eMedicine Specialties > Dermatology > Metabolic Diseases

Amyloidosis, Lichen: Treatment & Medication

Author: Sultan Al-Khenaizan, MBBS, FRCP(C), Consulting Staff, Departments of Dermatology and Internal Medicine, King Fahad National Guard Hospital, Saudi Arabia
Contributor Information and Disclosures

Updated: Nov 6, 2009

Treatment

Medical Care

Because of the growing appreciation of the importance of pruritus as the primary trigger for the deposition of amyloid, treatment modalities are directed toward the relief of pruritus.

  • Sedating antihistamines have been found to be moderately effective.
  • Menthol, in combination with other agents (eg, antihistamines), has been used successfully to relieve the pruritus associated with lichen amyloidosis.15
  • Topical and intralesional steroids are beneficial if combined with other modalities. Costanedo-Cazares et al reported improvement in lichen amyloidosis using treatment with 0.1% topical tacrolimus ointment.16
  • Topical dimethyl sulfoxide (DMSO), a chemical solvent, was used with moderate success, but failures are also reported.17,18,19 Pandhi et al reported a lack of effect with DMSO treatment for cutaneous amyloidosis.20
  • Anecdotes of both success and failure with etretinate have been reported.21,22,23 Acitretin, a prodrug of etretinate, has been used successfully to relieve pruritus and flatten hyperkeratotic papules in 2 patients with lichen amyloidosis.24
  • Sawamura et al reported satisfying improvement of lichen amyloidosis with pulsed dye laser. Both pruritus and the papular eruption of lichen amyloidosis improved.25
  • In a report emphasizing the localization of lichen amyloidosis in body regions with lower temperatures, narrow-band UVB was used to treat the patient; marked improvement of pruritus and clearing of the amyloid deposits was reported.26
  • In 2009, Aoki and Kawana reported successful treatment of lichen amyloidosis of the auricular concha using electrodesiccation.27

Surgical Care

Aggressive strategies proposed for the removal of amyloid include laser vaporization, dermabrasion, and excision of individual lesions. However, both the lesions and the pruritus usually promptly recur after these treatments.

In a prospective, side-by-side, controlled, clinical trial study, Ostovari et al used the Q-switched Nd:YAG laser (532- and 1064-nm) in 20 subjects with a clinical diagnosis and pathologic confirmation of macular amyloidosis. Using colorimetric score assessment and digital photographs before laser therapy and 8 weeks after treatment, they concluded that the 2 lasers (Q-switched 532- and 1064-nm Nd:YAG) are effective in reducing the degree of pigmentation in macular amyloidosis patches, with the 532-nm laser being more effective than the 1064-nm laser. The pictures shown in this paper were of low quality.28

Medication

The goal of pharmacotherapy for lichen amyloidosis is to reduce morbidity.

Antihistamines

These agents act by competitive inhibition of histamine at the H1 receptor. They may control itching by blocking effects of endogenously released histamine.


Chlorpheniramine (Chlor-Trimeton)

Competes with histamine or H1 receptor sites on effector cells in blood vessels and respiratory tract.

Adult

4 mg PO q4-6h; not to exceed 24 mg/d

Pediatric

<6 years: Not recommended
6-12 years: 2 mg PO q4-6h; not to exceed 12 mg/d
>12 years: Administer as in adults

CNS toxicity increases with coadministration of other CNS depressants, tricyclic antidepressants, MAOIs, and phenothiazines

Documented hypersensitivity; asthma attacks; narrow-angle glaucoma; symptomatic prostate hypertrophy; bladder neck obstruction; stenosing peptic ulcer

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

May cause significant confusional symptoms; not for administration to premature or full-term neonates; drowsiness, dizziness, and dryness of mouth are the most common adverse effects; patients should not operate vehicles or hazardous machinery


Diphenhydramine (Benadryl)

For symptomatic relief of symptoms caused by release of histamine in allergic reactions.

Adult

25-50 mg PO tid/qid

Pediatric

<10 kg: Not established
>10 kg: 12.5-25 mg PO tid/qid or 5 mg/kg/d PO divided tid/qid; not to exceed 300 mg/d

Potentiates effect of CNS depressants; because of alcohol content, do not give syr dosage form to patient taking medications that can cause disulfiramlike reactions

Documented hypersensitivity; MAOIs

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May exacerbate angle-closure glaucoma, hyperthyroidism, peptic ulcer, or urinary tract obstruction; drowsiness, dizziness, and xerostomia are the most common adverse effects; patients should not operate vehicles or hazardous machinery

Topical anti-inflammatory agents

This agent is an industrial solvent.


Dimethyl sulfoxide (Rimso-50)

May help relieve symptoms. DMSO, an oxidation product of dimethyl sulfide, is an exceptional solvent possessing a number of commercial uses.
Not an FDA-approved indication.

