Dr Varsha V S, Dr Suma Balan, Dr Jayakumar
Eleven year old boy 2nd child of non-consanguineous marriage presented with erythematous skin rashes and swelling over both lower limbs and severe pain of 5 days duration with out any fever.
Past history: H/o atopic dermatitis in the past.
Perinatal history normal
Grand father is on treatment for psoriasis.
On examination: Child was afebrile and alert. Vitals were stable.
Systemic examination was normal.
Local examination done showed multiple erythematous rashes over b/l lower limbs associated with painfull nodules .
Labs done showed neutrophilic leucocytosis , normal Hb and platelet with elevated CRP( 155), ESR: 85, RFT, LFT, SE were normal. Mantoux was strongly positive.
Chest xray done was normal.
IGRA was normal.
Gene expert was negative
Skin biopsy done showed epidermis with basket weave orthokeratin.
Small and medium vessels in dermis as well as subcutaneous fat show fibrinoid necrosis. Mixed inflammation and histiocytic collection seen in subcutaneous fat.
Impression: skin biopsy shows small and medium vessel vasculitis with c3 and IgM on direct immunofluresence.
Features are consistent with Poly arteritis nodosa. To rule out systemic involvement ophthalmology examination and Echo was done which were normal.
He was diagnosed with cutaneous Polyarteritis nodosa and managed with naproxen and steroids.
He responded to the given treatment well. Child was kept under strict follow up.
Description:
Cutaneous Polyarteritis Nodosa (cutaneous PAN) is a rare, chronic inflammatory condition affecting small- to medium-sized blood vessels in the skin. Incidence is not well-documented due to its rarity. Unlike systemic PAN, which involves multiple organs, cutaneous PAN is limited to the skin and occasionally peripheral nerves. Here’s an overview:
Symptoms
Skin Lesions:
Painful nodules, typically on the lower extremities.
Ulcers, livedo reticularis (a mottled, net-like discoloration), or purpura.
Pain: Lesions can be tender, and some patients experience burning or stinging sensations.
Recurrent Episodes: Symptoms often come in cycles, with periods of remission and flare-ups.
Causes
The exact cause is often unknown, but it is sometimes associated with:
Infections (e.g., streptococcal infections or hepatitis B/C).
Autoimmune diseases.
Trauma to the skin.
Differential diagnosis:
Leukocytoclastic vasculitis.
Other forms of medium-vessel vasculitis (e.g., systemic PAN, Kawasaki disease).
Hyper sensitivity vasculitis
Diagnosis
Biopsy:
Confirms vasculitis (inflammation of blood vessels).
Vasculitis of Medium- to Small-Sized Arteries:
Inflammation of the vessel wall.
Fibrinoid necrosis (destruction of the vessel wall with deposition of fibrin-like material).
Predominantly neutrophils, with lymphocytes and mononuclear cells in later stages.
Thrombosis:
Partial or complete occlusion of the affected vessels.
Ischemic Changes:
Evidence of tissue damage in the surrounding skin due to reduced blood supply (e.g., necrosis).
Laboratory Tests:
May include inflammatory markers (ESR, CRP), autoantibodies, or tests for associated infections.
Imaging (rarely needed):
To rule out systemic involvement or assess blood vessel changes.
Treatment
First-line:
Corticosteroids: To reduce inflammation.
NSAIDs: For pain and inflammation.
Immunosuppressants:
Methotrexate or azathioprine for more severe cases.
Antibiotics:
If associated with infection.
Lifestyle Adjustments:
Rest, compression stockings, and wound care for ulcers.
Prognosis
Cutaneous PAN generally has a better prognosis than systemic PAN. With appropriate treatment, symptoms can often be well-managed, although some patients experience recurrent episodes.
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