eMedicine Specialties > Dermatology > Diseases of the Dermis
Lichen Myxedematosus: Treatment & Medication
Updated: Jan 14, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
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,3 electron beams, high-dose dexamethasone, psoralen UV-A (PUVA), plasmapheresis, extracorporeal photophoresis, dermabrasion, and carbon dioxide laser excision.4 More recently, improvements have been reported with the use of thalidomide.5
- Topical tacrolimus reportedly has been successful in treating localized disease in a few patients.6
- 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.7
- 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.8
Consultations
Patients should be referred to an internal medicine specialist for a thorough physical examination.
Medication
Several chemotherapeutic agents have been tried9 ; of these, the most common is melphalan.
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.
Melphalan (Alkeran)
Inhibits mitosis by cross-linking DNA strands.
Adult
2-4 mg/d; may use cyclic protocol with 10 mg/d and prednisone for 7 d, repeat q6wk
Pediatric
Not established
Concurrent administration with cyclosporine increases nephrotoxicity; cimetidine and H2 antagonists increase gastric pH, decreasing effects
Documented hypersensitivity; severe bone marrow depression
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Bone marrow suppression is common adverse effect; patients must receive close medical supervision; advise patients about suppression of ovarian function and impairment of fertility; caution in previously diagnosed myelosuppression; amenorrhea and secondary malignancies including (eg, myeloproliferative syndromes, and leukemias) possible
Corticosteroids
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.
Prednisone (Deltasone, Orasone, Meticorten)
For use in cyclic protocol with melphalan. Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocytes and antibody production.
A recent case report describes systemic corticosteroid therapy; improvement in the skin was noted within 4 wk.
Adult
15 mg/d for 7 d, repeat q6wk
Pediatric
Not established
Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI disease
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
Thalidomide (Kevadon)
Marked improvement occurred in 3 patients within 2 mo and continued improvement after 4 mo. GI upset may be reduced with divided doses.
Adult
100 mg PO qd/bid, preferably qhs and with water; increase to 300 mg/d if necessary
Pediatric
Not established
May increase sedation of alcohol, barbiturates, chlorpromazine, and reserpine
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
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
More on Lichen Myxedematosus |
| Overview: Lichen Myxedematosus |
| Differential Diagnoses & Workup: Lichen Myxedematosus |
 Treatment & Medication: Lichen Myxedematosus |
| Follow-up: Lichen Myxedematosus |
| Multimedia: Lichen Myxedematosus |
| References |
| « Previous Page | Next Page » |
References
Montgomery H, Underwood LJ. Lichen myxedematosus; differentiation from cutaneous myxedemas or mucoid states. J Invest Dermatol. Mar 1953;20(3):213-36. [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].
Hill TG, Crawford JN, Rogers CC. Successful management of lichen myxedematosus. Report of a case. Arch Dermatol. Jan 1976;112(1):67-9. [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].
Jacob SE, Fien S, Kerdel FA. Scleromyxedema, a positive effect with thalidomide. Dermatology. 2006;213(2):150-2. [Medline].
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].
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].
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].
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].
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].
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].
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].
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].
Further Reading
Keywords
papular mucinosis, scleromyxedema, myxedematosus, plasma cell dyscrasia
