GENERALIZED ACQUIRED CUTIS LAXA ASSOCIATED WITH CELIAC-DISEASE - EVIDENCE OF IMMUNOGLOBULIN-A DEPOSITS ON THE DERMAL ELASTIC FIBERS

Acquired cutis laxa (ACL) is an uncommon elastolytic disorder of unkno wn aetiology. In rare instances, ACL has been reported in association with autoimmune diseases and dermal deposit of immunoglobulins, sugges ting that destruction of elastic tissue may be immunologically mediate d. We report a 35-year-old man with generalized acquired cutis laxa (G ACL) associated with a persistent papular erythematous eruption that h istopathologically showed some resemblance to dermatitis herpetiformis . A marked reduction and degeneration of dermal elastic fibres was not ed in biopsies from loose-hanging skin. Direct immunofluorescence from non-inflammatory loose skin revealed granular immunoglobulin A (IgA) deposits at the basement membrane zone and fibrillar IgA deposits in t he dermal papillae. IgA deposits were also observed on the elastic fib res of the reticular dermis. Electron microscopy of skin from the subm ammary fold revealed fragmented elastic fibres, partial absence of per ipheral microfibrils and abundant neutrophils, some of which were degr anulated and adjacent to elastic fibres. Immunoelectron microscopy of an erythematous papule revealed IgA deposits around dermal elastic fib res. Antigliadin, antireticulin and anti-endomysium antibodies were pr esent. Jejunal biopsies showed a gluten-sensitive enteropathy. A possi ble IgA-mediated immune mechanism for the development of GACL in our p atient is suggested.