scleredema (Buschke disease)

Introduction

A a misnomer because neither sclerosis nor edema is a feature.

Etiology

• antecedent febrile illness
  • following streptococcal infection in children
• diabetes mellitus
• blood dyscrasia - monoclonal gammopathy, multiple myeloma

Epidemiology

• uncommon

Pathology

• generally benign, self-limited skin disorder
• may involve the viscera
• deposition of mucin between dermal collagen bundles, greatest in the deep dermis

Clinical manifestations

• nonpitting induration of the skin with occasional erythema
• initial skin changes occur on the face, the neck, or the upper back
• patients may report difficulty smiling, opening their mouths, &/or wrinkling their foreheads
• skin changes tend to spread distally, but are usually confined to the upper part of the body
• patients with tongue involvement may complain of dysarthria

Laboratory

• throat culture to rule out group A Streptococcal pharyngitis
• antistreptolysin O titer
• fasting serum glucose or erythrocyte hemoglobin A1c to rule out diabetes mellitus
• serum protein electrophoresis to exclude monoclonal gammopathy

Complications

• rarely results in death

Management

• treat infection or other underlying disorder
• no therapy consistently effective

More general terms

• skin disease (dermatologic disorder, dermatopathy, dermatosis)

More specific terms

• diabetic scleredema

Additional terms

• scleroderma (diffuse cutaneous systemic sclerosis)
• scleromyxedema; lichen myxedematosus; papular mucinosis