eMedicine Specialties > Dermatology > Connective Tissue Diseases

Scleredema

Author: Misha A Rosenbach, MD, Staff Physician, Departments of Medicine and Dermatology, Hospital of the University of Pennsylvania
Coauthor(s): Victoria P Werth, MD, Professor of Dermatology and Medicine, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, Philadelphia Veterans Administration Hospital; Dina D Strachan, MD, Assistant Clinical Professor, Department of Dermatology, St Vincent's Medical Center
Contributor Information and Disclosures

Updated: Oct 7, 2008

Introduction

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.

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.
• 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.

Clinical

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.
  • 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.

Physical

• 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.

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.

• Febrile illness
  • Streptococcal infections^8,9 : 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.¹⁰
• Myelomatous disorders^11,12 : 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^13,14 : 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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