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.
- Klíčová slova
- PAPA syndrome, PASH syndrome, antibiotics, azathioprine, corticosteroids, hidradenitis suppurativa, pyoderma gangrenosum,
- MeSH
- antibakteriální látky terapeutické užití MeSH
- hidradenitis suppurativa * farmakoterapie diagnóza MeSH
- imunosupresiva terapeutické užití MeSH
- lidé středního věku MeSH
- lidé MeSH
- pyoderma gangrenosum * farmakoterapie diagnóza MeSH
- syndrom MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
- Názvy látek
- antibakteriální látky MeSH
- imunosupresiva MeSH
BACKGROUND: Pyoderma gangrenosum (PG) is a rare non-infectious inflammatory disease of unknown etiology that affects the skin and mucous membranes. The occurrence of pyoderma gangrenosum after a reduction mammoplasty is a very rare complication, which at first glance may seem like an infectious complication; in reality, however, it is an inflammatory disease. CASE: This case report describes a rare postoperative complication - pyoderma gangrenosum and its appropriate treatment in our patient who underwent reduction mammoplasty. CONCLUSION: Early detection of this complication is essential for the patients recovery because the primary surgical treatment can lead to worsening of the condition and is therefore contraindicated in such a case. Pyoderma gangrenosum must be treated conservatively with corticosteroids.
- Klíčová slova
- complications, pyoderma, pyoderma gangrenosum, reduction mammoplasty,
- MeSH
- lidé MeSH
- mamoplastika * škodlivé účinky MeSH
- pooperační komplikace etiologie MeSH
- pyoderma gangrenosum * etiologie MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky 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
- diferenciální diagnóza MeSH
- fasciitida nekrotizující diagnóza MeSH
- hormony kůry nadledvin terapeutické užití MeSH
- lidé MeSH
- poranění rotátorové manžety chirurgie MeSH
- pyoderma gangrenosum diagnóza farmakoterapie etiologie MeSH
- senioři MeSH
- výsledek terapie MeSH
- zákroky plastické chirurgie škodlivé účinky metody MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- hormony kůry nadledvin MeSH
The authors present a case of 61-year-old man with ulcerative colitis and with extraintestinal manifestation of the disease in the form of pyoderma gangraenosum. Multiple skin defects, which developed in atypical localisation (extensive affection of facial and hairy parts of the head) in patient with chronically active form of ulcerative colitis were complicated with bacterial contamination of methicilin-resistant strains of Staphylococcus aureus. After application of the parenteral feeding, corticotherapy and targeted antibiotic therapy the subjective and objective status of the patient markedly improved, stool frequency was reduced, admixture of blood in the stool disappeared, temperatures fell back and there was a decrease in activity of non-specific bowel inflammation in laboratory findings. However endoscopic examination of the intestine confirmed the finding of chronically active ulcerative colitis with ulcerations and bridging polyps. Patient was indicated to total colectomy, but he refused it.
- MeSH
- dermatózy skalpu patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- obličejové dermatózy patologie MeSH
- pyoderma gangrenosum komplikace patologie MeSH
- ulcerózní kolitida komplikace MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
An unusual clinical appearance and course of pyoderma gangraenosum (PG) in a 35-year-old woman is presented. Signs of both the ulcerative and vegetative forms of PG were expressed. The association of two systemic diseases, the autoimmune thyreopathy and the hyperandrogenic syndrome were observed in a female. The recommended conventional therapy for PG: corticosteroids, antibiotics, cyclosporine and cyclophosphamide yielded a poor response, whereas after thyroidectomy and reaching an euthyroid state the symptoms receded. This close association of PG and autoimmune thyreopathy supports the autoimmune concept of PG.
- MeSH
- antibakteriální látky terapeutické užití MeSH
- bakteriální infekce komplikace farmakoterapie MeSH
- dospělí MeSH
- Gravesova nemoc komplikace MeSH
- hyperandrogenismus komplikace MeSH
- imunosupresiva terapeutické užití MeSH
- karbimazol terapeutické užití MeSH
- lidé MeSH
- pyoderma gangrenosum farmakoterapie etiologie chirurgie MeSH
- thyreostatika terapeutické užití MeSH
- tyreoidektomie MeSH
- výsledek terapie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- antibakteriální látky MeSH
- imunosupresiva MeSH
- karbimazol MeSH
- thyreostatika 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.
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
- dospělí MeSH
- Gravesova nemoc komplikace MeSH
- lidé MeSH
- pyoderma gangrenosum komplikace diagnóza terapie MeSH
- ulcerózní kolitida komplikace MeSH
- vředy dolních končetin komplikace diagnóza terapie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
Pyoderma gangrenosum is a serious necrotizing skin disease frequently associated with diseases of internal organs. In rare instances it may develop after surgical operations or minor injuries causing traumatization of the skin. Our patient developed the disease immediately after cholecystectomy at the site of the scar on the abdomen, and disseminated foci developed also at other sites of the body. In the second patient it developed in the region of the stoma soon after its establishment during development of ulcerative colitis. Skin manifestations healed after systemic combined immunosuppressive treatment.
- MeSH
- cholecystektomie škodlivé účinky MeSH
- dospělí MeSH
- ileostomie škodlivé účinky MeSH
- lidé středního věku MeSH
- lidé MeSH
- pyoderma gangrenosum etiologie patologie terapie MeSH
- ulcerózní kolitida chirurgie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
