Scleredema associated with Sjögren’s syndrome

Abstract

Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Further investigation led to the diagnosis of primary Sjögren’s syndrome. The association between scleredema and autoimmune disorders has been rarely seen. To our knowledge, there are no other reports describing the association between primary Sjögren’s syndrome and scleredema adultorum of Buschke.

A case of concurrent systemic sclerosis and scleredema

“…Although this patient has a mildly elevated hemoglobin A1c level, suggestive of type 3 SAB, the absence of poorly controlled insulin-dependent diabetes suggests that this case is more consistent with the fourth type of SAB, described in association with miscellaneous conditions (ie, HIV, Sjögren syndrome, IgA deficiency, etc) 1, 4. Previous case studies have shown the coexistence of SAB with other rheumatologic diseases, including Sjögren syndrome, rheumatoid arthritis, ankylosing spondylitis, and dermatomyositis 4, 7, 8. To the best of our knowledge, there no report in the literature that describes the simultaneous appearance of SSc and SAB.…”

Concurrent Scleredema and Pyoderma Gangrenosum: Case Report and Review of Comorbid Conditions

“…However, comorbidities outside of these three types have also been observed in patients with scleredema; comorbid conditions are summarized in Table 1 [1,[5][6][7][8][9][10]. Some of the conditions include thyroid disease (hyperthyroidism), autoimmune disorders (Sjogren syndrome), and malignancy [8,9].…”

“…However, comorbidities outside of these three types have also been observed in patients with scleredema; comorbid conditions are summarized in Table 1 [1,[5][6][7][8][9][10]. Some of the conditions include thyroid disease (hyperthyroidism), autoimmune disorders (Sjogren syndrome), and malignancy [8,9]. A 30-year-old man with tear production impairment and salivary gland scintigraphy consistent with Sjogren syndrome was also found to have diffuse induration of the skin of the shoulders and upper arms; the diagnosis of scleredema was made by histopathologic examination [8].…”

“…Some of the conditions include thyroid disease (hyperthyroidism), autoimmune disorders (Sjogren syndrome), and malignancy [8,9]. A 30-year-old man with tear production impairment and salivary gland scintigraphy consistent with Sjogren syndrome was also found to have diffuse induration of the skin of the shoulders and upper arms; the diagnosis of scleredema was made by histopathologic examination [8]. Individual reports of scleredema patients with hematologic malignancy (chronic myeloid leukemia) have been described.…”

Rapidly Developed Multiple Face and Neck Skin Cancers in a Patient with Sjögren’s Syndrome: A Case Report

“…The common skin symptoms of Sjögren’s syndrome are dry skin and Raynaud’s phenomenon, which affected 55% and 35% of primary Sjögren’s syndrome patients, respectively [ 7 , 8 ]. However, there are few reports regarding the association of Sjögren’s syndrome with skin cancers, especially the development of multiple skin cancers.…”