Lipodermatosclerosis
| Lipodermatosclerosis | |
|---|---|
| Classification and external resources | |
| DiseasesDB | 30155 |
Lipodermatosclerosis (also known as "chronic panniculitis with lipomembranous changes",[1] "hypodermitis sclerodermiformis", "sclerosing panniculitis", and "stasis panniculitis"[2]:489) is a skin and connective tissue disease. It is a form of lower extremity panniculitis,[3] an inflammation of the layer of fat under the epidermis.[4]
Symptoms
Pain may be the first noticed symptom.[3] People with lipodermatosclerosis have tapering of their legs above the ankles, forming a constricting band resembling an inverted champagne bottle.[3][5] In addition, there may be brownish-red pigmentation and induration.[5]
Causes
The exact cause of lipodermatosclerosis is unknown.[3][6] Venous disease, such as venous incompetence, venous hypertension, and body mass (obesity) may be relevant to the underlying pathogenesis.[3]
Increased blood pressure in the veins (venous hypertension) can cause diffusion of substances, including fibrin, out of capillaries. Fibrotic tissue may predispose the tissue to ulceration. Recurrent ulceration and fat necrosis is associated with lipodermatosclerosis. In advanced lipodermatosclerosis the proximal leg swells from chronic venous obstruction and the lower leg shrinks from chronic ulceration and fat necrosis resulting in the inverted coke bottle appearance of the lower leg.[7]
Lipodermatosclerosis is most commonly diagnosed in middle-aged women.[3]
The origin of lipodermatosclerosis is probably multifactorial, involving tissue hypoxia, leakage of proteins into the interstitium, and leukocyte activation. Studies of patients with lipodermatosclerosis have demonstrated significantly decreased concentrations of cutaneous oxygen associated with decreased capillary density. Capillaries are virtually absent in areas of fibrotic scars, leading to a condition known as atrophie blanche or livedoid vasculopathy.[8]
Treatment
The management of lipodermatosclerosis may include treating venous insufficiency with leg elevation and elastic compression stockings; in some difficult cases, the condition may be improved with the additional use of the fibrinolytic agent, stanozol. Fibrinolytic agents use an enzymatic action to help dissolve blood clots.[3][4][5][9] Stanozol is injected directly into the affected area, Venous Ablation has also been known to help circulation in patients.
See also
Notes
- ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
- ↑ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
- 1 2 3 4 5 6 7 Bruce AJ. et al., Lipodermatosclerosis: Review of cases evaluated at Mayo Clinic. J Am Acad Dermatol. 2002.
- 1 2 Ginsburg PM, Ehrenpreis ED. NORD Guide to Rare Disorders. Philadelphia PA: Lippincott Williams & Wilkins; 2003.
- 1 2 3 Phelps RG, Shoji T. . The Mount Sinai Journal of Medicine. 2001;:. Available at: http://www.mssm.edu/msjournal/68/v68_pages262_267.pdf. February 13, 2008.
- ↑ Fischer DR, Matthews JB. Sleisenger & Fordtran’s Gastrointestinal and Liver Disease, 7th ed . In: . Diseases of the mesentery and omentum. Philadelphia, PA:Saunders, an imprint of Elsevier Inc.; 2002.
- ↑ Habif TP. Habif: Clinical Dermatology, 4th ed. In: . Stasis dermatitis and venous ulceration: Postphlebitic syndromes. New York, NY:Mosby, Inc.; 2004
- ↑ PubMed. Transcutaneous oxygen tension and capillary morphologic characteristics and density in patients with chronic venous incompetence. Franzeck UK, Bollinger A, Huch R, Huch A. Circulation. 1984;70(5):806.
- ↑ Camilleri MJ, Danil Su WP. Fitzpatrick’s Dermatology in General Medicine, 6th ed. In: . Panniculitis. New York, NY:McGraw-Hill; 2003.
External links
Note: This article contains material adapted from the public domain source "Lipodermatosclerosis: Questions and Answers", by the U.S federal government's Genetic and Rare Diseases Information Center