Generalized acquired cutis laxa associated with coeliac disease: evidence of immunoglobulin A deposits on the dermal elastic fibres.
Acquired cutis laxa (ACL) is an uncommon elastolytic disorder of unknown aetiology. In rare instances, ACL has been reported in association with autoimmune diseases and dermal deposit of immunoglobulins, suggesting that destruction of elastic tissue may be immunologically mediated. We report a 35-year-old man with generalized acquired cutis laxa (GACL) associated with a persistent papular erythematous eruption that histopathologically showed some resemblance to dermatitis herpetiformis. A marked reduction and degeneration of dermal elastic fibres was noted 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 the dermal papillae. IgA deposits were also observed on the elastic fibres of the reticular dermis. Electron microscopy of skin from the submammary fold revealed fragmented elastic fibres, partial absence of peripheral microfibrils and abundant neutrophils, some of which were degranulated and adjacent to elastic fibres. Immunoelectron microscopy of an erythematous papule revealed IgA deposits around dermal elastic fibres. Antigliadin, antireticulin and antiendomysium antibodies were present. Jejunal biopsies showed a gluten-sensitive enteropathy. A possible IgA-mediated immune mechanism for the development of GACL in our patient is suggested.
García-Patos
V
V
Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Pujol
R M
RM
Barnadas
M A
MA
Pérez
M
M
Moreno
A
A
Condomines
J
J
Gelpi
C
C
Rodríguez
J L
JL
De Moragas
J M
JM
D000328:Adult; D002446:Celiac Disease / Q000150:complications* / Q000276:immunology; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000276:immunology; D004547:Elastic Tissue / Q000276:immunology*; D019066:Facies; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis*; D008297:Male; D012867:Skin / Q000276:immunology*
D007070:Immunoglobulin A