Scleredema 

  • Author: Misha A Rosenbach, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jun 17, 2010
 

Background

Scleredema is an uncommon condition of unknown etiology. Scleredema is characterized by a nonpitting induration of the skin with occasional erythema. Scleredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. Although regarded as a benign, self-limited, skin disease, scleredema may be persistent and involve the viscera. Rarely, it may result in death.

The term scleredema is a misnomer because neither sclerosis nor edema is found on microscopic examination. The histologic findings of scleredema include deposition of mucin between dermal collagen bundles. The deposition is greatest in the deep dermis.

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Pathophysiology

The etiology of scleredema is unknown. Scleredema localized to the back is commonly associated with diabetes mellitus, while generalized scleredema may follow a viral illness. Comparative quantitative polymerase chain reaction (PCR) analysis of lesional and nonlesional skin in patients with scleredema has demonstrated an increase in collagen gene expression in affected sites.[1] Fibroblast culture of affected scleredema skin has shown increased procollagen synthesis. Likewise, serum from patients with scleredema has been shown to stimulate collagen production in normal skin fibroblasts.

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Epidemiology

Frequency

United States

Scleredema is rare, although diabetes-related scleredema is likely underreported.

International

Scleredema is rare.

Mortality/Morbidity

The morbidity of the skin changes of scleredema depends on the area of the body involved. Involvement of the skin over the joints may cause limited range of motion. Scleredema on the face can result in difficulty in opening the eyes and the mouth. Although rare, extensive truncal involvement may cause restrictive lung disease; one case of fatal scleredema was attributed to pneumonia developing secondary to extensive stiffness of the upper torso as a result of progressive scleredema.[2] Reports of recurrent cellulitis and delayed wound healing in areas of affected skin also exist. Note the following:

  • Unlike scleroderma, the tongue may be involved in scleredema, resulting in dysarthria and difficulty with mastication and tongue protrusion.
  • Cardiac involvement in scleredema patients is rare and may result in cardiomyopathy,[3] heart failure, arrhythmias, pericardial effusion, and unexplained murmurs.
  • Other organs that may be involved in scleredema include skeletal muscles, ocular muscles, the pharynx, the liver, parotid glands, pleurae, the peritoneum, and the spleen.
  • One case report describes scleredema limited to the periorbital region, which led to partial vision blockage.[4]
  • Esophageal involvement is usually thought to be a feature that distinguishes scleredema, which does not affect the esophagus, from scleroderma, which does affect it. In one report, a patient developed dysphagia presumably from scleredema of the upper part of the esophagus. Esophageal biopsy was not performed on this patient.[5]
  • Death from scleredema is uncommon. However, reports have described patients dying from the complications of visceral involvement and from extensive skin disease.

Race

No racial predilection is reported for scleredema.

Sex

A female preponderance is reported for scleredema, with a female-to-male ratio of 2:1, except in the type associated with adult-onset diabetes, which is more common in men than in women.

Age

Scleredema occurs in individuals of all ages.[6] Although scleredema is sometimes referred to as scleredema adultorum, 50% of scleredema cases occur in individuals younger than 20 years.

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Contributor Information and Disclosures
Author

Misha A Rosenbach, MD  Assistant Professor, Departments of Medicine and Dermatology, Hospital of the University of Pennsylvania

Misha A Rosenbach, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Academy, Medical Dermatology Society, and Women's Dermatologic Society

Disclosure: Centocor Grant/research funds None

Coauthor(s)

Victoria P Werth, MD  Professor of Dermatology and Medicine, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, Philadelphia Veterans Affairs Medical Center

Victoria P Werth, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American College of Rheumatology, Medical Dermatology Society, Phi Beta Kappa, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Susan M Swetter, MD  Director, Pigmented Lesion and Cutaneous Melanoma Clinic, Professor, Department of Dermatology, Stanford University Medical Center, Veterans Affairs Palo Alto Health Care System

Susan M Swetter, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society of Clinical Oncology, Eastern Cooperative Oncology Group, Pacific Dermatologic Association, Society for Investigative Dermatology, Society for Melanoma Research, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Lester F Libow, MD  Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Texas Medical Association

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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  14. Ikeda Y, Suehiro T, Abe T, et al. Severe diabetic scleredema with extension to the extremities and effective treatment using prostaglandin E1. Intern Med. Oct 1998;37(10):861-4. [Medline].

  15. Krasagakis K, Hettmannsperger U, Trautmann C, Tebbe B, Garbe C. Persistent scleredema of Buschke in a diabetic: improvement with high-dose penicillin. Br J Dermatol. Mar 1996;134(3):597-8. [Medline].

  16. Rota E, Nallino MG, Bainotti S, Formica M. Nephrogenic systemic fibrosis: an unusual scleroderma-like fibrosing disorder. Rheumatol Int. Aug 20 2009;[Medline].

  17. Manchanda Y, Das S, Sharma VK, Srivastava DN. Scleredema associated with carcinoma of the gall bladder. Br J Dermatol. Jun 2005;152(6):1373-4. [Medline].

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  23. Eberlein-Konig B, Vogel M, Katzer K, et al. Successful UVA1 phototherapy in a patient with scleredema adultorum. J Eur Acad Dermatol Venereol. Mar 2005;19(2):203-4. [Medline].

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  25. Hager CM, Sobhi HA, Hunzelmann N, et al. Bath-PUVA therapy in three patients with scleredema adultorum. J Am Acad Dermatol. Feb 1998;38(2 Pt 1):240-2. [Medline].

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Middle-aged man who has diabetes with scleredema on the upper part of the back.
Middle-aged man who has diabetes with scleredema on the upper part of the back.
 
 
 
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