Dysphagia and Woody Induration of Skin in a Patient with Type 2 Diabetes

What is the diagnosis?

By Soumya Chatterjee, MD, MS, FRCP

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A 54-year-old woman presented to our general rheumatology clinic with widespread skin induration on her face, neck, anterior and posterior chest wall, proximal and distal upper extremities, abdominal wall and thighs. She had a history of poorly controlled type 2 diabetes mellitus on long-term insulin as well as an immunoglobulin G lambda monoclonal gammopathy.

A 54-year-old woman with scleredema. Involvement of periorbital skin causing bilateral lagophthalmos.

Indurated skin on the nape of neck, upper back and arms.

She described a feeling of tightness in the skin and stiffness in the nape of her neck that made it difficult to tuck her chin during swallowing. She also experienced difficulty initiating and completing swallowing both liquids and solids, regardless of consistency. She even experienced difficulty swallowing saliva, which led to frequent coughing, choking and subsequent hoarseness, indicating possible microaspirations. Eating quickly tired her, and she had early satiety, resulting in an unintentional weight loss of 10 pounds in a year. She denied heartburn.

Her gait was normal. She denied paresthesias and showed no evidence of diabetic peripheral or autonomic neuropathy and no ophthalmoscopic evidence of diabetic retinopathy.

Pinpointing the source

A punch biopsy of the skin on her left upper back revealed absence of fibroblastic proliferation but indicated increased spaces between the collagen bundles of the reticular dermis, associated with increased dermal mucin. These are characteristic features of scleredema.

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Her labs showed elevated fasting blood glucose and persistent glycosuria. She had a normal CBC, metabolic panel and urinalysis. A swallow study showed mild oral phase and mid-moderate pharyngeal phase swallowing deficits.

Standard barium swallow identified a diffusely narrow esophageal caliber and motility that indicated aperistalsis. The barium tablet only cleared past the transverse aorta after several swallows of water and then obstructed again at the lower esophageal sphincter. The findings on barium swallow resembled the abnormalities seen in achalasia.

Standard barium swallow shows a diffusely narrow esophageal caliber. Reprinted with permission from Chatterjee S, Hedman BJ, Kirby DF. An unusual cause of dysphagia. J Clin Rheum. 2018;24(8):444-448.

Esophageal manometry revealed markedly abnormal motility in the distal two-thirds of the esophagus and total absence of the primary peristaltic wave. Of 10 swallows, only four showed normal peristalsis in the body of the esophagus (five weak, one failed). The study also revealed low resting pressure with normal relaxation in the lower esophageal sphincter. Esophagogastroduodenoscopy confirmed decreased motility.

A solid meal gastric-emptying study ruled out gastroparesis as the cause of her early satiety. We attributed this instead to scleredematous involvement of the skin of the anterior abdominal wall, preventing gastric expansion after meals.

Scleredematous involvement of the skin of the anterior abdominal wall.

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Scleredema adultorum of Buschke

Three common sclerosing skin conditions have been associated with dysphagia due to oropharyngeal and esophageal dysmotility: systemic sclerosis, scleromyxedema and chronic sclerodermatous graft-versus-host disease. This is the first reported case of significant symptomatic dysphagia in a fourth sclerosing skin disorder, scleredema adultorum of Buschke. These four conditions show significant differences in dermal pathology but have all been associated with dysphagia, indicating a potential mechanical problem leading to stiffness or atrophy of the pharyngeal and esophageal musculature across the underlying etiologies.

Patients with diabetic autonomic neuropathy also can experience esophageal motility disorders, but this patient had no evidence of autonomic neuropathy.

Clinical course

Our approach included speech pathology and more aggressive management of her diabetes. Prior reports have shown occasional partial reversal of cutaneous scleredema with better glycemic control, so we were hopeful that her dysphagia may improve. Unfortunately, although her weight is unchanged, the severity of her dysphagia has not decreased.

This case of scleredema adultorum of Buschke is the first in the literature to exhibit severe dysmotility involving the smooth muscle of the entire esophagus. More studies are needed in order to offer these patients effective therapies.

Dr. Chatterjee directs the Scleroderma Program in the Department of Rheumatic and Immunologic Diseases.