Acquired cutis laxa following urticarial vasculitis associated with IgA myeloma

Cutis laxa (CL) is an inherited or acquired connective tissue disorder characterized clinically by loosely hanging skin folds. There is often preceding cutaneous inflammatory eruption (ie, urticaria, eczema, erythema multiforme), and there is frequently internal organ involvement of the gastrointestinal, urogenital, pulmonary, and cardiovascular systems. Histologically, there are degenerative changes in the dermal elastic fibers. Of the few reports on this rare disorder, authors have speculated about an immune-mediated destruction of elastic fibers, and monoclonal gammopathies, such as multiple myeloma or heavy chain deposition disease, have a recognized association with CL. We report an unusual case of rapidly progressing acquired CL associated with leukocytoclastic vasculitis, IgA myeloma, and an immune complex–mediated glomerulonephritis. Light microscopy of the lax skin revealed complete absence of elastic fibers in areas of vasculitis.

Introduction

Cutis laxa (CL) is also known as dermatolysis, dermatomegaly, chalazoderma, pachydermatocele, dermatochalasia, and elastolysis.¹ Clinically, there is redundant skin with a wrinkled and loose appearance, giving rise to the descriptive term “bloodhound-like” facies when the eyelids, cheeks, and neck are involved. CL represents a heterogeneous group of connective tissue disorders that may be acquired or inherited and present as a generalized or local form. Light microscopy reveals few or absent elastic fibers in the dermis.²