pash syndrome - Health Topics
Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) syndrome is a rare condition characterized by clinical features of all three dermatologic conditions. The management of PASH syndrome is difficult, with no consensus on treatment guidelines. We review the classification, clinical presentation, disease associations, and therapeutic management of syndromic HS, focusing mainly on its autoinflammatory syndromes PASH, PAPASH, PsAPASH, and PASS.
Understanding the Context
PASH syndrome, a rare condition, can present with symptoms such as palpable breast lumps, skin changes like redness or warmth over the affected area, and pain. Patients may also experience breast swelling, inflammation, and tenderness. We report a new case of PASH syndrome, highlighting the features of the rare condition. For a better understanding of each of the three diseases and of the PASH syndrome, we reviewed the common and the particular aspects of these pathologies, looking from different angles of inflammation mechanisms.
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Key Insights
PASH syndrome is a recently identified hereditary autoinflammatory syndrome consisting of multiple neutrophilic dermatoses including: pyoderma gangrenosum (PG), acne, and hidradenitis suppurativa (HS). The triad of PG, acne, and HS occurs in extremely rare instances. In this review, we described a case of PASH syndrome in a patient who initially failed to respond to immunosuppressive treatment but responded to a combination of colchicine and thalidomide. We reviewed the relevant literature that focuses on PASH syndrome management. The clinical course of PASH syndrome varies, but most individuals typically present with recurrent and episodic severe, nodulocystic, scarring acne and HS lesions during adolescence.
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PASH syndrome, a rare neutrophilic dermatosis linked to autoinflammatory conditions like Braun Flaco, is characterized by Pyoderma gangrenosum, acne, and suppurative hidradenitis.