Introduction Pyogenic sterile Arthritis Pyoderma gangrenosum and Acne (PAPA) syndrome is a rare autosomal dominant inherited autoinflammatory disease caused by mutations in Proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1). In childhood, the syndrome is featured by recurrent sterile, erosive arthritis, potentially leading to joint destruction. By puberty, cutaneous symptoms b...

We present a female patient observed with painful violaceous plaques with central bullae and pustules on the lower limbs, rapidly transformed into ulcers, associated with bloody diarrhea, recurrent oral erosions and hyperthermia in the previous 3 months. Cutaneous biopsy was consistent with pyoderma gangrenosum, and intestinal diagnostic procedures revealed a non-classifiable inflammatory bowel...

Pyoderma gangrenosum, cystic acne, and aseptic arthritis are clinically distinct disorders within the broad class of inflammatory diseases. Although this triad of symptoms is rarely observed in a single patient, a three-generation kindred with autosomal-dominant transmission of these three disorders has been reported as "PAPA syndrome" (MIM 604416). We report mapping of a disease locus for fami...

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis of unknown origin. Clinically it starts with a pustule, nodule or bulla that rapidly progresses and turns into a painful ulcer with raised, undermined borders. The etiopathogenesis of PG remains unknown. However it is frequently associated with systemic diseases such as inflammatory bowel disease (IBD), haematological disorders or arthritis...

Pyoderma gangrenosum may precede, coexist or follow diverse systemic diseases. The bullous variety is usually associated with hematologic disorders. From 31 patients with pyoderma gangrenosum diagnosed in our hospital during 10 years, only 2 presented with the bullous type. One patient had previous diagnosis of chronic myeloid leukemia. Both had fast-progressive, painful lesions, and both showe...

A 19 year old female with pyoderma gangrenosum was referred to the oculoplastic clinic for evaluation of severe cicatricial ectropion of the right lower eyelid. Examination evidenced important scarring of lower eyelid and malar region with secondary ectropion. A full-thickness skin graft associated with a lateral tarsal strip procedure and scar tissue release was performed. The patient's cortic...

Pyoderma gangrenosum (PG) is an uncommon, chronic, painful, ulcerative skin disease of unknown origin, often associated with systemic diseases including inflammatory bowel disease, rheumatoid arthritis; monoclonal gammopathy, hepatitis and myeloproliferative disorders. The mainstay of therapy for PG has been high-dose corticosteroids but not all patients have a favourable outcome. Other systemi...

PURPOSE To investigate de adjunctive effect of Hyperbaric Oxygen Therapy in a group of patients with refractory Crohn's disease. METHODS A total of 29 subjects with refractory Crohn's disease were submitted to daily sessions of Hyperbaric Oxygen Therapy, in a 2800 Sechrist Monoplace Hyperbaric Chamber (Sechrist, USA) pressurized to 2.4 ATA. Each session lasted 2 hours. The endpoint was closur...

Introduction Pyogenic sterile Arthritis Pyoderma gangrenosum and Acne (PAPA) syndrome is a rare autosomal dominant inherited autoinflammatory disease caused by mutations of Proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1). In childhood, the syndrome is featured by recurrent sterile, erosive arthritis, potentially leading to joint destruction. By puberty, cutaneous symptoms b...

Results: Of the 31 patients, all presented with 1 or multiple painful necrotic skin ulcers on the laterodorsal part of the leg, with bilateral involvement in 16 of 31 cases (52%), and 16 were referred with suspected pyoderma gangrenosum. All patients had arterial hypertension, and 18 (58%) had diabetes. All patients had subcutaneous stenotic arteriolosclerosis on histologic analysis, with media...