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scleredema (Buschke disease)

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

Related

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

Specific

diabetic scleredema

General

skin disease (dermatologic disorder, dermatopathy, dermatosis)

References

  1. Rosenbach MA and Elston DM eMedicine: Scleredema http://emedicine.medscape.com/article/1066175-overview
  2. Wikipedia: Scleredema http://en.wikipedia.org/wiki/Scleredema
  3. DermNet NZ: Scleredema http://dermnetnz.org/immune/scleredema.html
  4. Medical Knowledge Self Assessment Program (MKSAP) 17, American College of Physicians, Philadelphia 2015