Acral localized acquired cutis laxa: Report of a case associated with inflammatory arthritis
Authors: Tess Peters, Cheryl Barnabe, Wedad Hanna, Duane Barber, Richard M. Haber
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: Acral localized acquired cutis laxa (ALACL) is a distinct variant of acquired cutis laxa characterized by loose redundant skin primarily confined to the distal extremities. It is often associated with signs or symptoms of a preceding inflammatory dermatosis, such as urticaria, erythema, swelling, or pruritus. Disease associations include multiple myeloma, idiopathic urticaria, Raynaud phenomenon, and rheumatoid arthritis. We report a case associated with inflammatory arthritis in childhood. A 41-year-old woman with a history of polyarticular juvenile idiopathic arthritis diagnosed at age 3 presented with asymptomatic loose, pendulous skin confined to her volar finger pads and toes. She noted the onset of loose skin at age 6 and described ill-defined erythema, warmth, and pruritus of her hands that preceded the development of the skin changes. The degree of skin laxity remained stable since childhood. There was no history of urticaria. Family history was relevant for a paternal grandfather with rheumatoid arthritis. On examination, there was loose redundant skin on the finger pads bilaterally that gave them a flat rounded appearance (Fig 1). The volar finger pads were soft on palpation and the skin remained depressed when pressure was applied, giving them an appearance that has been described as "chewing gum-like."1Martin L. Requena L. Yus E.S. Furio V. Farina M.C. Acrolocalized acquired cutis laxa.Br J Dermatol. 1996; 134: 973-976Crossref PubMed Scopus (25) Google Scholar The plantar surface of the toes had similar skin changes, although less striking. Facial appearance was normal and did not show advanced aging. Rheumatoid factor and anticyclic citrullinated peptide were negative. Plain radiography of the hands showed stable minor erosive changes. Skin biopsy samples were obtained from the volar surface of the left middle finger and unaffected skin on the left upper arm. The specimen from the left middle finger showed a marked decrease in elastic fibers. A neutrophilic inflammatory infiltrate was not identified, as was observed previously in ALACL.2Fisher B.K. Page E. Hanna W. Acral localized acquired cutis laxa.J Am Acad Dermatol. 1989; 21: 33-40Abstract Full Text PDF PubMed Scopus (40) Google Scholar, 3Kluger N. Molès J. Vanakker O.M. Pernet C. Berylot-Berry M. Bessis D. Acquired cutis laxa associated with IgA multiple myeloma, joint hyperlaxity and urticarial neutrophilic dermatosis.Acta Derm Venerol. 2014; 94: 743-744Crossref PubMed Scopus (7) Google Scholar The unaffected skin from the left arm showed a normal number of elastic fibers and normal morphology under electron microscopy. Ultrastructural examination of the specimen from the left middle finger showed elastic fibers fragmented into multiple electron dense pieces (Fig 2). ALACL was first described in 1989 by Fisher et al in a female patient following urticaria.2Fisher B.K. Page E. Hanna W. Acral localized acquired cutis laxa.J Am Acad Dermatol. 1989; 21: 33-40Abstract Full Text PDF PubMed Scopus (40) Google Scholar Biopsy from a urticarial lesion showed neutrophils attached to the surface of degenerated and normal elastic fibers.2Fisher B.K. Page E. Hanna W. Acral localized acquired cutis laxa.J Am Acad Dermatol. 1989; 21: 33-40Abstract Full Text PDF PubMed Scopus (40) Google Scholar Reed et al hypothesized that elastolysis may result from degradation by neutrophil elastase.4Reed W.B. Horowitz R.E. Beighton P. Acquired cutis laxa: primary generalized elastolysis.Arch Dermatol. 1971; 103: 661Crossref PubMed Scopus (82) Google Scholar The association of ALACL with inflammatory arthritis in childhood has not been reported thus far. Rongioletti et al described a case of ALACL associated with rheumatoid arthritis and myopathy.5Rongioletti F. Cutolo M. Bondavalli P. Rebora A. Acral localized acquired cutis laxa associated with rheumatoid arthritis.J Am Acad Dermatol. 2002; 46: 128-130Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar A recent report of ALACL demonstrating a perivascular neutrophilic infiltrate with IgA deposits in superficial dermal vessels suggested a common pathogenic mechanism with autoinflammatory syndromes such as Schnitzler syndrome or adult-onset Still disease.3Kluger N. Molès J. Vanakker O.M. Pernet C. Berylot-Berry M. Bessis D. Acquired cutis laxa associated with IgA multiple myeloma, joint hyperlaxity and urticarial neutrophilic dermatosis.Acta Derm Venerol. 2014; 94: 743-744Crossref PubMed Scopus (7) Google Scholar Including the case reported, we have identified 13 reports of ALACL in the literature to date.1Martin L. Requena L. Yus E.S. Furio V. Farina M.C. Acrolocalized acquired cutis laxa.Br J Dermatol. 1996; 134: 973-976Crossref PubMed Scopus (25) Google Scholar, 2Fisher B.K. Page E. Hanna W. 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Acral localized acquired cutis laxa.Int J Dermatol. 2013; 52: 983-986Crossref PubMed Scopus (4) Google Scholar Of patients with ALACL, 54% described a prodrome of erythema, edema, urticaria, or pruritus involving the hands. Given the consistent localization and disease associations, we propose that ALACL be recognized as a distinct entity within the spectrum of acquired cutis laxa.