Alpha-1-antitrypsin deficiency associated with panniculitis treated with plasma exchange therapy
| Autor: | Nelson Hidekazu Tatsui, Evandro A. Rivitti, Libania Paz‐Melgar, Alfredo Mendrone, Maria Denise F. Takahashi, Marcello Menta Simonsen Nico, Priscila de Oliveira |
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| Rok vydání: | 2004 |
| Předmět: |
Adult
medicine.medical_specialty Pathology Panniculitis Cirrhosis Dermatology Lobular panniculitis Gastroenterology Diagnosis Differential alpha 1-Antitrypsin Deficiency Internal medicine Skin Ulcer medicine Humans Protease inhibitor (pharmacology) Buttocks Leg Alpha 1-antitrypsin deficiency Plasma Exchange medicine.diagnostic_test business.industry medicine.disease Alternative treatment medicine.anatomical_structure Skin biopsy Female business |
| Zdroj: | International Journal of Dermatology. 43:693-697 |
| ISSN: | 1365-4632 0011-9059 |
| DOI: | 10.1111/j.1365-4632.2004.02054.x |
| Popis: | Background Alpha-1-antitrypsin is the principal serum protease inhibitor. In addition to the well-recognized association with early-onset emphysema and cirrhosis, alpha-1-antitrypsin deficiency may be associated with panniculitis. The treatment of this type of panniculitis presents a significant challenge. Previous attempts using immunosuppressive, anti-inflammatory, and cytotoxic drugs have shown variable results. Aim To report a case of alpha-1-antitrypsin deficiency-associated panniculitis treated with plasma exchange therapy. Methods A 23-year-old patient developed painful red nodules on her thighs and buttocks with spontaneous ulceration and discharge of oily fluid. A skin biopsy specimen showed septal and lobular panniculitis. The serum alpha-1-antitrypsin level was 22 mg/dL. She was treated with plasma exchange therapy. Results Treatment of this patient with plasma exchange therapy led to the control of the cutaneous lesions. Conclusions Plasma exchange therapy represents an alternative treatment which restores serum and tissue alpha-1-antitrypsin levels. This method is proposed because of its clinical benefits and greater availability. |
| Databáze: | OpenAIRE |
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