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
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.
- Keywords
- complications, pyoderma, pyoderma gangrenosum, reduction mammoplasty,
- MeSH
- Humans MeSH
- Mammaplasty * adverse effects MeSH
- Postoperative Complications etiology MeSH
- Pyoderma Gangrenosum * etiology MeSH
- Check Tag
- Humans MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Case Reports 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
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
- Scalp Dermatoses pathology MeSH
- Middle Aged MeSH
- Humans MeSH
- Facial Dermatoses pathology MeSH
- Pyoderma Gangrenosum complications pathology MeSH
- Colitis, Ulcerative complications MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Publication type
- English Abstract MeSH
- Journal Article MeSH
- Case Reports 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
- Anti-Bacterial Agents therapeutic use MeSH
- Bacterial Infections complications drug therapy MeSH
- Adult MeSH
- Graves Disease complications MeSH
- Hyperandrogenism complications MeSH
- Immunosuppressive Agents therapeutic use MeSH
- Carbimazole therapeutic use MeSH
- Humans MeSH
- Pyoderma Gangrenosum drug therapy etiology surgery MeSH
- Antithyroid Agents therapeutic use MeSH
- Thyroidectomy MeSH
- Treatment Outcome MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Names of Substances
- Anti-Bacterial Agents MeSH
- Immunosuppressive Agents MeSH
- Carbimazole MeSH
- Antithyroid Agents 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
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
- Cholecystectomy adverse effects MeSH
- Adult MeSH
- Ileostomy adverse effects MeSH
- Middle Aged MeSH
- Humans MeSH
- Pyoderma Gangrenosum etiology pathology therapy MeSH
- Colitis, Ulcerative surgery MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Female MeSH
- Publication type
- English Abstract MeSH
- Journal Article MeSH
- Case Reports MeSH
The occurrence of decreased activity of the immune system was studied in a group of 66 dogs with various combinations of pyoderma and demodicosis. Our complex examination of the dogs included the following: leukocyte count, differential count, phagocytosis, blastogenic lymphocyte transformation and quantitation of total serum immunoglobulins, lysozyme and haemolytic complement. Immunosuppression was found in 19 (28.8%) cases. Immunosuppression was rare in dogs with demodicosis and did not appear without a concurrent pyoderma. An increase in the neutrophil counts and total serum immunoglobulin levels significant was found in dogs with demodicosis combined with pyoderma. On the contrary, marked immunosuppression was detected in dogs with deep pyoderma. A considerable immunosuppression was present in 7 of 10 German shepherds dog pyoderma (GSP). Significant depressions were found in phagocyte activity and lymphocyte activity. Immunosuppression was observed in 4 of 9 dogs in other breeds with uncomplicated deep pyoderma. All groups of dogs with pyoderma showed a significant increase in total serum immunoglobulins. Conclusion from these findings is that deep pyoderma more than Demodicosis was concerned with immunosuppression. German shepherds with deep pyoderma had more expressed immunosuppression than other breeds.
- MeSH
- Immune Tolerance MeSH
- Mite Infestations immunology veterinary MeSH
- Dog Diseases immunology MeSH
- Dogs MeSH
- Pyoderma immunology veterinary MeSH
- Animals MeSH
- Check Tag
- Dogs MeSH
- Animals MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
Perianal pyoderma (hidradenitis suppurativa-acne conglobata) is a chronic disease which in its progressive form cannot be treated by conservative methods. Extensive suppurative foci must be radically excised incl. all systems of fistulas right down to the fascia and the defects which develop are allowed to heal per secundam or after several weeks they can at a different period of time be covered by a skin transplant. The authors present their 30-year experience with surgical treatment of 39 patients. Treatment was in all instances successful after radical surgical excision. All these patients have fully recovered as regards work capacity as well as social aspects.
- MeSH
- Chronic Disease MeSH
- Adult MeSH
- Hidradenitis Suppurativa diagnosis surgery MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Anus Diseases diagnosis surgery MeSH
- Pyoderma diagnosis surgery MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Male MeSH
- Female MeSH
- Publication type
- English Abstract MeSH
- Journal Article MeSH