Osteomielitis aséptica craneal asociada a pioderma gangrenoso / Cranial aseptic osteomyelitis associated with pyoderma gangrenosum

Las dermatosis neutrofílicas (DN) constituyen un grupo heterogéneo de enfermedades. Excepcionalmente, las DN pueden acompañarse de acúmulos de neutrófilos estériles en otros tejidos distintos a la piel. Una paciente de 34 años consultó por una cefalea que no respondía al tratamiento analgésico. Una TAC demostró una lesión osteolítica a nivel parietal derecho, cuyo estudio histopatológico sugería una osteomielitis. Un año después del inicio de la cefalea desarrolló un pioderma gangrenoso en cara anterior de ambas piernas. Tras tratamiento con corticoterapia sistémica se resolvieron las lesiones cutáneas y la cefalea. La afectación ósea en las dermatosis neutrofílicas es excepcional. Habitualmente afecta a la población infantil en el contexto de una osteomielitis crónica recurrente multifocal (OCRM). Solo se han descrito dos casos en adultos, una paciente de 26 años, con una OCRM desde la infancia, y un varón de 67 años que desarrolló una osteomielitis aséptica en continuidad de un pioderma gangrenoso (AU)

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the other one in contiguity with the cutaneous lesions of pyoderma gangrenosum (AU)

Pioderma gangrenoso simulando un carcinoma epidermoide / Pyoderma Gangrenosum Mimicking a Squamous Cell Carcinoma

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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.

Utilidad de la ecografía en el diagnóstico y seguimiento del pioderma gangrenoso / Usefulness of ultrasound in the diagnosis and follow-up of pyoderma gangrenosum

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[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.