Vasculitis limited to skin, not elsewhere classified (L95)
ICD-10 code L95 covers vasculitis limited to the skin without involvement of other organs. These codes help specify different types of skin-limited vasculitis conditions for accurate diagnosis and treatment documentation.
The category includes several distinct codes: L95.0 for Livedoid vasculitis, also known by terms such as livedoid vasculopathy or idiopathic livedo reticularis, which affects blood vessels in the lower limbs causing skin ulcers and specific rash patterns. L95.1 represents Erythema elevatum diutinum, a rare chronic condition involving red or purple skin lesions, sometimes called primary cutaneous vasculitis. L95.8 captures other specified types of skin-limited vasculitis, covering a wide array of related diseases like purpuric dermatosis, nodular vasculitis, or urticarial vasculitis with immune components. Lastly, L95.9 is used when the vasculitis is limited to the skin but unspecified, including reactive or necrotizing types. These ICD-10 codes guide healthcare providers and coders in identifying the correct classification based on clinical features and synonyms associated with each condition.
Instructional Notations
Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
- angioma serpiginosum L81.7
- Henoch-Schönlein purpura D69.0
- hypersensitivity angiitis M31.0
- lupus panniculitis L93.2
- panniculitis NOS M79.3
- panniculitis of neck and back M54.0
- polyarteritis nodosa M30.0
- relapsing panniculitis M35.6
- rheumatoid vasculitis M05.2
- serum sickness T80.6
- urticaria L50
- Wegener's granulomatosis M31.3
Clinical Terms
The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.
Erythema Induratum
A type of panniculitis characterized histologically by the presence of granulomas, vasculitis, and necrosis. It is traditionally considered to be the tuberculous counterpart of nodular vasculitis, but is now known to occur without tuberculous precedent. It is seen most commonly in adolescent and menopausal women, is initiated or exacerbated by cold weather, and typically presents as one or more recurrent erythrocyanotic nodules or plaques on the calves. The nodules may progress to form indurations, ulcerations, and scars.
Livedo Reticularis
A condition characterized by a reticular or fishnet pattern on the skin of lower extremities and other parts of the body. This red and blue pattern is due to deoxygenated blood in unstable dermal blood vessels. The condition is intensified by cold exposure and relieved by rewarming.
Livedoid Vasculopathy
A rare cutaneous thrombotic disease due to occlusion of dermal vessels. It is characterized by purpuric maculae and ulcerations especially during summer which form scars called atrophie blanche. It is more associated with other syndromes (e.g., PROTEIN C DEFICIENCY; HYPERHOMOCYSTEINEMIA). Livedo reticularis with systemic involvement and stroke is SNEDDON SYNDROME.
Schnitzler Syndrome
An extremely rare condition manifested as monoclonal IMMUNOGLOBULIN M dysproteinemia without features of lymphoproliferative disease, but with chronic urticaria, fever of unknown origin, disabling bone pain, hyperostosis, and increased erythrocyte sedimentation rate.
Sneddon Syndrome
A systemic non-inflammatory arteriopathy primarily of middle-aged females characterized by the association of LIVEDO RETICULARIS, multiple thrombotic CEREBRAL INFARCTION; CORONARY DISEASE, and HYPERTENSION. Elevation of antiphospholipid antibody titers (see also ANTIPHOSPHOLIPID SYNDROME), cardiac valvulopathy, ISCHEMIC ATTACK, TRANSIENT; SEIZURES; DEMENTIA; and chronic ischemia of the extremities may also occur. Pathologic examination of affected arteries reveals non-inflammatory adventitial fibrosis, thrombosis, and changes in the media (From Jablonski, Dictionary of Syndromes & Eponymic Diseases, 2d ed; Adams et al., Principles of Neurology, 6th ed, p861; Arch Neurol 1997 Jan;54(1):53-60). Mutations in the CECR1 gene (ADA2 protein, human) are associated with Sneddon syndrome.
