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Lichen Myxedematosus Clinical Presentation

  • Author: Elizabeth A Liotta, MD; Chief Editor: Dirk M Elston, MD  more...
Updated: Jul 15, 2016


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.


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.[1, 2]

Montgomery and Underwood described four clinical presentations and grouped the different manifestations in a classification system as follows[3] :

  • Generalized lichenoid papular form
  • Discrete papular form
  • Localized or generalized lichenoid papules
  • Urticarial plaques and nodular eruptions that usually lead to the lichenoid form


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.

Note the image below.

Grouped, erythematous, flesh-colored, dome-shaped Grouped, erythematous, flesh-colored, dome-shaped papules are present on the hand and fingers.


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.



The etiology is unknown. See Pathophysiology.

Contributor Information and Disclosures

Elizabeth A Liotta, MD Chief Dermatologist and Sole Proprietor, Integrated Skin Care Centers

Elizabeth A Liotta, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

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, Society for Investigative Dermatology

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

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, American Society of Dermatopathology

Disclosure: Nothing to disclose.

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Grouped, erythematous, flesh-colored, dome-shaped papules are present on the hand and fingers.
Low-power photomicrograph of a histopathologic specimen demonstrates the wide separation of the collagen fibers near the large collection of mucin in the dermis.
High-power photomicrograph of a histopathologic specimen reveals the large collections of mucin and plump stellate fibroblasts in the dermis.
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