eMedicine Specialties > Dermatology > Diseases of the Dermis

Cutis Laxa (Elastolysis): Treatment & Medication

Author: Daniel J Hogan, MD, Clinical Professor of Internal Medicine (Dermatology), NOVA Southeastern University; Investigator, Hill Top Research, Florida Research Center
Coauthor(s): Tina Molis, MD, PhD, Staff Physician, Department of Radiology, St Francis Medical Center, University of Illinois at Peoria
Contributor Information and Disclosures

Updated: Jan 22, 2009

Treatment

Medical Care

  • No treatment exists to prevent disease progression, although dapsone can be used acutely to control swelling in persons with acquired cutis laxa (elastolysis), consistent with the suspected role of neutrophil elastase.
  • Penicillamine and doxycycline are ineffective.
  • Cutis Laxa Internationale is an international support group for patients with cutis laxa (elastolysis).

Surgical Care

  • Surgical correction of redundant skin folds, prolapses, or hernias may be undertaken. However, surgery often produces only temporary benefit.
  • Botulinum toxin injections are being considered for improving the aged appearance and facial defects seen in persons with cutis laxa (elastolysis).18

Consultations

  • Consult a dermatologist for evaluation of the underlying cause of cutis laxa (elastolysis).
  • Consult an internal medicine specialist for evaluation of the underlying cause of cutis laxa (elastolysis) and internal organ involvement.
  • Cutis laxa (elastolysis) increases the risk for aortic aneurysm; therefore, regular cardiac monitoring is recommended to avert a potentially fatal aortic rupture.
  • Affected individuals may require a consultation with a pulmonologist.
  • Consultation with a geneticist is suggested, particularly for patients presenting in childhood.

Medication

The goals of pharmacotherapy are to eradicate the infection, to reduce morbidity, and to prevent complications.

Antibiotics

These agents may be useful to control the acute phase.


Dapsone (Avlosulfon)

Bactericidal and bacteriostatic against mycobacteria; mechanism of action is similar to that of sulfonamides, with which competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Anti-inflammatory mechanism of action may be related to inhibition of halide-myeloperoxidase system of neutrophils.

Adult

25-400 mg PO in divided doses (average adult dose, 100 mg/d)

Pediatric

1-2 mg/kg/d PO in divided doses

Trimethoprim, probenecid, and folic acid antagonists (eg, pyrimethamine, methotrexate) increase levels; activated charcoal, PABA, and rifampin decrease levels; sulfonamides and hydroxychloroquine may increase hemolysis

Absolute: Documented hypersensitivity
Relative: G-6-PD deficiency (especially in African Americans, persons of Middle Eastern heritage, and Asians), significant cardiopulmonary or hematologic disease, sulfa allergy (cautious use in patients with sulfa allergy may be attempted; cross-reactivity is relatively rare and mild)

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

Perform weekly blood cell counts (first mo), then perform WBC counts monthly (6 mo), and then perform blood counts semiannually; discontinue if significant reduction in platelets, leukocytes, or hematopoiesis occurs; caution in methemoglobin reductase deficiency, G-6-PD deficiency (patients receiving >200 mg/d), or hemoglobin M because of high risk for hemolysis and Heinz body formation; caution in patients exposed to other agents or conditions (eg, infection, diabetic ketosis) capable of producing hemolysis; peripheral neuropathy can occur (rare); phototoxicity may occur when exposed to UV light

More on Cutis Laxa (Elastolysis)

Overview: Cutis Laxa (Elastolysis)
Differential Diagnoses & Workup: Cutis Laxa (Elastolysis)
Treatment & Medication: Cutis Laxa (Elastolysis)
Follow-up: Cutis Laxa (Elastolysis)
Multimedia: Cutis Laxa (Elastolysis)
References
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References

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Further Reading

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Keywords

cutis laxa, elastolysis, CL, cutis pendula, dermatochalasis, elastolysis, dermatomegaly, pachydermatocele, elastolysis cutis laxa, generalized elastolysis, generalized elastorrhexis

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Contributor Information and Disclosures

Author

Daniel J Hogan, MD, Clinical Professor of Internal Medicine (Dermatology), NOVA Southeastern University; Investigator, Hill Top Research, Florida Research Center
Daniel J Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, and Canadian Dermatology Association
Disclosure: Nothing to disclose.

Coauthor(s)

Tina Molis, MD, PhD, Staff Physician, Department of Radiology, St Francis Medical Center, University of Illinois at Peoria
Disclosure: Nothing to disclose.

Medical Editor

Susan M Swetter, MD, Director, Pigmented Lesion and Cutaneous Melanoma Clinic, Associate Professor, Department of Dermatology, Stanford University Medical Center, Veterans Affairs Palo Alto Health Care System
Susan M Swetter, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society of Clinical Oncology, Eastern Cooperative Oncology Group, Pacific Dermatologic Association, Society for Investigative Dermatology, Society for Melanoma Research, 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

Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center
Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery
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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