Imaging findings of sterile pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome: differential diagnosis and review of the literature.

Pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome is a rare autosomal-dominant autoinflammatory disease of incomplete penetrance and variable expression. PAPA syndrome is the result of a mutation in the proline serine threonine phosphatase-interacting protein 1 (PSTPIP1/CD2BP1) gene located on chromosome 15, which results in an abnormal overproduction of the pro-inflammatory cytokine interleukin-1ß (IL-1). This syndrome clinically manifests as early onset of recurrent episodes of acute aseptic inflammation of the joints, generally occurring in the first two decades of life, followed by manifestation of characteristic skin lesions in the third decade, after an obvious decline in the joint symptoms. Although uncommon, the potential clinical implications of PAPA syndrome warrant an appropriate diagnosis in a timely fashion.

[Pyoderma gangraenosum after implantation of a shoulder endoprothesis]. / Pyoderma gangraenosum nach Implantation einer Schulterendoprothese.

Pyoderma gangrenosum is an inflammatory, ulcerative, neutrophilic dermatosis that is clinically characterized by rapidly evolving, painful, necrotic skin lesions. It is typically associated with chronic systemic inflammatory or neoplastic diseases, but may also occur secondary to cutaneous injury or surgery (pathergy). Post-surgical pyoderma gangrenosum typically develops around surgical sites within the immediate postoperative period, mimicking early wound infection. However, common treatment strategies including antibiotics and debridement fail to improve or even worsen symptoms. Postoperative pyoderma gangrenosum has been most commonly reported from breast and visceral surgery. We present a case of postoperative pyoderma gangrenosum following total shoulder arthroplasty.

Saddle-nose and bilateral cauliflower ear deformities with pyoderma gangrenosum-like ulcers, cavitary pulmonary lesions, digital gangrene and pulselessness in a young female.

We report a young female who presented with saddle-nose and bilateral cauliflower ear deformities along with pyoderma gangrenosum-like ulcers, digital gangrene and pulselessness. Subsequently, she was found to have bilateral conductive hearing loss, a corneal opacity, mild aortic regurgitation and radiological evidence of cavitary changes in lungs and aortoarteritis. Our patient had a constellation of symptoms which posed a diagnostic challenge. Finally, a diagnosis of relapsing polychondritis with several unusual features was made. Overlap with Takayasu's arteritis and granulomatosis with polyangitis, which has been reported rarely in the literature, cannot be excluded.

Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm.

A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit.

[Pyoderma gangrenosum with lung involvement treated with infliximab]. / Pyoderma gangrenosum avec localisations pulmonaires traité par infliximab.

BACKGROUND: Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis often associated with systemic diseases, particularly chronic inflammatory bowel diseases. Extracutaneous manifestations (articular, ocular, renal, pulmonary, hepatosplenic, muscular) exist, with lung involvement being the most common. PATIENT AND METHODS: We report a case of PG with skin and lung involvements in a patient treated with high-dose corticosteroids in a setting of severe ulcerative colitis (UC). Lung involvement was diagnosed during the pretreatment assessment performed prior to initiation of anti-TNFα therapy. Infliximab resulted in rapid improvement of the lung and skin lesions. DISCUSSION: In the event of simultaneous cutaneous PG lesions and lung lesions suggestive of abscess, visceral involvement should be suspected. First-line treatment consists of oral corticosteroids. In our patient, the occurrence of lesions under corticosteroids in UC militated in favour of anti-TNFα therapy, which proved effective.

[Pulmonary abscess and Pyoderma gangrenosum]. / Abcès pulmonaire et Pyoderma gangrenosum.

INTRODUCTION: Pyoderma gangrenosum is the ulcerative form of neutrophilic dermatoses. The most frequent extracutaneous localizations are the lungs, joints, and digestive tract. CASE RECORD: We report a case of Pyoderma gangrenosum, which presented first as an aseptic lung abscess. The first cutaneous lesions occurred 9 months later, with skin ulcerations on the thorax and on surgical scars. The histological diagnosis was made on skin biopsies. There was no associated abnormality except for IgA monoclonal gammapathy. Clinical improvement was noted with immunosuppressive treatment. DISCUSSION: This infrequent case report underlines that lung abscesses may be of non-infectious origin, that in Pyoderma gangrenosum, skin lesions may be come several months after extracutaneous manifestations, among which lungs abcesses are the most frequent.

[Pyoderma gangrenosum revealing Takayasu's arteritis]. / Pyoderma gangrenosum révélant une artérite de Takayasu.

BACKGROUND: Pyoderma gangrenosum is a form of neutrophilic dermatosis often associated with inflammatory disease or blood dyscrasias. We report a case of pyoderma gangrenosum revealing Takayasu's arteritis. OBSERVATION: A 26 year-old woman was followed since 2002 for pyoderma gangrenosum occurring during pregnancy and regressing under general corticosteroids. In January 2004, immediately after childbirth, she presented an extensive ulcerated lesion on the right forearm. Histological examination of a biopsy sample from this solution pointed towards pyoderma gangrenosum. Somatic examination showed reduced peripheral pulse on the right side, asymmetric blood pressure between the two arms and vascular murmur in the carotid, femoral and renal arteries. Based on laboratory and x-ray examinations, Takayasu's arteritis was diagnosed and chronic viral hepatitis B detected. Resumption of treatment with prednisone (1 mg/kg/d) resulted in resolution of the wounds within 1 month. DISCUSSION: Although pyoderma gangrenosum is a classic complication in Takayasu's arteritis in Japan, this combination is rare in Europe, North America and North Africa. This case was singular in terms of the occurrence of pyoderma gangrenosum with each pregnancy. However, a number of epidemiological and clinical features were consistent with those reported in the literature, namely presentation predominantly in female patients, young age and involvement of the limbs. In 60% of cases, pyoderma gangrenosum precedes Takayasu's arteritis. The recommended mode of treatment comprises oral corticosteroids as first-line therapy followed by cyclosporine as second-line treatment.

Radiological associations with dermatological disease.

Skin disease and its treatment may have radiological connotations. Though this article is not a comprehensive account of dermatological and radiological associations, it provides an eclectic selection of some common and some rare clinical entities, which the practicing radiologist may recognize or remember.

Pyoderma gangrenosum: a challenging complication of bilateral mastopexy.

A case of pyoderma gangrenosum progressively developing after bilateral mastopexy at the surgical site is described. The described case was successfully treated with corticosteroids, the application of the dermal regeneration template Integra and autologous skin grafts. This approach was able to save the patient's life and to generate a high-quality aesthetical outcome. The article reported the case, reviewed the literature of pyoderma gangrenosum related to mastopexy or augmentation mammoplasty and discussed the use of a dermal regeneration template to optimise aesthetical results after reconstructive surgery.