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Chapter 53: Scleredema

Puneet K. Bhullar; Aaron R. Mangold; Mark R. Pittelkow

SCLEREDEMA

Therapeutic Approach

Scleredema is a rare connective tissue disease of unknown etiology, which clinically presents with diffuse symmetric woody skin induration and nonpitting edema. The course of postinfectious scleredema does not seem to be affected by antibiotics. Treating the associated diseases by improving glucose control or treatment of paraproteinemia may lead to improvement of scleredema. Treatment may not be necessary unless the disease is symptomatic or causing functional issues. Our preferred agent is phototherapy.1-3 Physical therapy may be of benefit individuals with functional issues. Monoclonal gammopathy-associated scleredema often responds to intravenous immunoglobulin and/or extracorporeal photophoresis. There has been benefit shown with local radiotherapy or electron-beam irradiation. Other immunosuppressive such as methotrexate are of limited value. See Table 53-1.

Table 53-1Scleredema Treatment Table
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Table 53-1 Scleredema Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Procedural Therapy
Ultraviolet A1 phototherapy   Induce collagenase Started at 10 J/cm2 × 1 treatment, increased to 15 J/cm2 × 1 treatment, and then increased and held at 20 J/cm2 for the next 28 treatments for a total of 30 treatments. Risk of carcinogenesis None III1-4
Psoralen plus ultraviolet A phototherapy (PUVA)   Lymphocytotoxic effect

Initial: 0.5-0.6 J/cm2

Oral 8-MOP: 0.6 mg/kg-0.8 mg/kg

Frequency: 2-3 times per week

Dose titration: 10%-20% every 72 h

 Burning, erythema, pruritus, nausea and headache, chronic actinic skin damage, dyskeratotic and precancerous skin conditions, nonmelanoma skin cancer, immunological alterations and cataract formation Annual skin examination, eye examination III
Electron-beam irradiation   Causing damage to their DNA 7-9 MeV of 20-24 Gy in 10-12 fractions Fatigue, itching, tanning, and burns None III5
Extracorporeal photopheresis   Apoptosis of treated leukocytes 2 d/mo until disease improvement Shortness of breath, coughing, or raised blood pressure None III6
Intravenous immunoglobulin   Downregulate antibody production by B cells 2 g/kg (over 2-5 d) every 4 wk until disease improvement Flushing, headache, malaise, fever, chills, fatigue and lethargy, renal impairment, thrombosis, arrhythmia, hemolytic anemia, and transfusion-related acute lung injury None III7
Narrowband UVB phototherapy   Suppression of T-cell responses; induces T-cell apoptosis 50%-80% minimal erythema dose, 3 times per week until remission, then taper over 3-6 wk Skin: transient erythema and pruritis None III8,9

No FDA-approved therapy.

Levels of evidence are based on the Journal of the American Academy of Dermatology guidelines: level IA evidence includes evidence from meta-analysis of randomized controlled trials; level IB evidence includes evidence from ≥1 randomized controlled trial; level IIA evidence includes evidence from ≥1 controlled study without randomization; level IIB evidence includes evidence from ≥1 other type of experimental study; level III evidence includes evidence from nonexperimental descriptive studies, such as comparative studies, correlation studies, and case control studies; and level IV evidence includes evidence from expert committee reports or opinions ...

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