eMedicine Specialties > Dermatology > Metabolic Diseases

Amyloidosis, Macular: 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: Sep 25, 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 in macular amyloidosis and lichen amyloidosis.
    • Sedating antihistamines have been found to be moderately effective.
    • Topical dimethyl sulfoxide (DMSO), a chemical solvent, and intralesional steroids are beneficial if combined with other modalities. DMSO has been used with moderate success, but failures have also been reported.9,10,11 Pandhi et al and Lim et al reported a lack of effect with DMSO treatment for cutaneous amyloidosis.12,13
    • Treatment with ultraviolet B (UV-B) light can provide symptomatic relief.
    • Sawamura et al reported satisfying improvement of lichen amyloidosis with pulsed dye laser therapy. Both pruritus and the papular eruption of lichen amyloidosis improved.14

Surgical Care

  • Aggressive strategies proposed for the removal of amyloid include laser vaporization, dermabrasion, and excision of individual lesions. However, lesions and pruritus usually promptly recur after these treatments.
  • Electrodesiccation and curettage provided an acceptable result in one report.15
  • In a prospective, side-by-side, controlled, clinical trial study, Ostovari et al used the Q-switched Nd:YAG laser (532 nm and 1064 nm) in 20 subjects with a clinical diagnosis and pathology 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 are effective in reducing the degree of macular amyloidosis pigmentation, with the 532-nm laser being more effective than the 1064-nm laser. The pictures shown in this paper were of low quality.8

Medication

The goal of pharmacotherapy 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

<2 years: Not established
2-6 years: 1 mg PO divided q4-6h; not to exceed 6 mg/d
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

Drowsiness, dizziness, and dryness of mouth are the most common adverse effects; not for administration to premature or full-term neonates


Diphenhydramine (Benadryl, Belix)

For symptomatic relief of pruritus caused by endogenous release of histamine.

Adult

25-50 mg PO tid/qid; not to exceed 400 mg/d

Pediatric

12.5-25 mg PO tid/qid or 5 mg/kg/d or 150 mg/m2/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

Drowsiness, dizziness, and dryness of mouth are the most common adverse effects; may exacerbate angle-closure glaucoma, hyperthyroidism, peptic ulcer, and urinary tract obstruction

Topical anti-inflammatory agents

This industrial solvent has been used with mixed results.


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

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, Macular

Overview: Amyloidosis, Macular
Differential Diagnoses & Workup: Amyloidosis, Macular
Treatment & Medication: Amyloidosis, Macular
Follow-up: Amyloidosis, Macular
Multimedia: Amyloidosis, Macular
References
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References

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Further Reading

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Keywords

amyloidosis, macular amyloidosis, amyloid, amyloid-P, serum amyloid-P, SAP, Sipple syndrome, MA

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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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