Widespread scleredema accompanied with a monoclonal gammopathy in a patient with advanced ankylosing spondylitis

Abstract

Scleredema is a rare cutaneous mucinosis characterized by chronic diffuse induration of the skin, and it is occasionally associated with a monoclonal gammopathy (MG). Ankylosing spondylitis (AS) is noted to be another, chronic systemic inflammatory disorder of the axial skeleton that may accompany the MG. However, patients with scleredema and AS accompanied with a MG have not been reported in the literature. We here report a 40-yr-old man with scleredema and advanced AS accompanied with a MG of IgA-kappa protein. Widespread, long-standing scleredema has been developed over 10 yrs after the initial manifestation of AS. It is uncertain whether the coexistence of scleredema and AS is more than coincidental.

Fig. 1

There is a marked induration of the skin on the posterior neck.

Fig. 2

The radiograph of lumbar spine and pelvis shows near total ankylosis of bilateral sacroiliac joints and a bamboo spine appearance with bilateral syndesmophytes (arrows) at the thoracolumbar vertebrae.

Fig. 3

(A) The collagen bundles are thickened and separated by clear spaces leading to fenestrations of the collagen (hematoxylin and eosin, ×200). (B) Mucin deposits are present between the collagen bundles (Alcian blue stain, ×200).