eMedicine Specialties > Dermatology > Diseases of the Dermis

Lichen Myxedematosus

Author: Elizabeth A Liotta, MD, Assistant Professor, Department of Dermatology, Uniformed Services University of the Health Sciences
Contributor Information and Disclosures

Updated: Jan 14, 2009

Introduction

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

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 follows1 :
    • 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.

Causes

More on Lichen Myxedematosus

Overview: Lichen Myxedematosus
Differential Diagnoses & Workup: Lichen Myxedematosus
Treatment & Medication: Lichen Myxedematosus
Follow-up: Lichen Myxedematosus
Multimedia: Lichen Myxedematosus
References
[ CLOSE WINDOW ]

References

  1. Montgomery H, Underwood LJ. Lichen myxedematosus; differentiation from cutaneous myxedemas or mucoid states. J Invest Dermatol. Mar 1953;20(3):213-36. [Medline].

  2. 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].

  3. Hill TG, Crawford JN, Rogers CC. Successful management of lichen myxedematosus. Report of a case. Arch Dermatol. Jan 1976;112(1):67-9. [Medline].

  4. 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].

  5. Jacob SE, Fien S, Kerdel FA. Scleromyxedema, a positive effect with thalidomide. Dermatology. 2006;213(2):150-2. [Medline].

  6. Rongioletti F, Zaccaria E, Cozzani E, Parodi A. Treatment of localized lichen myxedematosus of discrete type with tacrolimus ointment. J Am Acad Dermatol. Mar 2008;58(3):530-2. [Medline].

  7. 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].

  8. 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].

  9. 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].

  10. Chanda JJ. Scleromyxedema. Cutis. Nov 1979;24(5):549-52. [Medline].

  11. Dinneen AM, Dicken CH. Scleromyxedema. J Am Acad Dermatol. Jul 1995;33(1):37-43. [Medline].

  12. 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].

  13. Kovary 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].

  14. Lang E, Goos M. [Internal disease findings in Arndt-Gottron scleromyxedema]. Dtsch Med Wochenschr. May 23 1986;111(21):820-3. [Medline].

  15. 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].

  16. Picascia DD, Magid ML, Minkin RB. Pruritic papular eruption. Papular mucinosis (lichen myxedematosus). Arch Dermatol. Jul 1989;125(7):986-7, 990. [Medline].

  17. 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].

  18. Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol. Feb 2001;44(2):273-81. [Medline].

  19. 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].

  20. Wright RC, Franco RS, Denton D, Blaney DJ. Scleromyxedema. Arch Dermatol. Jan 1976;112(1):63-6. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

papular mucinosis, scleromyxedema, myxedematosus, plasma cell dyscrasia

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Elizabeth A Liotta, MD, Assistant Professor, Department of Dermatology, Uniformed Services University of the Health Sciences
Elizabeth A Liotta, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Mark W Cobb, MD, Consulting Staff, WNC Dermatological Associates
Mark W Cobb, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society of Dermatopathology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine 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.

RELATED EMEDICINE ARTICLES
 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.