Lipodermatosclerosis is a
skin
Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation.
Other cuticle, animal coverings, such as the arthropod exoskeleton, have diffe ...
and
connective tissue
Connective tissue is one of the four primary types of animal tissue, along with epithelial tissue, muscle tissue, and nervous tissue. It develops from the mesenchyme derived from the mesoderm the middle embryonic germ layer. Connective tissue ...
disease
A disease is a particular abnormal condition that negatively affects the structure or function of all or part of an organism, and that is not immediately due to any external injury. Diseases are often known to be medical conditions that a ...
. It is a form of lower extremity
panniculitis,
[Bruce AJ. et al., Lipodermatosclerosis: Review of cases evaluated at Mayo Clinic. J Am Acad Dermatol. 2002.] an
inflammation
Inflammation (from la, wikt:en:inflammatio#Latin, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or Irritation, irritants, and is a protective response involving im ...
of the layer of fat under the
epidermis
The epidermis is the outermost of the three layers that comprise the skin, the inner layers being the dermis and Subcutaneous tissue, hypodermis. The epidermis layer provides a barrier to infection from environmental pathogens and regulates the ...
.
[Ginsburg PM, Ehrenpreis ED. NORD Guide to Rare Disorders. Philadelphia PA: Lippincott Williams & Wilkins; 2003.]
Signs and symptoms
Pain may be the first noticed symptom.
People with lipodermatosclerosis have tapering of their legs above the ankles, forming a constricting band resembling an inverted champagne bottle.
[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.] In addition, there may be brownish-red pigmentation and
induration
A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of thi ...
.
Causes
The exact cause of lipodermatosclerosis is unknown.
Venous disease, such as venous incompetence, venous hypertension, and
body mass
Human body weight is a person's mass or weight.
Strictly speaking, body weight is the measurement of weight without items located on the person. Practically though, body weight may be measured with clothes on, but without shoes or heavy accessor ...
(''
obesity
Obesity is a medical condition, sometimes considered a disease, in which excess body fat has accumulated to such an extent that it may negatively affect health. People are classified as obese when their body mass index (BMI)—a person's ...
'') may be relevant to the underlying
pathogenesis
Pathogenesis is the process by which a disease or disorder develops. It can include factors which contribute not only to the onset of the disease or disorder, but also to its progression and maintenance. The word comes from Greek πάθος ''pat ...
.
Increased
blood pressure
Blood pressure (BP) is the pressure of circulating blood against the walls of blood vessels. Most of this pressure results from the heart pumping blood through the circulatory system. When used without qualification, the term "blood pressur ...
in the veins (venous hypertension) can cause diffusion of substances, including
fibrin
Fibrin (also called Factor Ia) is a fibrous, non-globular protein involved in the clotting of blood. It is formed by the action of the protease thrombin on fibrinogen, which causes it to polymerize. The polymerized fibrin, together with pla ...
, out of capillaries. Fibrotic tissue may predispose the tissue to
ulceration
An ulcer is a discontinuity or break in a bodily membrane that impedes normal function of the affected organ. According to Robbins's pathology, "ulcer is the breach of the continuity of skin, epithelium or mucous membrane caused by sloughing o ...
. Recurrent ulceration and fat
necrosis
Necrosis () is a form of cell injury which results in the premature death of cells in living tissue by autolysis. Necrosis is caused by factors external to the cell or tissue, such as infection, or trauma which result in the unregulated dig ...
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.
Lipodermatosclerosis is most commonly diagnosed in middle-aged women.
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.
Diagnosis
Diagnosis is clinical, based on observation. Biopsy is rarely required.
Treatment
The management of lipodermatosclerosis may include treating venous insufficiency with leg elevation and elastic
compression stockings
Compression stockings (Flight Socks, Support Bandage) are a specialized hosiery designed to help prevent the occurrence of, and guard against further progression of, venous disorders such as edema, phlebitis and thrombosis. 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 clot
A thrombus (plural thrombi), colloquially called a blood clot, is the final product of the blood coagulation step in hemostasis. There are two components to a thrombus: aggregated platelets and red blood cells that form a plug, and a mesh of cr ...
s.
[Camilleri MJ, Danil Su WP. Fitzpatrick’s Dermatology in General Medicine, 6th ed. In: . Panniculitis. New York, NY:McGraw-Hill; 2003.]
Stanozol is injected directly into the affected area, Venous Ablation has also been known to help circulation in patients.
See also
*
Panniculitis
*
List of cutaneous conditions
Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier agai ...
Notes
''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''
External links
*
{{Disorders of subcutaneous fat
Conditions of the subcutaneous fat