Review of modern techniques and analysis of patient outcomes with benign familial pemphigus Gougereau–Haley–Haley
Benign familial pemphigus (BFP) Gougereau–Haley–Haley is a rare autosomal dominant genodermatosis caused by mutations of the ATP2C1 gene responsible for the function of calcium-dependent ATPases regulating calcium sequestration in the Golgi apparatus. The article provides an overview of modern metho...
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| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
State Scientific Center of Dermatovenereology and Cosmetology
2024-12-01
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| Series: | Vestnik Dermatologii i Venerologii |
| Subjects: | |
| Online Access: | https://vestnikdv.ru/jour/article/viewFile/16783/pdf |
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| Summary: | Benign familial pemphigus (BFP) Gougereau–Haley–Haley is a rare autosomal dominant genodermatosis caused by mutations of the ATP2C1 gene responsible for the function of calcium-dependent ATPases regulating calcium sequestration in the Golgi apparatus. The article provides an overview of modern methods of treatment of various groups of drugs, including drugs for topical treatment (topical glucocorticosteroids, calcipotriol, combined drugs), systemic drugs (glucocorticosteroids, apremilast), injection techniques (intradermal administration of botulinum toxin) currently used.
Taking into account the lack of clinical recommendations for the treatment of patients with benign familial pemphigus, to evaluate the effectiveness of treatment methods described in modern publications.
The observational study included 4 patients with clinically and histologically verified benign familial pemphigus, and described the results of therapy using techniques using intradermal administration of botulinum toxin A (3 patients) and apremilast (1 patient). The results of observations of patients with BFP and photo documentation are presented.
BFP is a rare dermatosis, the description of treatment methods for which is scarce in various publications, large-scale randomized studies are needed to determine the comparative effectiveness and safety of various drugs and physiotherapy techniques for the treatment of this disease. The material presented in the article will help practitioners to offer patients possible methods of BFP therapy and take into account the experience of colleagues. |
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| ISSN: | 0042-4609 2313-6294 |