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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| Format: | Article |
| Language: | English |
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State Scientific Center of Dermatovenereology and Cosmetology
2024-12-01
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| Series: | Vestnik Dermatologii i Venerologii |
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| Online Access: | https://vestnikdv.ru/jour/article/viewFile/16783/pdf |
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| author | Marianna B. Drozhdina Sergey V. Koshkin |
| author_facet | Marianna B. Drozhdina Sergey V. Koshkin |
| author_sort | Marianna B. Drozhdina |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-e1ab902bca8d4a60b11c81d1cd530bb9 |
| institution | Kabale University |
| issn | 0042-4609 2313-6294 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | State Scientific Center of Dermatovenereology and Cosmetology |
| record_format | Article |
| series | Vestnik Dermatologii i Venerologii |
| spelling | doaj-art-e1ab902bca8d4a60b11c81d1cd530bb92025-02-05T20:41:44ZengState Scientific Center of Dermatovenereology and CosmetologyVestnik Dermatologii i Venerologii0042-46092313-62942024-12-011006708010.25208/vdv167831315Review of modern techniques and analysis of patient outcomes with benign familial pemphigus Gougereau–Haley–HaleyMarianna B. Drozhdina0https://orcid.org/0000-0002-7689-8350Sergey V. Koshkin1https://orcid.org/0000-0002-6220-8304Kirov State Medical UniversityKirov State Medical UniversityBenign 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.https://vestnikdv.ru/jour/article/viewFile/16783/pdfbenign familial pemphigusglucocorticosteroidsnaltrexoneapremilastbotulinum toxin |
| spellingShingle | Marianna B. Drozhdina Sergey V. Koshkin Review of modern techniques and analysis of patient outcomes with benign familial pemphigus Gougereau–Haley–Haley Vestnik Dermatologii i Venerologii benign familial pemphigus glucocorticosteroids naltrexone apremilast botulinum toxin |
| title | Review of modern techniques and analysis of patient outcomes with benign familial pemphigus Gougereau–Haley–Haley |
| title_full | Review of modern techniques and analysis of patient outcomes with benign familial pemphigus Gougereau–Haley–Haley |
| title_fullStr | Review of modern techniques and analysis of patient outcomes with benign familial pemphigus Gougereau–Haley–Haley |
| title_full_unstemmed | Review of modern techniques and analysis of patient outcomes with benign familial pemphigus Gougereau–Haley–Haley |
| title_short | Review of modern techniques and analysis of patient outcomes with benign familial pemphigus Gougereau–Haley–Haley |
| title_sort | review of modern techniques and analysis of patient outcomes with benign familial pemphigus gougereau haley haley |
| topic | benign familial pemphigus glucocorticosteroids naltrexone apremilast botulinum toxin |
| url | https://vestnikdv.ru/jour/article/viewFile/16783/pdf |
| work_keys_str_mv | AT mariannabdrozhdina reviewofmoderntechniquesandanalysisofpatientoutcomeswithbenignfamilialpemphigusgougereauhaleyhaley AT sergeyvkoshkin reviewofmoderntechniquesandanalysisofpatientoutcomeswithbenignfamilialpemphigusgougereauhaleyhaley |