Guha Ashrith, MD, MPH

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Thick skin on the back

Author(s)

Guha Ashrith, MD, MPH

Rajpreet Arora, MD

Figure 1. Erythematous induration of the skin limited to the back.

A 66-year-old obese black woman with long-standing uncontrolled type 2 diabetes mellitus (hemoglobin A_1c 15.1%) presents with an indurated, wood-like thickening of the skin on her back, with mild pitting (Figure 1). This condition has been present for 3 years and is associated with diffuse erythema. She denies any history of Raynaud phenomenon, arthralgias, dysphagia, or rashes. Her antinuclear antibody titer is highly positive at 1:640 dilution, with a speckled pattern. All other autoantibody tests (antitopoisomerase-I, Sjögren antibodies, anti-Smith and anti-Smith/ribonucleoprotein, and antiphospholipid nucleoprotein, and antiphospholipid antibodies) are negative. Serum electrophoresis and urinary porphobilinogen levels are normal.

Q: Which is the correct diagnosis?

Scleroderma (systemic sclerosis)
Scleredema diabeticorum
Amyloidosis
Cutaneous sarcoidosis
Porphyria cutanea tarda

A: The correct answer is scleredema diabeticorum, a common, underdiagnosed skin manifestation of uncontrolled diabetes mellitus seen in 2.5% to 14% of diabetic patients.^1,2 It most often presents with the insidious onset of painless induration and nonpitting thickening of the skin, predominantly on the upper back and neck. Biopsy of the skin usually reveals thickening of the dermis with deposition of collagen and hyaluronic acid without an inflammatory infiltrate.³

Of note, patients may present with similar skin changes acutely in conditions such as postinfectious scleredema (scleredema of Buschke) and paraproteinemias.

Treatment of scleredema is usually difficult, but options include radiotherapy, ultraviolet light therapy, low-dose methotrexate, psoralen, and extracorporeal photopheresis.^4–7

References

Cole GW, Headley J, Skowsky R. Scleredema diabeticorum: a common and distinct cutaneous manifestation of diabetes mellitus. Diabetes Care 1983; 6:189–192.
Sattar MA, Diab S, Sugathan TN, Sivanandasingham P, Fenech FF. Scleroedema diabeticorum: a minor but often unrecognized complication of diabetes mellitus. Diabet Med 1988; 5:465–468.
Varga J, Gotta S, Li L, Sollberg S, Di Leonardo M. Scleredema adultorum: case report and demonstration of abnormal expression of extracellular matrix genes in skin fibroblasts in vivo and in vitro. Br J Dermatol 1995; 132:992–999.
Seyger MM, van den Hoogen FH, de Mare S, van Haelst U, de Jong EM. A patient with a severe scleroedema diabeticorum, partially responding to low-dose methotrexate. Dermatology 1999; 198:177–179.
Lee MW, Choi JH, Sung KJ, Moon KC, Koh JK. Electron beam therapy in patients with scleredema. Acta Derm Venereol 2000; 80:307–308.
Bowen AR, Smith L, Zone JJ. Scleredema adultorum of Buschke treated with radiation. Arch Dermatol 2003; 139:780–784.
Beers WH, Ince A, Moore TL. Scleredema adultorum of Buschke: a case report and review of the literature. Semin Arthritis Rheum 2006; 35:355–359.

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Guha Ashrith, MD, MPH
Division of Cardiology, Department of Internal Medicine, University of Iowa Hospitals and Clinics, Iowa City

Rajpreet Arora, MD
Division of Rheumatology, Department of Internal Medicine, University of Texas Health Science Center, Houston

Address: Guha Ashrith, MD, MPH, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, T 411 GH, Iowa City, IA 52242; e-mail [email protected]

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Cleveland Clinic Journal of Medicine - 77(2)

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Cleveland Clinic Journal of Medicine

Type 2 Diabetes ICYMI

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Page Number

90-91

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Author(s)

Guha Ashrith, MD, MPH

Rajpreet Arora, MD

Author(s)

Guha Ashrith, MD, MPH

Rajpreet Arora, MD

Author and Disclosure Information

Guha Ashrith, MD, MPH
Division of Cardiology, Department of Internal Medicine, University of Iowa Hospitals and Clinics, Iowa City

Rajpreet Arora, MD
Division of Rheumatology, Department of Internal Medicine, University of Texas Health Science Center, Houston

Address: Guha Ashrith, MD, MPH, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, T 411 GH, Iowa City, IA 52242; e-mail [email protected]

Author and Disclosure Information

Guha Ashrith, MD, MPH
Division of Cardiology, Department of Internal Medicine, University of Iowa Hospitals and Clinics, Iowa City

Rajpreet Arora, MD
Division of Rheumatology, Department of Internal Medicine, University of Texas Health Science Center, Houston

Address: Guha Ashrith, MD, MPH, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, T 411 GH, Iowa City, IA 52242; e-mail [email protected]

Article PDF

media_2a9e037_90.pdf

Article PDF

media_2a9e037_90.pdf

A 66-year-old obese black woman with long-standing uncontrolled type 2 diabetes mellitus (hemoglobin A_1c 15.1%) presents with an indurated, wood-like thickening of the skin on her back, with mild pitting (Figure 1). This condition has been present for 3 years and is associated with diffuse erythema. She denies any history of Raynaud phenomenon, arthralgias, dysphagia, or rashes. Her antinuclear antibody titer is highly positive at 1:640 dilution, with a speckled pattern. All other autoantibody tests (antitopoisomerase-I, Sjögren antibodies, anti-Smith and anti-Smith/ribonucleoprotein, and antiphospholipid nucleoprotein, and antiphospholipid antibodies) are negative. Serum electrophoresis and urinary porphobilinogen levels are normal.

