Medical Care

Treatment is unnecessary for postinfectious scleredema as it is typically self-limited. However, in rapidly progressive, fulminant cases, systemic therapy is recommended.^[32]Physical therapy, intravenous immunoglobulin, or UVA-1 phototherapy could be considered.

Types 2 and 3 scleredema associated with a monoclonal gammopathy or diabetes typically does not regress spontaneously, and no therapy is consistently effective. Most therapeutic successes described are limited to single case reports or small case series; there are no comparative data and no approved algorithm for scleredema treatment. Expert opinion also differs. In general, it is agreed that treatment of an identified cause is warranted. For type 1 scleredema, antimicrobial agents are used if indicated. For type 2 scleredema, annual screening for a lymphoproliferative disorder, and for those with a monoclonal gammopathy, referral to a hematologist are, indicated. Physical therapy should be considered for all types. However, expert opinion often differs based on personal experience. In a 2017 publication of the European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, first-line recommendations were UVA-1 phototherapy or psoralen with UVA (PUVA).^[33]

Treatments described in case reports and small case series

A number of therapies, including systemic steroids,^{[26, 34, 35]}cyclosporine,^{[26, 36]}methotrexate,^[37]high-dose penicillin,^{[15, 26]}penicillamine, electron beam,^{[38, 39]}and glycemic control with prostaglandin E₁ (PGE₁)^[14]have been reported with mixed success. A report using tamoxifen for its antifibrotic properties noted improvements in two patients.^[40]Incidental improvement was noted in one case of scleredema during allopurinol therapy.^[41]A 2018 report described three cases of successful treatment with tranilast.^[42]

Case reports in the literature describe improvement with UVA-1 phototherapy,^{[26, 43, 44]}narrow-band UVB phototherapy,^{[45, 46]}and PUVA either administered systemically or topically.^{[26, 47, 48, 49, 50]}Case reports also describe these successfully used in combination with colchicine,^{[51, 52]}methotrexate,^[53]and physiotherapy.^[54]In the authors’ experience, narrow-band UVB phototherapy was not successful.

Physical modalities, such as ultrasonic massage with physical therapy, may improve range of motion and quality of life for some patients alone or in combination with other therapies.^[55]

In cases associated with myeloma, chemotherapy directed at the hematologic malignancy has been reported to result in concomitant improvement of the skin disease.^[56]For patients with paraproteinemia, extracorporeal photophoresis has been used.^[57]

Multiple reports described efficacy with intravenous immunoglobulin therapy in postinfectious, diabetic, and monoclonal gammopathy–associated scleredema that were refractory to more standard therapies.^{[58, 59, 60]}

A 2015 multicenter retrospective review of associations and treatment response among 44 patients was published, finding some response to physiotherapies, systemic steroids, UVA-1, PUVA, and a few combination therapies in a limited number of patients.^[26]

Treatment of associated conditions

Appropriate antibiotic therapy should be started in scleredema patients if infection is detected, although antibiotics do not appear to shorten the course of skin findings in scleredema.^[61]

Treatment of detected blood dyscrasias or diabetes mellitus should be completed.

Consultations

Consultation with a dermatologist to confirm the diagnosis is recommended.

Consultation with a hematologist may be necessary. Refer patients in cases of detected blood dyscrasias and/or monoclonal gammopathies.

Consultation with a physical therapist may be necessary. Refer patients with range-of-motion difficulties. Prompt attention to physical therapy can potentially reduce long-term range-of-motion limitations.

Diet

No restrictions are necessary.

Activity

No restrictions are necessary.

Long-Term Monitoring

Patients with longstanding scleredema should be periodically monitored using the results of serum protein and immunoprotein electrophoresis to detect the development of paraproteinemia or myeloma. Blood dyscrasias may occur several years after the onset of scleredema.

Medication

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Media Gallery

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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Author

Lisa K Pappas-Taffer, MD Assistant Professor of Clinical Dermatology, University of Pennsylvania School of Medicine; Attending Physician, Hospital of the University of Pennsylvania; Staff Physician, Veterans Affairs Medical Center

Lisa K Pappas-Taffer, MD is a member of the following medical societies: American Academy of Dermatology, Association of Professors of Dermatology, Atlantic Dermatologic Society, Medical Dermatology Society, Philadelphia Dermatological Society, Rheumatologic Dermatology Society

Disclosure: Nothing to disclose.

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, Phi Beta Kappa, Society for Investigative Dermatology, Medical Dermatology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster 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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

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, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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

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

Disclosure: Nothing to disclose.