AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Background

The terms lichen myxedematosus, papular mucinosis, and scleromyxedema are used interchangeably to describe the same disorder. A spectrum of disease appears to exist, with the more localized, less severe forms, which are generally called lichen myxedematosus or papular mucinosis, and the more sclerotic, diffuse form, which is referred to as scleromyxedema. Lichen myxedematosus is a rare skin disorder characterized by fibroblast proliferation and mucin deposition in the dermis, in the absence of thyroid disease.

Pathophysiology

The etiology is unknown; however, the disease is commonly associated with plasma cell dyscrasia. The basic defect is hypothesized to be a fibroblast disorder, which causes the increased mucin deposition in the skin. Most patients have a monoclonal paraprotein band, usually of the immunoglobulin G (IgG) type. The association between this paraprotein and the mucin deposition is not clear, and the protein does not directly stimulate fibroblast proliferation.

Mortality/Morbidity

Lichen myxedematosus is usually a chronic disease. Although most patients have a monoclonal paraproteinemia, they rarely have associated multiple myeloma. However, when myeloma is present, the patient generally has a poor prognosis. Patients with cardiac or pulmonary involvement also have poor prognoses.

Race

Persons of any race can be affected.

Sex

No sex-related predilection is reported.

Age

Most individuals with lichen myxedematosus are aged 30-70 years.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

History

• Lichen myxedematosus or papular mucinosis
• • Patients report a slow onset of asymptomatic or mildly pruritic papules, which may be localized or generalized.
  • Patients are otherwise healthy and do not have systemic symptoms.
• Scleromyxedema
• • Patients with this form present with more widespread progressive induration and decreased mobility of the face, fingers, and extremities.
  • Patients are also noted to have cysts and urticarial lesions.
  • Patients may report systemic symptoms, such as dysphagia or weakness, and symptoms that resemble those of organic brain disease.
• Montgomery and Underwood described 4 clinical presentations and grouped the different manifestations in a classification system as follows:
• • Generalized lichenoid papular form
  • Discrete papular form
  • Localized or generalized lichenoid papules
  • Urticarial plaques and nodular eruptions that usually lead to the lichenoid form

Physical

• Lichen myxedematosus, or papular mucinosis
• • The primary lesion is a 2- to 4-mm, dome-shaped, and flesh-colored or erythematous papule.
  • Regarding the distribution, these lesions may coalesce into grouped lichenoid papules and are found on the dorsal hands, face, or extensor surfaces of the arms and legs.
  • Papules often have a striking pattern of parallel ridges.
  • In patients with the generalized lichenoid form, facial ridges and facial folds may be distorted; this condition is called leonine faces. Patients with leonine faces may have difficulty opening their mouths.
• Scleromyxedema
• • The primary lesions may involve widespread erythematous, indurated skin that resembling scleroderma, with diffuse tightness of the skin.
  • The range of motion of the face, fingers, and extremities is decreased.
  • The systemic manifestations include restrictive and obstructive pulmonary dysfunction, cardiovascular abnormalities, and polyarthritis. Obstructive and restrictive lung disease is often manifested by dyspnea on exertion.
  • Gastrointestinal symptoms (most commonly dysphagia) are related to esophageal aperistalsis.
  • Severe proximal muscle weakness, polyarthritis, and symptoms resembling those of organic brain disease are present. Inflammatory myopathy is also reported.
  • Ophthalmologic symptoms include ectropion and corneal opacities.
  • Cardiovascular abnormalities occur in 10% of cases.
  • One study revealed that digital vasoreactivity is the most common vascular abnormality.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Other Problems to be Considered

Progressive systemic sclerosis

Leonine facies: The differential diagnosis of this condition includes mycosis fungoides, mastocytosis, amyloid, lepromatous leprosy, lichen myxedematosus, actinic reticuloid, leishmaniasis, lipoid proteinosis, and progressive nodular histiocytosis.

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Lab Studies

• Serum protein immunoelectrophoresis generally reveals the presence of a serum paraprotein (usually 7S-IgG) with lambda light chains.
• Few patients may have myeloma or Waldenström macroglobulinemia.
• Thyroid function test results are normal.
• Findings of other laboratory test are usually normal.

