Lipodermatosclerosis | |
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Other names | Chronic panniculitis with lipomembranous changes, [1] hypodermitis sclerodermiformis, sclerosing panniculitis, stasis panniculitis [2] : 489 |
Specialty | Dermatology |
Lipodermatosclerosis 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]
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]
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]
Diagnosis is clinical, based on observation. Biopsy is rarely required.
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.
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
An ulcer is a sore on the skin or a mucous membrane, accompanied by the disintegration of tissue. Ulcers can result in complete loss of the epidermis and often portions of the dermis and even subcutaneous fat. Ulcers are most common on the skin of the lower extremities and in the gastrointestinal tract. An ulcer that appears on the skin is often visible as an inflamed tissue with an area of reddened skin. A skin ulcer is often visible in the event of exposure to heat or cold, irritation, or a problem with blood circulation.
Panniculitis is a group of diseases whose hallmark is inflammation of subcutaneous adipose tissue. Symptoms include tender skin nodules, and systemic signs such as weight loss and fatigue.
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 this system is as a barrier against the external environment.
Stasis dermatitis refers to the skin changes that occur in the leg as a result of "stasis" or blood pooling from insufficient venous return; the alternative name of varicose eczema comes from a common cause of this being varicose veins.
Venous ulcer is defined by the American Venous Forum as "a full-thickness defect of skin, most frequently in the ankle region, that fails to heal spontaneously and is sustained by chronic venous disease, based on venous duplex ultrasound testing." Venous ulcers are wounds that are thought to occur due to improper functioning of venous valves, usually of the legs. They are an important cause of chronic wounds, affecting 1% of the population. Venous ulcers develop mostly along the medial distal leg, and can be painful with negative effects on quality of life.
Livedo reticularis is a common skin finding consisting of a mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin. The discoloration is caused by reduction in blood flow through the arterioles that supply the cutaneous capillaries, resulting in deoxygenated blood showing as blue discoloration. This can be a secondary effect of a condition that increases a person's risk of forming blood clots, including a wide array of pathological and nonpathological conditions. Examples include hyperlipidemia, microvascular hematological or anemia states, nutritional deficiencies, hyper- and autoimmune diseases, and drugs/toxins.
Lupus vulgaris are painful cutaneous tuberculosis skin lesions with nodular appearance, most often on the face around the nose, eyelids, lips, cheeks, ears and neck. It is the most common Mycobacterium tuberculosis skin infection. The lesions may ultimately develop into disfiguring skin ulcers if left untreated.
Subcutaneous fat necrosis of the newborn is a rare form of lobular panniculitis occurring in newborns that is usually self-remitting and non-recurring. Proposed causes include perinatal stress, local trauma, hypoxia and hypothermia, though the exact cause is unknown. It has been suggested that the brown fat seen in newborns is more sensitive to hypoxic injury than fat seen in adults, and that such hypoxia, usually in the context of a complicated birth, leads to the fat necrosis. Complications can include hypercalcemia, hyperlipidemia, dehydration, hypoglycemia, seizures, vomiting, constipation, and thrombocytopenia, and can present months after the onset of SCFN symptoms.
A venous lake is a generally solitary, soft, compressible, dark blue to violaceous, 0.2- to 1-cm papule commonly found on sun-exposed surfaces of the vermilion border of the lip, face and ears. Lesions generally occur among the elderly.
The term venous translucence has been used in phlebology since 1996 by surgeon Pedro Fernandes Neto during ambulatory clinical exams in Brazil. His results were published in the annals of the national and international congresses of angiology. Venous translucence is the process of reflective image visualization of veins by light, which reaches up to the superficial venous system. It is a non-invasive method. Since it is a simple, low-cost technique it can be repeated as needed, which is useful in disease-process monitoring. It is a new diagnostic procedure, still undergoing investigation; more analysis is necessary to hone its technical aspects. Venous translucence is based on optical physics. It is caused by the refraction, absorption and reflection of light. The color which is not absorbed is reflected, and is the one that is seen. Therefore, venous translumination is based on the incidence of luminosity on the vein, where part of the light is absorbed and another reflected.
Sclerema neonatorum is a rare and severe skin condition that is characterized by diffuse hardening of the subcutaneous tissue with minimal inflammation. It usually affects premature, ill newborns. Prognosis is poor.
Cutaneous small-vessel vasculitis (CSVV), also known as hypersensitivity vasculitis, cutaneous leukocytoclastic vasculitis, hypersensitivity angiitis, cutaneous leukocytoclastic angiitis, cutaneous necrotizing vasculitis and cutaneous necrotizing venulitis, is inflammation of small blood vessels, characterized by palpable purpura. It is the most common vasculitis seen in clinical practice.
Arterial insufficiency ulcers are mostly located on the lateral surface of the ankle or the distal digits. They are commonly caused by peripheral artery disease (PAD).
Elephantiasis nostras is a cutaneous condition, a final hypertrophic fibrosis following longstanding chronic lymphangitis.
Livedoid vasculopathy is a chronic cutaneous disease seen predominantly in young to middle-aged women. One acronym used to describe its features is "Painful purpuric ulcers with reticular pattern of the lower extremities" (PURPLE).
Acroangiodermatitis of Mali is a rare cutaneous condition often characterized by purplish-blue to brown papules and plaques on the medial and lateral malleolus of both legs.
Weber–Christian disease, is a cutaneous condition characterized by recurrent subcutaneous nodules that heal with depression of the overlying skin.
Tuberculous gumma is a skin condition characterized histologically by massive necrosis. Restated, this is a skin condition that results from hematogenous dissemination of mycobacteria from a primary focus, resulting in firm, nontender erythematous nodules that soften, ulcerate, and form sinuses.
Stasis papillomatosis is a disease characterized by chronic congestion of the extremities, with blood circulation interrupted in a specific area of the body. A consequence of this congestion and inflammation is long-term lymphatic obstruction. It is also typically characterized by the appearance of numerous papules. Injuries can range from small to large plates composed of brown or pink, smooth or hyperkeratotic papules. The most typical areas where injuries occur are the back of the feet, the toes, the legs, and the area around a venous ulcer formed in the extremities, although the latter is the rarest of all. These injuries include pachydermia, lymphedema, lymphomastic verrucusis and elephantosis verracosa. The disease can be either localized or generalized; the localized form makes up 78% of cases. Treatment includes surgical and pharmaceutical intervention; indications for partial removal include advanced fibrotic lymphedema and elephantiasis. Despite the existence of these treatments, chronic venous edema, which is a derivation of stasis papillomatosis, is only partially reversible. The skin is also affected and its partial removal may mean that the skin and the subcutaneous tissue are excised. A side effect of the procedure is the destruction of existing cutaneous lymphatic vessels. It also risks papillomatosis, skin necrosis and edema exacerbation.