دورية أكاديمية

Cutaneous manifestation of α₁-antitrypsin deficiency: panniculitis absent on biopsy.

التفاصيل البيبلوغرافية
العنوان: Cutaneous manifestation of α₁-antitrypsin deficiency: panniculitis absent on biopsy.
المؤلفون: Streicher JL; Department of Dermatology, Indiana University School of Medicine, 550 N University Blvd, Ste 3240, Indianapolis, IN 46202, USA. jennalstreicher@gmail.com., Sheehan MP, Armstrong AB, Mousdicas N
المصدر: Cutis [Cutis] 2014 Jun; Vol. 93 (6), pp. 303-6.
نوع المنشور: Case Reports; Journal Article
اللغة: English
بيانات الدورية: Publisher: Frontline Medical Communications Country of Publication: United States NLM ID: 0006440 Publication Model: Print Cited Medium: Internet ISSN: 2326-6929 (Electronic) Linking ISSN: 00114162 NLM ISO Abbreviation: Cutis Subsets: MEDLINE
أسماء مطبوعة: Publication: Parsippany, NJ : Frontline Medical Communications
Original Publication: New York : Cahners Pub Co.
مواضيع طبية MeSH: Panniculitis/*drug therapy , Panniculitis/*etiology , alpha 1-Antitrypsin Deficiency/*complications , alpha 1-Antitrypsin Deficiency/*drug therapy, Anti-Infective Agents/administration & dosage ; Dapsone/administration & dosage ; Female ; Humans ; Middle Aged ; Panniculitis/pathology ; Treatment Outcome ; alpha 1-Antitrypsin/administration & dosage
مستخلص: Patients with α₁-antitrypsin (AAT) deficiency may develop cutaneous manifestations of the disorder that histologically appear as panniculitis. Algorithms consistently emphasize measuring AAT levels when both clinical and histological features of deficiency are present; however, the patient's medical history and a physical examination alone can be extremely helpful in guiding the physician to the diagnosis of AAT deficiency. We describe a patient who presented with the classic clinical findings of AAT deficiency-associated panniculitis with surprising absence of panniculitis on repeated deep incisional biopsies. We propose a triad of classic findings that should alert the clinician to check the patient's serum AAT levels, even in the absence of panniculitis on histologic evaluation. Consideration of this clinical triad may prevent delays in the diagnosis of AAT deficiency, as early lesions may not yet demonstrate subcutaneous fat involvement.
المشرفين على المادة: 0 (Anti-Infective Agents)
0 (alpha 1-Antitrypsin)
8W5C518302 (Dapsone)
تواريخ الأحداث: Date Created: 20140708 Date Completed: 20150619 Latest Revision: 20140708
رمز التحديث: 20240628
PMID: 24999642
قاعدة البيانات: MEDLINE