Skin nontumor
Dermal perivascular and vasculopathic reaction patterns
Livedoid vasculopathy
Last author update: 1 August 2011
Last staff update: 8 February 2024
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PubMed Search: Vasculitis [title] skin
Table of Contents
Definition / general | Terminology | Clinical features | Treatment | Microscopic (histologic) description | Positive stainsCite this page: Hamodat M. Livedoid vasculopathy. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorsegmentedhyalinizingvasculitis.html. Accessed December 26th, 2024.
Definition / general
- Manifestation of a thrombogenic vasculopathy in which occlusion of small dermal vessels by fibrin thrombi is the primary event
Terminology
- Also called atrophie blanche of Milian
Clinical features
- Affects lower legs, particularly ankles and dorsum of feet, rarely the extensor arm surfaces below the elbows
- Elevated levels of fibrinopeptide A are suggestive of a thrombogenic state
- Several cases with lupus-type anticoagulant and increased level of anticardiolipin antibodies have been reported
- Other associations include protein C deficiency, factor V Leiden mutation, prothrombin mutation, plasminogen activator inhibitor-1 promoter mutation (OMIM 173360), antithrombin III deficiency, methylenetetrahydrofolate reductase mutation (OMIM 607093), homocysteinemia and essential cryoglobulinemia, all of which contribute to a hypercoagulable condition
- Description: painful purpuric ulcers with reticular patterning on the lower extremities
Treatment
- Antiplatelet drugs and antithrombotic drugs may be useful
- Also dapsone, nicotinic acid, intravenous immunoglobulin, PUVA therapy, danazol and hyperbaric oxygen therapy
- Warfarin therapy is sometimes used to treat the associated / concurrent abnormalities in one or more clotting factors
- Also cessation of smoking, low dose aspirin, oral pentoxifylline and oral dipyrimadole
- Folic acid is recommended for those with an MTHFR mutation, hydrochloroquine for patients with antiphospholipid antibodies and anabolic steroids (stanozolol or danazol) for patients with cryofibrinogenemia
Microscopic (histologic) description
- Dermal capillaries have focal endothelial proliferation and wall thickening by PAS+ material, with fibrin thrombi causing occlusion
- Specific changes vary by age of lesion
- Primary event is formation of hyaline thrombi in lumen of small vessels in upper and mid dermis; rarely deeper vessels are involved; PAS+ diastase resistant fibrinoid material also present in walls of blood vessels and in perivascular stroma; also infarction of superficial dermis, often with a small area of ulceration
- Sometimes a thin parakeratotic layer is present overlying infarcted or atrophic epidermis
- Epidermis adjacent to ulceration may be spongiotic; a sparse perivascular lymphocytic infiltrate may be present, but there is no vasculitis
- Neutrophils, if present, are usually sparse and confined to infarcted upper dermis and ulcer base
- Often extravasated red cells in upper dermis
- Small blood vessels often increased in adjacent papillary dermis, but this is a common feature in biopsies from lower legs
- In older lesions, there is thickening and hyalinization of dermal vessels with some endothelial edema and proliferation
- Fibrinoid material may also be present in vessel walls
- In even older lesions, there is dermal sclerosis and scarring with some dilated lymphatics and epidermal atrophy; there may be a small amount of hemosiderin in upper dermis
- As old lesions may become involved again, it is possible to find dermal sclerosis in some early lesions
Positive stains
- Immunofluorescence will demonstrate fibrin in vessel walls in early lesions; in later stages there are also immunoglobulins and complement components in broad bands about vessel walls
