Scleredema Clinical Presentation

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

History

The initial skin changes of scleredema occur on the face, the neck, or the upper part of the back. Patients may report difficulty in smiling, in opening their mouths, and in wrinkling their foreheads. They may have limited range of motion. The skin changes tend to spread distally, and they are usually confined to the upper part of the body. However, a case of scleredema localized on the thighs has been reported.[7] Hands and feet are typically spared. Scleredema patients with tongue involvement may report dysarthria.

Scleredema can be categorized into 3 clinical subgroups. Each has a different history, course, and prognosis. Note the following:

  • Group 1 includes scleredema after acute respiratory infection (scleredema adultorum). Patients in group 1 have a history of a preceding febrile illness, particularly an upper respiratory tract streptococcal infection. The onset of the skin lesions is rapid, and the condition usually clears spontaneously in 6 months to 2 years. The duration is not affected by the use of antibiotics. The term scleredema adultorum is considered by some to be a misnomer because most pediatric patients fall into this group.
  • Group 2 includes scleredema patients whose disease tends to occur insidiously, with no preceding illness. This group encompasses cases associated with a monoclonal gammopathy.
  • Group 3 is scleredema associated with diabetes mellitus (scleredema diabeticorum) and includes patients with preexisting diabetes, which is typically adult in onset and often type 1. This disorder tends to occur more often in males, and this subgroup of patients typically experiences a more protracted course that is refractory to therapy. As in group 2, the onset of skin lesions is insidious. The upper back typically demonstrates erythema and induration. A pebbled appearance may evolve.
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Physical

Physical findings are as follows:

  • Primary lesion: The lesions of scleredema are ill-defined, woody, nonpitting, indurated plaques. Generalized cases may clinically mimic edema. Erythema, hyperpigmentation, and/or a peau d'orange appearance of the affected areas may be present. The epidermis overlying the indurated areas wrinkles when pinched.
  • Distribution: Scleredema is usually most evident in the upper part of the body, specifically the face, the neck, the trunk, and the extremities. However, a case of scleredema confined to the thighs has been reported.[7] Hands and feet are typically spared in scleredema. Scleredema patients with extensive facial involvement may appear expressionless and may have difficulty in opening their mouths. Scleredema patients may have difficulty with tongue protrusion. These patients may also report dysarthria. Tongue involvement helps distinguish scleredema from scleroderma, which never affects the tongue.
  • Color: Affected skin may be flesh-colored, erythematous, or hyperpigmented (diffuse in one report).[8]
  • Note the images below:Middle-aged man who has diabetes with scleredema oMiddle-aged man who has diabetes with scleredema on the upper part of the back. Middle-aged man who has diabetes with scleredema oMiddle-aged man who has diabetes with scleredema on the upper part of the back.
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Causes

The cause of scleredema is unknown; however, it is associated with a number of conditions. Many patients have no history of an acute antecedent illness. Note the following:

  • Febrile illness: Streptococcal infections[9, 10] : An upper respiratory tract infection (typically pharyngitis) is the most common cause of scleredema in patients in group 1.
  • Other infections: The onset of scleredema has also been associated with cytomegalovirus, influenza, measles, pertussis, mumps, diphtheria, typhus fever, encephalitis, and dental abscesses.
  • Trauma: Rare reports exist of scleredema occurring after trauma to the affected area. One report has described geometric scleredema due to mechanical stress.[11]
  • Myelomatous disorders[12, 13] : A subset of patients in group 2 has shown a well-established relationship between scleredema with paraproteins and multiple myeloma. One analysis of 52 patients with group 2 scleredema revealed 25% had plasma cell dyscrasia, including 3 patients with multiple myeloma and 10 with monoclonal gammopathy of unknown significance.
  • Diabetes mellitus[14, 15] : Patients usually have a long-standing history of diabetes, which tends to be type 1 and difficult to control. Diabetes-associated scleredema persists indefinitely and is unaffected by insulin therapy.
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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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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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