Adult

50% solution in water applied topically over affected area

Pediatric

Not established

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Garliclike breath odor and taste in the mouth due to excretion of small amount of DMSO as dimethyl sulfide (usually lasts only 24-48 h)

More on Amyloidosis, Lichen

Overview: Amyloidosis, Lichen
Differential Diagnoses & Workup: Amyloidosis, Lichen
Treatment & Medication: Amyloidosis, Lichen
References
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References

  1. Eanes ED, Glenner GG. X-ray diffraction studies on amyloid filaments. J Histochem Cytochem. Nov 1968;16(11):673-7. [Medline].

  2. Bugalho MJ, Limbert E, Sobrinho LG, et al. A kindred with multiple endocrine neoplasia type 2A associated with pruritic skin lesions. Cancer. Dec 1 1992;70(11):2664-7. [Medline].

  3. Chabre O, Labat F, Pinel N, Berthod F, Tarel V, Bachelot I. Cutaneous lesion associated with multiple endocrine neoplasia type 2A: lichen amyloidosis or notalgia paresthetica?. Henry Ford Hosp Med J. 1992;40(3-4):245-8. [Medline].

  4. Gagel RF, Levy ML, Donovan DT, Alford BR, Wheeler T, Tschen JA. Multiple endocrine neoplasia type 2a associated with cutaneous lichen amyloidosis. Ann Intern Med. Nov 15 1989;111(10):802-6. [Medline].

  5. Kousseff BG, Espinoza C, Zamore GA. Sipple syndrome with lichen amyloidosis as a paracrinopathy: pleiotropy, heterogeneity, or a contiguous gene?. J Am Acad Dermatol. Oct 1991;25(4):651-7. [Medline].

  6. Kousseff BG. Multiple endocrine neoplasia 2 (MEN 2)/MEN 2A (Sipple syndrome). Dermatol Clin. Jan 1995;13(1):91-7. [Medline].

  7. Nunziata V, di Giovanni G, Lettera AM, D'Armiento M, Mancini M. Cutaneous lichen amyloidosis associated with multiple endocrine neoplasia type 2A. Henry Ford Hosp Med J. 1989;37(3-4):144-6. [Medline].

  8. Pacini F, Fugazzola L, Bevilacqua G, Viacava P, Nardini V, Martino E. Multiple endocrine neoplasia type 2A and cutaneous lichen amyloidosis: description of a new family. J Endocrinol Invest. Apr 1993;16(4):295-6. [Medline].

  9. Robinson MF, Furst EJ, Nunziata V, et al. Characterization of the clinical features of five families with hereditary primary cutaneous lichen amyloidosis and multiple endocrine neoplasia type 2. Henry Ford Hosp Med J. 1992;40(3-4):249-52. [Medline].

  10. Rothberg AE, Raymond VM, Gruber SB, Sisson J. Familial medullary thyroid carcinoma associated with cutaneous lichen amyloidosis. Thyroid. Jun 2009;19(6):651-5. [Medline].

  11. Doutre MS, Beylot C, Couzigou P, Beraud C. Lichen amyloidosis in Alagille syndrome. Arch Dermatol. Oct 1991;127(10):1590-1. [Medline].

  12. Maddison B, Namazi MR, Samuel LS, et al. Unexpected diminished innervation of epidermis and dermoepidermal junction in lichen amyloidosus. Br J Dermatol. Aug 2008;159(2):403-6. [Medline].

  13. Apaydin R, Gurbuz Y, Bayramgurler D, Muezzinoglu B, Bilen N. Cytokeratin expression in lichen amyloidosus and macular amyloidosis. J Eur Acad Dermatol Venereol. May 2004;18(3):305-9. [Medline].

  14. Weyers W, Weyers I, Bonczkowitz M, Diaz-Cascajo C, Schill WB. Lichen amyloidosus: a consequence of scratching. J Am Acad Dermatol. Dec 1997;37(6):923-8. [Medline].

  15. Frolich M, Enk A, Diepgen TL, Weisshaar E. Successful treatment of therapy-resistant pruritus in lichen amyloidosis with menthol. Acta Derm Venereol. 2009;89(5):524-6. [Medline].

  16. Castanedo-Cazares JP, Lepe V, Moncada B. Lichen amyloidosis improved by 0.1% topical tacrolimus. Dermatology. 2002;205(4):420-1. [Medline].

  17. Hsieh SD, Yamamoto R, Saito K, et al. Amyloidosis presented with whitening and loss of hair which improved after dimethylsulfoxide (DMSO) treatment. Jpn J Med. Aug 1987;26(3):393-5. [Medline].

  18. Lim KB, Tan SH, Tan KT. Lack of effect of dimethyl sulphoxide (DMSO) on amyloid deposits in lichen amyloidosis. Br J Dermatol. Sep 1988;119(3):409-10. [Medline].

  19. Ozkaya-Bayazit E, Kavak A, Gungor H, Ozarmagan G. Intermittent use of topical dimethyl sulfoxide in macular and papular amyloidosis. Int J Dermatol. Dec 1998;37(12):949-54. [Medline].

