Alpha-1 antitrypsin deficiency-associated panniculitis, published in the American Academy of Dermatology, January 29, 2021
Author
Marion Wilkens, Chairwoman Alpha1 Germany eV.
The article was published in the Journal of the American Academy of Dermatology on January 29, 2021. Alpha-1 antitrypsin deficiency-associated panniculitis.
The authors have attempted to clarify typical characteristics and examination methods of AATD-associated panniculitis and to evaluate the evidence regarding therapeutic options.
They came to the following conclusions:
AATD may be more common in patients with panniculitis than previously thought. Patients with panniculitis and systemic disease have a high mortality risk. Although most cases are associated with the severe ZZ genotype, moderate genotypes may also predispose to panniculitis. Dapsone remains the most cost-effective treatment option, while intravenous AAT augmentation remains the most effective. Finally, glucocorticoids appear to be ineffective in this setting.
