Acral Acquired Cutis Laxa Associated with IgA Multiple Myeloma, Joint Hyperlaxity and Urticarial Neutrophilic Dermatosis

Abstract

Acral localised acquired cutis laxa (ALACL) is a very rare form of acquired cutis laxa characterised by loose redundant skin folds confined to the hands and feet, giving the appearance of premature aging, and histological loss of dermal elastic tissue (1-9). We report here a new case of ALACL associated with IgA multiple myeloma and also, for the first time, with joint hyperlaxity and recurrent neutrophilic urticarial dermatosis.CASE REPORT (for detailed methods see Appendix S1 1 )A 40-year-old man presented in 2007 for the management of ALACL and monoclonal IgA gammapathy. He had chronic urticarial dermatosis of the extremities, mostly involving the hands, which had manifested in 1996 and progressively worsened, with repeated swelling of the fingers. ALACL was diagnosed at that time in association with an unusual hyperlaxity of the distal interphalangeal joints. Over the past several years, he has received multiple treatments including methotrexate, colchicine, hydroxychloroquine, intravenous gammaglobulins and dapsone. All have either been ineffective or had debilitating side effects. Oral prednisone resulted in complete remission of the urticarial lesions, with steroid dependence estimated at 20-30 mg/day, but this did not prevent the progression in joint laxity or cutis laxa. Physical examination disclosed the loose, redundant skin of all fingers (Fig. 1A). On demand, he was able to completely dislocate multiple interphalangeal joints painlessly and effortlessly (Fig. 1B). Laboratory tests showed a monoclonal IgA lambda peak with hypogammaglobulinemia (4.9 g/l; normal 7.1-15.6 g/l) with decreased IgG (3.92 g/l; normal 6.9-14 g/l) and normal serum levels of IgM and IgA. Urinary immunoelectrophoresis showed increased lambda light chains. Hand X-rays revealed articular dislocations of most of the proximal and distal interphalangeal joints. Bone marrow aspiration revealed 13% plasma cells with dystrophy, consistent with IgA myeloma. A biopsy specimen taken from an urticarial papule of the palmar hand revealed a dense dermal interstitial and perivascular neutrophilic infiltrate and leukocytoclasia without oedema or vasculitis (Fig. 2), consistent with neutrophilic urticarial dermatosis. Amyloid Congo red staining was negative in all biopsy specimens. Between 2007 and 2012, the following treatments were all ineffective: plasmapheresis (6 sessions), anakinra (3 months), infliximab (4 months), rituximab (4 sessions) and alkeran (2 sessions). Unfortunately, the patient chose not to follow-up.Direct immunofluorescence investigation of lesional skin revealed abundant IgA deposits that decorated the dermoepidermal junction (DEJ) and the capillary network of the dermal papillae (Fig. 3A). A control specimen from non-lesional forearm skin showed only faint staining in the DEJ (data not shown). Electron microscopy showed conspicuous diminution of the elastic fibres with normal-appearing collagen fibres; macrophages were observed phagocyting the elastic fibres. Direct immunoelectron microscopy further localised the IgA depos...

Case of acquired cutis laxa with preceding urticarial eruption treated by diphenyl sulfone

“…Thus, acquired CL might have been triggered by urticarial eruption. In three cases,2, 3, 4 a biopsy was performed on an urticarial lesion, and revealed the presence of neutrophils, while the elastic fibers were thin and fragmented. In our case, neutrophils adhered to elastic fibers.…”

Acral localized acquired cutis laxa as presenting sign of underlying systemic amyloidosis

“…This form of cutis laxa appears to be an acquired condition, and it has been associated with either preceding inflammatory conditions or amyloid deposition with an underlying plasma cell dyscrasia. [1][2][3][4][5][6][7][8][9][10] The diagnosis can be challenging, because of both its rarity and the subtle histopathologic findings of involved skin.…”

“…ALACL is rare condition characterized by loose, redundant skin of the hands or feet which histopathologically is consistent with other forms of cutis laxa with reduced or fragmented dermal elastic fibers. This form of cutis laxa appears to be an acquired condition, and it has been associated with either preceding inflammatory conditions or amyloid deposition with an underlying plasma cell dyscrasia 1‐10 . The diagnosis can be challenging, because of both its rarity and the subtle histopathologic findings of involved skin.…”

“…These cases have been associated with episodes of recurrent inflammation, 2‐5 or with amyloid deposition within the affected tissue as a presenting sign of systemic plasma cell dyscrasia 6‐9 . One case reported ALACL in a patient with chronic urticarial dermatosis and IgA myeloma but without amyloid deposition 10 . In this report, we describe a patient with ALACL as the sole presenting sign of underlying systemic primary amyloidosis requiring autologous stem cell transplantation.…”

“…[6][7][8][9] One case reported ALACL in a patient with chronic urticarial dermatosis and IgA myeloma but without amyloid deposition. 10 In this report, we describe a patient with ALACL as the sole presenting sign of underlying systemic primary amyloidosis requiring autologous stem cell transplantation.…”