Hidradenitis suppurativa (HS) is a chronic inflammatory disease that is frequently associated with syndromes, such as those within the PAPA spectrum. Syndromic HS presents unique management challenges, as it often shows resistance to conventional therapies. Pyoderma gangrenosum is a rare inflammatory neutrophilic dermatosis that is often seen in association within the spectrum of autoinflammatory diseases. The PAPA spectrum disorders include a group of autoinflammatory diseases characterized by mutations in the PSTPIP1 gene or by clinical manifestations that closely resemble or overlap with those of PAPA syndrome. Each syndrome (PASH, PAPASH, PsAPASH, PASS, PAC, and PAMI syndrome) in this spectrum highlights specific inflammatory pathways and symptoms, providing insight into targeted therapeutic approaches. Here, we present a rare case of incomplete PASH (pyoderma gangrenosum and hidradenitis suppurativa) syndrome successfully managed with a standard combination of antibiotics (ceftriaxone and metronidazole) and corticosteroids (methylprednisolone), followed by immunosuppressant (azathioprine) and corticosteroids (dexamethasone). We review both novel and established/standard treatment options, with an emphasis on treatment outcomes. Conventional therapies remain both effective and affordable, providing valuable alternatives for patients.

• Keywords
• PAPA syndrome, PASH syndrome, antibiotics, azathioprine, corticosteroids, hidradenitis suppurativa, pyoderma gangrenosum,
• MeSH
• Anti-Bacterial Agents therapeutic use   MeSH
• Hidradenitis Suppurativa * drug therapy   diagnosis   MeSH
• Immunosuppressive Agents therapeutic use   MeSH
• Middle Aged MeSH
• Humans MeSH
• Pyoderma Gangrenosum * drug therapy   diagnosis   MeSH
• Syndrome MeSH
• Check Tag
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH
• Review MeSH
• Names of Substances
• Anti-Bacterial Agents MeSH
• Immunosuppressive Agents MeSH

UNLABELLED: Gangrenous pyoderma is a rare complication of surgical treatment mimicking bacterial necrotizing fasciitis. The characteristic symptoms include a pre-existing immune disorder, negative results of the relevant microbiological examinations, no response to any antibiotic therapy, and deteriorating of the condition after radical debridement. Another typical feature is a discrepancy between an ominous appearance of the wound accompanied by leucocytosis and an elevated CRP level, and the patient s good general condition with low-grade fever and no signs of sepsis. The case of a 68-year-old man who underwent an elective open reconstruction of rotator cuff tears of the left shoulder is presented. The surgery was complicated with pyoderma gangrenosum that was first treated unsuccessfully by repeated revision surgery and broad-spectrum antibiotic therapy. The patient was ultimately cured with corticosteroids. The sequellae included limited motion of the shoulder joint and extensive destruction and scarring of the adjacent soft tissue. KEY WORDS: gangrenous pyoderma, necrotizing fasciitis, corticosteroids.

• MeSH
• Diagnosis, Differential MeSH
• Fasciitis, Necrotizing diagnosis   MeSH
• Adrenal Cortex Hormones therapeutic use   MeSH
• Humans MeSH
• Rotator Cuff Injuries surgery   MeSH
• Pyoderma Gangrenosum diagnosis   drug therapy   etiology   MeSH
• Aged MeSH
• Treatment Outcome MeSH
• Plastic Surgery Procedures adverse effects   methods   MeSH
• Check Tag
• Humans MeSH
• Male MeSH
• Aged MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH
• Names of Substances
• Adrenal Cortex Hormones MeSH

Pyoderma gangrenosum (PG) is rather rare chronic dermatosis presenting as cutaneous necrosis followed by development of expanding chronic ulcer with well-demarcated, undermined, violaceous borders. Diagnosis of PG is predominantly based on the clinical presentation and course. Histopathologic findings may be non-specific. Half of patients suffer from a somatic systemic disease. We describe 92-year-old lady presenting with large non-healing ulcer of the anterolateral calf with the diagnosis of atypical PG. In a discussion differential diagnosis of cutaneous ulcers mimicking PG is reviewed and the treatment possibilities summarised.

• MeSH
• Diagnosis, Differential MeSH
• Humans MeSH
• Pyoderma Gangrenosum * diagnosis   pathology   therapy   MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Check Tag
• Humans MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Female MeSH
• Publication type
• English Abstract MeSH
• Journal Article MeSH
• Case Reports MeSH

A 29-year-old male patient with the anamnesis of inflammatory bowel disease and Grave-Basedowov disease was hospitalized because of rapidly spreading skin defect with affected muscle on the left shin. This skin defect appeared after the significant decreasing of corticoids. The small skin trauma preceded the pyoderma gangrenosum. First the skin disease was not right diagnosed and patient was cured by the excision of the defect. It caused tissue disintegration, muscle necrosis and extension of the defect. The whole leg was endangered. Patient was cured with corticoids and cyclosporin A after the right diagnosis. The defect healed and laboratory inflammatory markers decreased. The immunosuppresive therapy was changed to azathioprin, the corticoid therapy was interrupted. After three months the defect was healed.

• MeSH
• Adult MeSH
• Graves Disease complications   MeSH
• Humans MeSH
• Pyoderma Gangrenosum complications   diagnosis   therapy   MeSH
• Colitis, Ulcerative complications   MeSH
• Leg Ulcer complications   diagnosis   therapy   MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Male MeSH
• Publication type
• English Abstract MeSH
• Journal Article MeSH
• Case Reports MeSH