Imaging Studies

• Chest radiographic findings are usually normal in healthy patients.

Other Tests

• In patients with systemic symptoms such as dysphagia, dyspnea, or neurologic symptoms, the specific systems affected should be evaluated.

Histologic Findings

Lesions have large depositions of mucin in the dermis. Numerous plump stellate fibroblasts develop throughout the dermis. The mucin stains with periodic acid-Schiff and Alcian blue at pH 2.5 but not pH 0.4, and it metachromatically stains with toluidine blue at pH 3.0. The mucin has been identified as hyaluronic acid, a nonsulfated acid mucopolysaccharide.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Medical Care

The treatment of lichen myxedematosus is difficult and often ineffective. Many therapeutic approaches have been tried.

• These approaches include treatment with retinoids, orthovoltage radiation, electron beams, high-dose dexamethasone, psoralen UV-A (PUVA), plasmapheresis, extracorporeal photophoresis, dermabrasion, and carbon dioxide laser excision. More recently, improvements have been reported with the use of thalidomide.
• Various reports in the literature describe treatment successes and failures with these methods.
• A 2004 article reported improvement with thalidomide in 3 patients in whom other therapies had failed. All 3 showed marked improvement of skin lesions within the first 2 months of therapy and continued improvement after 4 months of therapy. The doses of thalidomide ranged from 100 mg at bedtime to 400 mg in divided doses; the adverse effects were better tolerated at the 200-mg/d dose in divided doses.
• • Adverse effects of thalidomide most commonly include mild sedation and constipation.
  • Thalidomide is also teratogenic.
  • Finally, nerve conductions studies have revealed the occurrence of peripheral neuropathy in approximately 25% of patients. In a different 2004 study, high-dose dexamethasone was helpful in 1 patient, who received the drug for 3 weeks, after which it was tapered to a lower dose for 4 months.

Consultations

Patients should be referred to an internal medicine specialist for a thorough physical examination.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Several chemotherapeutic agents have been tried; of these, the most common is melphalan.

Drug Category: Alkylating agents

Successful short-term improvements are reported with melphalan treatment; however, in one study, a significant number of cases had fatal complications due to hematologic malignancies and sepsis.

Drug Category: Corticosteroids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Drug Name	Thalidomide (Kevadon)
Description	Marked improvement occurred in 3 patients within 2 mo and continued improvement after 4 mo. GI upset may be reduced with divided doses.
Adult Dose	100 mg PO qd/bid, preferably qhs and with water; increase to 300 mg/d if necessary
Pediatric Dose	Not established
Contraindications	Documented hypersensitivity; sexually active males not using latex condom (risk to fetus from semen of patients taking thalidomide unknown), women of childbearing potential not using 2 forms of contraception
Interactions	May increase sedation of alcohol, barbiturates, chlorpromazine, and reserpine
Pregnancy	X - Contraindicated; benefit does not outweigh risk
Precautions	Perform pregnancy test within 24 prior to initiating therapy (qwk during first month, followed by qmo in women with regular menstrual cycles or q2wk with irregular menstrual cycles); bradycardia may occur; use protective measures (eg, sunscreens, protective clothing) against exposure to sunlight or UV light (eg, tanning beds); prescribing physician must register with the "System for Thalomid Education and Prescribing Safety" (STEPS) program established by manufacturer

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Further Outpatient Care

• Patients should undergo periodic follow-up.
• Patients should be referred to specialists for evaluation as needed for specific systemic complaints.

Prognosis

• For lichen myxedematosus, the prognosis is a chronic course with little tendency for spontaneous resolution.
• Scleromyxedema usually has a poorer prognosis than that of the other forms. Typical causes of death include complications of systemic therapeutic agents such as melphalan or systemic disease such as cardiovascular involvement.
• Although most patients have a monoclonal paraproteinemia, they rarely have associated multiple myeloma. However, when myeloma is present, the patient generally has a poor prognosis.
• Patients with cardiac or pulmonary involvement also have a poor prognosis.

Patient Education

• Patients with lichen myxedematosus have a long-term, disfiguring, possibly disabling disorder. The risks and benefits of systemic therapy must be weighed carefully.

