Related Experiment Video
Updated: Nov 19, 2025
10:21
Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma
Published on: September 20, 2024
627
Alpha-1 antitrypsin deficiency-associated panniculitis
Alessandro N Franciosi1, James Ralph2, Naoimh J O'Farrell3
1Department of Medicine, Beaumont Hospital, Dublin, Ireland; Irish Centre for Genetic Lung Disease, Royal College of Surgeons in Ireland, Dublin, Ireland.
Journal of the American Academy of Dermatology
|January 31, 2021
Summary
Alpha-1 antitrypsin deficiency (AATD) is a rare cause of panniculitis. Intravenous AAT augmentation is effective, while glucocorticoids are ineffective for AATD-associated panniculitis.
Area of Science:
- Medical Science
- Genetics
- Dermatology
Background:
- Panniculitis is a rare, potentially fatal condition linked to alpha-1 antitrypsin deficiency (AATD).
- Limited evidence exists for managing AATD-associated panniculitis, primarily from case reports.
Purpose of the Study:
- To define the characteristics of AATD-associated panniculitis.
- To review diagnostic approaches.
- To assess therapeutic interventions for AATD-panniculitis.
Main Methods:
- Comprehensive literature search of MEDLINE, PubMed, and reference lists.
- Included articles and abstracts from 1970 to 2020.
- Focused on keywords: panniculitis, alpha-1 antitrypsin deficiency, and Weber-Christian disease.
Main Results:
- 117 cases of AATD-panniculitis identified; AATD found in 15% of biopsy-proven panniculitis cases.
- Systemic steroids showed no clinical response.
- Dapsone had frequent remission failures, but intravenous AAT augmentation therapy was generally effective.
Conclusions:
- AATD may be underdiagnosed in panniculitis patients.
- Panniculitis with systemic illness carries a high mortality risk.
- Intravenous AAT augmentation is the most effective treatment; glucocorticoids are ineffective.
Related Concept Videos
01:24Chronic Pancreatitis I: Introduction
445
The pancreas, an elongated and flat gland situated behind the stomach, serves a vital function in digesting food and managing blood sugar levels.
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
445
01:27Acute Pancreatitis I: Introduction
848
Pancreatitis is inflammation of the pancreas, an organ located behind the stomach. It can be either acute or chronic.
Acute pancreatitis is characterized by rapid inflammation of the pancreas, often caused by factors like gallstone blockage or excessive alcohol consumption. Chronic pancreatitis, on the other hand, is a slow, progressive inflammation that may result from long-term alcohol abuse, obstructions in the pancreatic duct, or genetic factors.
The causes of acute pancreatitis include:
Acute pancreatitis is characterized by rapid inflammation of the pancreas, often caused by factors like gallstone blockage or excessive alcohol consumption. Chronic pancreatitis, on the other hand, is a slow, progressive inflammation that may result from long-term alcohol abuse, obstructions in the pancreatic duct, or genetic factors.
The causes of acute pancreatitis include:
848
01:29Chronic Pancreatitis II: Collaborative Care
180
The management of chronic pancreatitis is multifaceted, involving a comprehensive approach that includes thorough assessment, diagnostic testing, and a variety of management strategies.
Assessment:
Assessment:
180
01:30Acute Pancreatitis II: Clinical Manifestations and Management
439
Acute pancreatitis presents a complex medical emergency characterized by rapid onset inflammation of the pancreas, demanding timely diagnosis and management to prevent complications. The condition primarily manifests through severe upper abdominal pain that often radiates to the back. This pain intensifies following the consumption of fatty foods. Accompanying symptoms such as nausea, vomiting, abdominal distention, fever, dyspnea, cyanosis, and jaundice can vary in intensity but significantly...
439
01:17Gastritis-II: Pathophysiology
867
Gastritis is marked by disruption of the mucosal barrier that usually protects the stomach tissue from digestive juices and manifests in acute and chronic forms.
In acute gastritis, the gastric mucosa becomes swollen and red and undergoes superficial erosion. Superficial ulceration may lead to bleeding.
In chronic gastritis, persistent or repeated insults lead to chronic inflammatory changes and, eventually, thinning or atrophy of the gastric tissue.
Gastritis can stem from various causes, each...
In acute gastritis, the gastric mucosa becomes swollen and red and undergoes superficial erosion. Superficial ulceration may lead to bleeding.
In chronic gastritis, persistent or repeated insults lead to chronic inflammatory changes and, eventually, thinning or atrophy of the gastric tissue.
Gastritis can stem from various causes, each...
867
01:30Peripheral Artery Disease I: Introduction
150
Peripheral artery disease (PAD) predominantly results from atherosclerosis, which involves the accumulation of fatty deposits, or plaques, within the walls of arteries. This causes them to narrow and harden, significantly reducing blood flow. PAD predominantly affects the legs, particularly the arteries supplying the thighs and calves. In rare cases, it may involve other arteries, including those in the arms.Etiology of PAD:The principal cause of PAD is atherosclerosis, which results from fatty...
150