J Rheum Dis.  2011 Sep;18(3):197-202. 10.4078/jrd.2011.18.3.197.

A Case of Scleredema Mimicking Systemic Sclerosis with Hypoalbuminemia Induced by Malabsorption in Alcoholic Chronic Pancreatitis

• Kim T
• Kim J
• Kim HU
• Kim J

• KMID: 2223150
• DOI: http://doi.org/10.4078/jrd.2011.18.3.197

Abstract

Scleroderma pathogenesis is the accumulation of extracellular matrix proteins and is a relatively rare connective tissue disorder characterized by skin fibrosis, obliterative vasculopathy, and distinct autoimmune abnormalities. However, many other clinical conditions known collectively as the scleroderma-like syndrome present with substantial skin fibrosis and may be confused with scleroderma, sometimes leading to an incorrect diagnosis. Due to this, early and correct diagnosis is very important to for appropriate treatment available for scleroderma-like syndrome. We report a rare case of scleredema mimicking systemic sclerosis with hypoalbuminemia induced by malabsorption in alcoholic chronic pancreatitis. In this case, the patient's skin sclerosis and joint contracture dramatically improved after high dose steroid theraphy.

Keyword

Scleredema; Systemic sclerosis; Malabsorption; Alcoholic chronic pancreatitis; Steroid therapy

MeSH Terms

Alcoholics
Connective Tissue
Contracture
Extracellular Matrix Proteins
Fibrosis
Humans
Hypoalbuminemia
Joints
Pancreatitis, Chronic
Scleredema Adultorum
Scleroderma, Systemic
Sclerosis
Skin
Extracellular Matrix Proteins

Figure

• 

  Figure 1. Physical examination of range of motion in knee joint and sclerotic skin in lower extremities. (A) Range of motion in both knees joint was less than 90 degree. (B, C) Ill-defined, oranged-hued, pitting indurative edema of both shins and foot dorsums. (D, E) The Skin of upper thigh (A) compared normal skin of forearm (B) does not have been pinched very hard because of sclerosis.

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  Figure 2. (A) Enhanced Abdominal pelvic CT revealed granular calcification and generalized atrophy in the pancreas without a dilated pancreatic duct and pancreas swelling. (B) Colonoscopic finding was revealed microvesicular fat drop without mucosal lesion.

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  Figure 3. Skin biopsy in Rt. lateral thigh. (A, B) Large areas of subcutaneous fat replaced by newly formed collagen which consists of thick, hyalinized hypocellular bundles (A, H&E stain, ×50), (B, H&E, ×100). (C, D) Increased thickened wavy collagen bundles in the reticular dermis without manifest inflammatory infiltrations. (C, Masson-Trichrome stain, ×50), (D, Masson's-Trichrome stain, ×100).

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  Figure 4. Both knee joint cont-ractures were improved enough to be walking alone in the hospital at 19 days after admission.

Reference

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