to be independent. Most patients want to have their own home, a job, a partner, and a family. Desires vary, and achieving them often requires overcoming numerous obstacles.

Improving early diagnosis and treatment of genetic diseases, as well as access to prenatal diagnostics, means that more women are reaching reproductive age and can make informed choices regarding pregnancy and childbirth. Despite these advancements, the management of pregnancy, delivery, and subsequent care for CBE is rarely addressed in accessible literature.

CBE includes a group of rare, heterogeneous, hereditary diseases characterized by skin and mucous membrane instability. In response to friction or mechanical trauma, the layers of skin peel apart, leading to the formation of blisters and erosions on the skin and mucous membranes. There are four main forms of the dermatosis, based on the level of ultrastructural skin separation: simple (UPBE), junctional (JPBE), dystrophic (DPBE), and Kindler syndrome (KS).

Patients with CBE exhibit varying severity of the disease both within and between subtypes. For example, patients with the simple form of CBE may experience limited impact of the disease on their daily lives and have a normal life expectancy, while patients with severe recessive dystrophic CBE face significant morbidity and life-limiting complications.

Thus, CBE is a rare disease that presents challenges for conducting research. Familiarity with the provided information will help to: improve the effectiveness and quality of pregnancy and childbirth management in women of reproductive age with CBE; reduce disparities in clinical practice on an international level; and highlight initiatives aimed at improving quality related to clinical practice guidelines.

The existing clinical heterogeneity among subtypes limits the ability to generalize recommendations for different patient groups. Most of the available literature pertains to prenatal diagnosis of severe forms and the management of newborns with CBE. Specific guidelines for antenatal, intranatal, and postpartum care have not been comprehensively studied. Accumulating factual data and interpreting it will help improve the quality of pregnancy preservation, childbirth management, and postpartum care in women affected by CBE.

Aliyev Kh.I.-Associate Professor of the Department of Dermatovenerology named after Professor Zoirov P.T.