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P(A)SH Syndrome: Case Presentation and Short Update of Related Disorders.
Simona Kordeva1, Alice Hristova2, Valentina Broshtilova3
1Onkoderma - Clinic for Dermatology, Venereology and Dermatologic Surgery, Sofia, Bulgaria. simonakordeva97@gmail.com.
Acta Medica (Hradec Kralove)
|April 3, 2025
Summary
Syndromic hidradenitis suppurativa (HS), like PASH syndrome, can be challenging to treat. This case highlights successful management using conventional antibiotics and corticosteroids, demonstrating their continued efficacy.
Area of Science:
- Dermatology and Immunology
- Autoinflammatory Diseases
- Syndromic Hidradenitis Suppurativa
Background:
- Hidradenitis suppurativa (HS) is a chronic inflammatory condition often linked to autoinflammatory syndromes.
- Syndromic HS, including PASH (Pyoderma Gangrenosum, Acne, and HS), presents treatment resistance to standard therapies.
- PAPA spectrum disorders involve genetic mutations (PSTPIP1) or overlapping clinical features of autoinflammatory conditions.
Purpose of the Study:
- To report a rare case of incomplete PASH syndrome.
- To evaluate the management of syndromic HS with conventional treatments.
- To review established and novel therapeutic strategies for syndromic HS.
Main Methods:
- A case presentation of incomplete PASH syndrome.
- Treatment involved a combination of intravenous antibiotics (ceftriaxone, metronidazole) and corticosteroids (methylprednisolone).
- Subsequent treatment included an immunosuppressant (azathioprine) and corticosteroids (dexamethasone).
Main Results:
- Successful management of incomplete PASH syndrome was achieved with the described treatment regimen.
- Conventional therapies demonstrated effectiveness in this challenging case of syndromic HS.
- Review of treatment outcomes emphasizes the value of established therapeutic options.
Conclusions:
- Conventional antibiotic and corticosteroid combinations can effectively manage challenging cases of syndromic HS, such as PASH syndrome.
- Established treatments remain valuable and affordable alternatives for patients with syndromic HS.
- Understanding specific inflammatory pathways in PAPA spectrum disorders aids in targeted therapeutic approaches.
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