Q: Which is the correct diagnosis?

Scleroderma (systemic sclerosis)
Scleredema diabeticorum
Amyloidosis
Cutaneous sarcoidosis
Porphyria cutanea tarda

A: The correct answer is scleredema diabeticorum, a common, underdiagnosed skin manifestation of uncontrolled diabetes mellitus seen in 2.5% to 14% of diabetic patients.^1,2 It most often presents with the insidious onset of painless induration and nonpitting thickening of the skin, predominantly on the upper back and neck. Biopsy of the skin usually reveals thickening of the dermis with deposition of collagen and hyaluronic acid without an inflammatory infiltrate.³

Of note, patients may present with similar skin changes acutely in conditions such as postinfectious scleredema (scleredema of Buschke) and paraproteinemias.

Treatment of scleredema is usually difficult, but options include radiotherapy, ultraviolet light therapy, low-dose methotrexate, psoralen, and extracorporeal photopheresis.^4–7

A 66-year-old obese black woman with long-standing uncontrolled type 2 diabetes mellitus (hemoglobin A_1c 15.1%) presents with an indurated, wood-like thickening of the skin on her back, with mild pitting (Figure 1). This condition has been present for 3 years and is associated with diffuse erythema. She denies any history of Raynaud phenomenon, arthralgias, dysphagia, or rashes. Her antinuclear antibody titer is highly positive at 1:640 dilution, with a speckled pattern. All other autoantibody tests (antitopoisomerase-I, Sjögren antibodies, anti-Smith and anti-Smith/ribonucleoprotein, and antiphospholipid nucleoprotein, and antiphospholipid antibodies) are negative. Serum electrophoresis and urinary porphobilinogen levels are normal.

Q: Which is the correct diagnosis?

Scleroderma (systemic sclerosis)
Scleredema diabeticorum
Amyloidosis
Cutaneous sarcoidosis
Porphyria cutanea tarda

A: The correct answer is scleredema diabeticorum, a common, underdiagnosed skin manifestation of uncontrolled diabetes mellitus seen in 2.5% to 14% of diabetic patients.^1,2 It most often presents with the insidious onset of painless induration and nonpitting thickening of the skin, predominantly on the upper back and neck. Biopsy of the skin usually reveals thickening of the dermis with deposition of collagen and hyaluronic acid without an inflammatory infiltrate.³

Of note, patients may present with similar skin changes acutely in conditions such as postinfectious scleredema (scleredema of Buschke) and paraproteinemias.

Treatment of scleredema is usually difficult, but options include radiotherapy, ultraviolet light therapy, low-dose methotrexate, psoralen, and extracorporeal photopheresis.^4–7

References

Cole GW, Headley J, Skowsky R. Scleredema diabeticorum: a common and distinct cutaneous manifestation of diabetes mellitus. Diabetes Care 1983; 6:189–192.
Sattar MA, Diab S, Sugathan TN, Sivanandasingham P, Fenech FF. Scleroedema diabeticorum: a minor but often unrecognized complication of diabetes mellitus. Diabet Med 1988; 5:465–468.
Varga J, Gotta S, Li L, Sollberg S, Di Leonardo M. Scleredema adultorum: case report and demonstration of abnormal expression of extracellular matrix genes in skin fibroblasts in vivo and in vitro. Br J Dermatol 1995; 132:992–999.
Seyger MM, van den Hoogen FH, de Mare S, van Haelst U, de Jong EM. A patient with a severe scleroedema diabeticorum, partially responding to low-dose methotrexate. Dermatology 1999; 198:177–179.
Lee MW, Choi JH, Sung KJ, Moon KC, Koh JK. Electron beam therapy in patients with scleredema. Acta Derm Venereol 2000; 80:307–308.
Bowen AR, Smith L, Zone JJ. Scleredema adultorum of Buschke treated with radiation. Arch Dermatol 2003; 139:780–784.
Beers WH, Ince A, Moore TL. Scleredema adultorum of Buschke: a case report and review of the literature. Semin Arthritis Rheum 2006; 35:355–359.

References

Cole GW, Headley J, Skowsky R. Scleredema diabeticorum: a common and distinct cutaneous manifestation of diabetes mellitus. Diabetes Care 1983; 6:189–192.
Sattar MA, Diab S, Sugathan TN, Sivanandasingham P, Fenech FF. Scleroedema diabeticorum: a minor but often unrecognized complication of diabetes mellitus. Diabet Med 1988; 5:465–468.
Varga J, Gotta S, Li L, Sollberg S, Di Leonardo M. Scleredema adultorum: case report and demonstration of abnormal expression of extracellular matrix genes in skin fibroblasts in vivo and in vitro. Br J Dermatol 1995; 132:992–999.
Seyger MM, van den Hoogen FH, de Mare S, van Haelst U, de Jong EM. A patient with a severe scleroedema diabeticorum, partially responding to low-dose methotrexate. Dermatology 1999; 198:177–179.
Lee MW, Choi JH, Sung KJ, Moon KC, Koh JK. Electron beam therapy in patients with scleredema. Acta Derm Venereol 2000; 80:307–308.
Bowen AR, Smith L, Zone JJ. Scleredema adultorum of Buschke treated with radiation. Arch Dermatol 2003; 139:780–784.
Beers WH, Ince A, Moore TL. Scleredema adultorum of Buschke: a case report and review of the literature. Semin Arthritis Rheum 2006; 35:355–359.