  20. Pandhi R, Kaur I, Kumar B. Lack of effect of dimethylsulphoxide in cutaneous amyloidosis. J Dermatolog Treat. Mar 2002;13(1):11-4. [Medline].

  21. Aram H. Failure of etretinate (RO 10-9359) in lichen amyloidosus. Int J Dermatol. Apr 1986;25(3):206. [Medline].

  22. David M, Ingber A, Ben-Chetrit A, Sandbank J, Sandbank M. Effect of etretinate on lichen amyloidosus. Dermatologica. 1987;175(6):302-3. [Medline].

  23. Marschalko M, Daroczy J, Soos G. Etretinate for the treatment of lichen amyloidosis. Arch Dermatol. May 1988;124(5):657-9. [Medline].

  24. Choi JY, Sippe J, Lee S. Acitretin for lichen amyloidosus. Australas J Dermatol. May 2008;49(2):109-13. [Medline].

  25. Sawamura D, Sato-Matsumura KC, Shibaki A, Akiyama M, Kikuchi T, Shimizu H. A case of lichen amyloidosis treated with pulsed dye laser. J Eur Acad Dermatol Venereol. Mar 2005;19(2):262-3. [Medline].

  26. Parsi K, Kossard S. Thermosensitive lichen amyloidosis. Int J Dermatol. Dec 2004;43(12):925-8. [Medline].

  27. Aoki M, Kawana S. Lichen amyloidosis of the auricular concha: successful treatment with electrodesiccation. J Dermatol. Feb 2009;36(2):116-7. [Medline].

  28. Ostovari N, Mohtasham N, Oadras MS, Malekzad F. 532-nm and 1064-nm Q-switched Nd:YAG laser therapy for reduction of pigmentation in macular amyloidosis patches. J Eur Acad Dermatol Venereol. Apr 2008;22(4):442-6. [Medline].

  29. Breathnach SM, Bhogal B, Dyck RF, De Beer FC, Black MM, Pepys MB. Immunohistochemical demonstration of amyloid P component in skin of normal subjects and patients with cutaneous amyloidosis. Br J Dermatol. Aug 1981;105(2):115-24. [Medline].

  30. Breathnach SM, Melrose SM, Bhogal B, et al. Amyloid P component is located on elastic fibre microfibrils in normal human tissue. Nature. Oct 22 1981;293(5834):652-4. [Medline].

  31. Eriksson S, Janciauskiene S, Merlini G. The putative role of alpha-1-antitrypsin in the disaggregation of amyloid lambda fibrils. J Intern Med. Feb 1995;237(2):143-9. [Medline].

  32. Kuligowski ME, Chang A. Non-itchy lichen amyloidosus. Int J Dermatol. Oct 1992;31(10):747. [Medline].

  33. Lambert WC. Cutaneous deposition disorders. In: Farmer ER, Hood AF, eds. Pathology of the Skin. Vol 432. Norwalk, Conn: Appleton & Lange; 1990:50.

  34. Li JJ, McAdam KP. Human amyloid P component: an elastase inhibitor. Scand J Immunol. Sep 1984;20(3):219-26. [Medline].

  35. Monfrecola G, Iandoli R, Bruno G, Martellotta D. Lichen amyloidosus: a new therapeutic approach. Acta Derm Venereol. 1985;65(5):453-5. [Medline].

  36. Piette WW. Myeloma, paraproteinemias, and the skin. Med Clin North Am. Jan 1986;70(1):155-76. [Medline].

  37. Ruzicka T, Donhauser G, Linke RP, Landthaler M, Bieber T. [Cutaneous amyloidoses]. Hautarzt. May 1990;41(5):245-55. [Medline].

  38. Shirahama T, Cohen AS. High-resolution electron microscopic analysis of the amyloid fibril. J Cell Biol. Jun 1967;33(3):679-708. [Medline].

  39. Touart DM, Sau P. Cutaneous deposition diseases. Part I. J Am Acad Dermatol. Aug 1998;39(2 Pt 1):149-71; quiz 172-4. [Medline].

  40. Wang WJ. Clinical features of cutaneous amyloidoses. Clin Dermatol. Apr-Jun 1990;8(2):13-9. [Medline].

  41. Wong CK. Lichen amyloidosus. A relatively common skin disorder in Taiwan. Arch Dermatol. Sep 1974;110(3):438-40. [Medline].

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Further Reading

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Keywords

lichen amyloidosis, LA, amyloid deposits, multiple endocrine neoplasia type 2A, MEN 2A, Sipple syndrome

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Contributor Information and Disclosures

Author

Sultan Al-Khenaizan, MBBS, FRCP(C), Consulting Staff, Departments of Dermatology and Internal Medicine, King Fahad National Guard Hospital, Saudi Arabia
Disclosure: Nothing to disclose.

Medical Editor

Catharine Lisa Kauffman, MD, FACP, Georgetown Dermatology and Georgetown Dermpath
Catharine Lisa Kauffman, MD, FACP is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Royal Society of Medicine, Society for Investigative Dermatology, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Warren R Heymann, 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

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

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.

 
 
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