MULTIMEDIA

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Media file 1:  Grouped, erythematous, flesh-colored, dome-shaped papules are present on the hand and fingers.
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Media file 2:  Low-power photomicrograph of a histopathologic specimen demonstrates the wide separation of the collagen fibers near the large collection of mucin in the dermis.
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Media type:  Photo

Media file 3:  High-power photomicrograph of a histopathologic specimen reveals the large collections of mucin and plump stellate fibroblasts in the dermis.
	View Full Size Image
Media type:  Photo

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

• Chanda JJ. Scleromyxedema. Cutis. Nov 1979;24(5):549-52. [Medline].
• Dinneen AM, Dicken CH. Scleromyxedema. J Am Acad Dermatol. Jul 1995;33(1):37-43. [Medline].
• Gonzalez J, Palangio M, Schwartz J, Klainer AS, Bisaccia E. Scleromyxedema with dermato-neuro syndrome. J Am Acad Dermatol. May 2000;42(5 Pt 2):927-8. [Medline].
• Hill TG, Crawford JN, Rogers CC. Successful management of lichen myxedematosus. Report of a case. Arch Dermatol. Jan 1976;112(1):67-9. [Medline].
• Horn KB, Horn MA, Swan J, Singhal S, Guitart J. A complete and durable clinical response to high-dose dexamethasone in a patient with scleromyxedema. J Am Acad Dermatol. Aug 2004;51(2 Suppl):S120-3. [Medline].
• Howsden SM, Herndon JH Jr, Freeman RG. Lichen myxedematosus. A dermal infiltrative disorder responsive to cyclophosphamide therapy. Arch Dermatol. Oct 1975;111(10):1325-30. [Medline].
• Jacob SE, Fien S, Kerdel FA. Scleromyxedema, a positive effect with thalidomide. Dermatology. 2006;213(2):150-2. [Medline].
• Kaymen AH, Nasr A, Grekin RC. The use of carbon dioxide laser in lichen myxedematosus. J Dermatol Surg Oncol. Aug 1989;15(8):862-5. [Medline].
• Kövary PM, Vakilzadeh F, Macher E, Zaun H, Merk H, Goerz G. Monoclonal gammopathy in scleredema. Observations in three cases. Arch Dermatol. Sep 1981;117(9):536-9. [Medline].
• Lang E, Goos M. [Internal disease findings in Arndt-Gottron scleromyxedema]. Dtsch Med Wochenschr. May 23 1986;111(21):820-3. [Medline].
• Lin YC, Wang HC, Shen JL. Scleromyxedema: An experience using treatment with systemic corticosteroid and review of the published work. J Dermatol. Mar 2006;33(3):207-10. [Medline].
• Montgomery H, Underwood LJ. Lichen myxedematosus; differentiation from cutaneous myxedemas or mucoid states. J Invest Dermatol. Mar 1953;20(3):213-36. [Medline].
• Picascia DD, Magid ML, Minkin RB. Pruritic papular eruption. Papular mucinosis (lichen myxedematosus). Arch Dermatol. Jul 1989;125(7):986-7, 990. [Medline].
• Rongioletti F. Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg. Jun 2006;25(2):100-4. [Medline].
• Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol. Feb 2001;44(2):273-81. [Medline].
• Sansbury JC, Cocuroccia B, Jorizzo JL, Gubinelli E, Gisondi P, Girolomoni G. Treatment of recalcitrant scleromyxedema with thalidomide in 3 patients. J Am Acad Dermatol. Jul 2004;51(1):126-31. [Medline].
• Truhan AP, Roenigk HH Jr. Lichen myxedematosus. An unusual case with rapid progression and possible internal involvement. Int J Dermatol. Mar 1987;26(2):91-5. [Medline].
• Wright RC, Franco RS, Denton D, Blaney DJ. Scleromyxedema. Arch Dermatol. Jan 1976;112(1):63-6. [Medline].
• Yaron M, Yaron I, Yust I, Brenner S. Lichen myxedematosus (scleromyxedema) serum stimulates hyaluronic acid and prostaglandin E production by human fibroblasts. J Rheumatol. Feb 1985;12(1):171-5. [Medline].

Article Last Updated: Sep 20, 2006