ABSTRACT
Although polyarteritis nodosa (PAN) may result in thrombosis or aneurysm formation in any organ in the body, hepatobiliary complications are unusual. We reviewed seven cases that demonstrated the diagnostic difficulties and therapeutic options available in the management of hepatobiliary PAN. No consistent sign that indicated the severity of hepatobiliary PAN could be identified. In cases of thrombotic PAN, acalculus cholecystitis usually could be diagnosed preoperatively. Early tissue diagnosis and aggressive intervention are required for appropriate patient treatment. If the diagnosis is unclear, a preoperative muscle or skin biopsy specimen is often helpful in establishing a tissue diagnosis of PAN, even if no obvious pathologic condition is evident. Patients who undergo celiotomy for acalculus cholecystitis or peritoneal signs of an unclear origin should have tissue specimens (gallbladder wall, liver, or omentum) submitted for pathologic study. Angiography may be diagnostic preoperatively or when results of biopsies are equivocal. In addition, early angiography can define the extent of visceral involvement and permit control by embolization of hemorrhage secondary to aneurysm rupture. Awareness of the possibilities of thrombotic, ischemic, or bleeding complications from PAN allows more aggressive and rapid management of abdominal complaints, especially in patients who are receiving immunosuppressant therapy.
Subject(s)
Aneurysm/etiology , Cholecystitis/etiology , Hepatic Artery , Polyarteritis Nodosa/complications , Adult , Aged , Aged, 80 and over , Aneurysm/pathology , Cholecystitis/pathology , Female , Hepatic Artery/pathology , Humans , Male , Middle Aged , Polyarteritis Nodosa/pathologySubject(s)
Chediak-Higashi Syndrome , Adolescent , Agranulocytosis , Albinism , Anemia, Hemolytic , Animals , Bacterial Infections , Cattle , Chediak-Higashi Syndrome/diagnosis , Chediak-Higashi Syndrome/immunology , Child , Consanguinity , Female , Hemorrhagic Disorders , Hepatomegaly , Humans , Hypersensitivity, Delayed/immunology , Leukocytes , Lysosomes , Male , Mice , Mink , Neurologic Manifestations , Nystagmus, Pathologic , SplenomegalyABSTRACT
BACKGROUND: Commonly used treatments for pyoderma gangrenosum are medical, with immunosuppressive agents employed most often. OBJECTIVE: To report a case and discuss the indications for radical surgical treatment of pyoderma gangrenosum. METHODS: Analysis of a case of Crohn's disease-associated pyoderma gangrenosum treated with immunosuppression followed by amputation, and a review of the literature on surgical management of pyoderma gangrenosum. RESULTS: In unstable patients with intractable multiple medical problems, surgical treatment of pyoderma gangrenosum may be indicated by the existence of these life-threatening comorbidities. The recent literature suggests that surgical management of pyoderma gangrenosum may also be appropriate in other special circumstances. CONCLUSIONS: Surgical management, including amputation, may have a role in the management of pyoderma gangrenosum. Further research is needed to delineate precisely the circumstances and patient factors that are appropriate indications for such surgery.
Subject(s)
Pyoderma Gangrenosum/surgery , Aged , Amputation, Surgical , Biopsy, Needle , Chronic Disease , Combined Modality Therapy , Drug Therapy, Combination , Emergencies , Fatal Outcome , Female , Humans , Leg/microbiology , Leg/pathology , Leg/surgery , Necrosis , Pyoderma Gangrenosum/diagnosis , Pyoderma Gangrenosum/microbiologyABSTRACT
The percent of conventionally stained circulating neutrophils containing abnormal giant granules in Chediak-Higashi mink, cattle and mice is highly variable and strikingly less than the 100% found in neutrophils from similarly affected humans. In contrast, using either peroxidase or Sudan Black, it can be demonstrated that 100% of circulating neutrophils in all affected individuals of the four species studied contain the abnormal granules. Minor species differences in the shape of the granules were noted. The significance of these findings as well as the variations in granular staining by the several techniques employed are discussed.