Blepharochalasis: Possibly associated with matrix metalloproteinases

Abstract

Blepharochalasis is a rare disorder characterized by recurrent episodic swelling of eyelids, eventually leading to atrophy of skin. Although immunological mechanisms may be involved in the degradation of elastic fibers, the pathogenesis is not well characterized. We report a 10-year-old Japanese boy with an 11-month history of the swelling of bilateral upper eyelids with atrophic skin. Attacks of non-painful swelling in eyelids with erythema have recurred several times a month and lasted for 2-3 days. Histological examination revealed perivascular and interstitial infiltration of lymphocytes in the dermis. Elastica Van Gieson staining showed a marked decrease of elastic fibers throughout the dermis. Results of direct and indirect immunofluorescence analyses were negative. Staining of matrix metalloproteinase (MMP)-3 and MMP-9 was observed in and around infiltrating cells in the dermis, suggesting that MMP-3 and MMP-9 may play, in part, roles in the development of blepharochalasis, and that inhibitor of MMP may have a possibility of therapeutic application.

Pilomatricoma with bullous‐like/anetodermic appearance: Possibly associated with matrix metalloproteinases

“…Matrix metalloproteinase‐9 and ‐12 are known to be capable of degrading elastic fibers . In mid‐dermal elastolysis, a previous study showed the degradation of elastic fibers and the overexpression of MMP‐9 in infiltrating inflammatory cells, such as leukocytes and macrophages .…”

Cutaneous Marginal Zone B-cell Lymphoma Evolving into Anetoderma: A Role of Matrix Metalloproteinases?

“…MMP-9 has stronger elastolytic potential than MMP-2 in human skin (10). In previous studies, the loose skin diseases with the loss of elastic fibres, including anetoderma, mid-dermal elastolysis and blepharochalasis were associated with the overexpression of MMP-9 in inflammatory infiltrates such as leucocytes and macrophages (11)(12)(13). Furthermore, it has been reported that MMP-9 was secreted from neoplastic plasma cells in multiple myeloma, and MMP-9 degraded extracellular matrix and facilitated tumour cell invasion (14).…”

Blepharochalasis: something to cry about

“…An etiologic role for proteinases, including matrix metalloproteinases (MMP), has been suggested by the finding of a neutrophilic infiltrate in some cases of blepharochalasis . Most recently, staining for MMP showed positivity for MMP‐3 and MMP‐9 in one case, suggesting that metalloproteinase dysregulation may contribute to disease pathogenesis . MMP‐2 and MMP‐9 are known to have extensive binding sites for elastin and are central to the process of elastin degradation .…”

“…12 Most recently, staining for MMP showed positivity for MMP-3 and MMP-9 in one case, suggesting that metalloproteinase dysregulation may contribute to disease pathogenesis. 13 MMP-2 and MMP-9 are known to have extensive binding sites for elastin and are central to the process of elastin degradation. 14 Interestingly, MMP-9 is naturally occurring in human tears, with concentrations 200 times greater when awakening from sleep than during midday or before sleep hours.…”