Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.24.3 (collagenase)
 18,340 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To our knowledge, necrotizing lid tumors occurring in the context of a necrotizing lobular panniculitis have not yet been described in the literature. Our patient, a 75-year-old male, presented with indolent, nonmoveable subcutaneous lumps that were centrally ulcerating. They appeared first on his upper lid, then on his lower lid and thereafter on the neck, back, upper extremity and abdomen, especially in the area of his cholecystectomy scar. A thorough work-up, which included repeated histopathological examinations performed by several laboratories, led to the diagnosis of an idiopathic necrotizing lobular panniculitis. A serum level determination performed later revealed no alpha 1-antitrypsin deficiency. As the eye-lid's subcutaneous tissue lacks fat, the association between the necrotizing lid tumors and the necrotizing panniculitis appears to be a paradox. In spite of the normal alpha 1-antitrypsin serum levels--determined when the patient's lesions had long ago healed--we think in retrospect that the differential diagnosis should have included the possibility of a decreased alpha 1-antitrypsin serum level (e.g. heterozygous MZ-phenotype). An alpha 1-antitrypsin level deficiency--with resulting decreased inhibition of collagenase and elastase--could account for the necrotizing process that also occurred in the eyelid's subcutaneous tissue. In the Van Gieson stain of this patient's eyelid biopsy, fragmentation of all visible collagen and elastic fibers was noted. In our opinion, the differential diagnosis does include lid involvement with secondary panniculitis caused by partially decreased alpha 1-antitrypsin serum levels and--by exclusion--idiopathic necrotizing lobular panniculitis. Therapeutic possibilities are briefly discussed.
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PMID:[Recurrent eyelid tumor in necrotizing panniculitis]. 161 47

Three patients (a 71-year-old man and 2 women, 73 and 50 years, respectively) with recurrent panniculitis associated with alpha 1-antitrypsin deficiency are presented. Because the concept of chronic and exaggerated inflammatory response in the patients with alpha 1-antitrypsin deficiency is based on the theory of protease-antiprotease imbalance, we suggest that tetracyclines will alleviate this condition. Indeed, tetracyclines were found to inhibit collagenase activity. The total remission of the condition in these 3 patients underlines for the first time the effects of doxycycline on a condition characterized by deficiency of the antiprotease system. A review of the 23 reported cases of panniculitis associated with alpha 1-antitrypsin deficiency is presented in table form.
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PMID:Use of anti-collagenase properties of doxycycline in treatment of alpha 1-antitrypsin deficiency panniculitis. 167 18