Polyangiitis microscopic cutaneous M30.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Apoplexia cutanea boyfriend; cPAN; Cutaneous PAN; Cutaneous periarteritis nodosa; Cutaneous polyarteritis nodosa; kPAN; Livedo with nodules; Lymphocytic Arteritis; Macular Arteritis; Panarteritis nodosa cutaneous; Periarteritis nodosa cutaneous; polyarteritis nodosa cutanea

History
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Lindberg 1931; Fisher and Orkin 1964

Definition
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Rare, eminently chronic, progressive, monoorganic, dermal minus variant of systemic (ANCA-positive) polyarteritis nodosa with a disease pattern that affects the medium-sized arteries at the border between dermis and subcutis. A systemic involvement as in the systemic form of polyarteritis nodosa is not usually present.

Occurrence/Epidemiology
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w>m (in contrast to systemic PAN); cutaneous PAN is more frequent than classical systemic PAN .

Etiopathogenesis
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Not confirmed; detection of immunoglobulins in the small arteries. Common association with hepatitis B/C infections. Also infections caused by parvovirus B19 and Mycobacterium tuberculosis. In children also streptococcus infections.

Medications are also listed as causes. Minocycline seems to play a special role in acne therapy.

In a larger collective of cutaneous PAN patients (n=50) antiphospholipid antibodies were found in a high percentage (90%). This induces endothelial activation with an inflammatory and procoagulatory vascular reaction.

Manifestation
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The mean age of onset of the disease is 40-50 (with a wide range of 11-74) years (Kato A et al. 2018). Patients with ulcerated cutaneous PAN seem to be somewhat older.

Localization
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Mainly located on the extensor sides of the lower extremities.

Clinical features
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Painful nodules and plaques: often beginning with recurrent, 1.0 - 5.0 cm large, coarse, usually very pressure-sensitive, also spontaneously painful, reddish to livid vasculitic plaques or nodules (iceberg phenomenon).

Painful ulcers (60%): due to vasculitis with consecutive thrombosis of medium-sized vessels, painful ulcers develop (sometimes the very painful ulcers are the main clinical symptom). Healing of the ulcers with formation of flat, partially hyperpigmented scars.

Livedo racemosa (90%) of the lower legs.

Petechial haemorrhages, especially in the ankle area.

Mild extracutaneous symptoms (detectable in 50%): arthralgia (often in the ankle joints); myalgia or peripheral neuropathies (lower leg) are less frequent.

Laboratory
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No significant increase in inflammation parameters (BSG; CRP). In larger collectives (Kawakami 2011) in 90% of kPAN cases antiphospholipid antibody detection (70% anti-phosphatidyl-prothrombin antibody; 60% lupus anticoagulant; 20% anti-cardiolipin antibody), relevant ANA titers, positive rheumatoid factor or cryoglobulins.

Histology
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Histopathological algorithm of (cutaneous) polyarteritis nodosa (varies according to Ratzinger et al. 2105)

Remark: the deep lesional excision biopsy leads to the diagnosis !

Accentuated around larger vessels in skin and subcutis
Concerns arterioles and arteries in the subcutis or at the border between cutis and subcutis.
perivascular, intramural and intraluminal leukocytoclasia
Damage to endothelial cells
Fibrin in/around vessel walls
Perivascular extravasation of erythrocytes
No edema in the papillary dermis
Pathologist.Changes restricted to vascular positions, no extravascular, interstitial or soft tissue granulomas
Variable (rather low) eosinophilia
plasma cells or fibrosclerosis to a lesser extent
Reorganization due to lymphocytic vasculitis

Differential diagnosis
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External therapy
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In very mild cases external glucocorticoids in the occlusive dressing such as 0.1% betamethasone cream, 0.1% mometasone ointment (e.g. Ecural) may be sufficient. For foci on the lower legs compression therapy.

Internal therapy
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  • Antibiotic therapy is necessary if haemolytic pharyngeal streptococci are detected.
  • Mild cases respond well to anti-inflammatory drugs such as diclofenac (e.g. Voltaren Drg.) 50-150 mg/day p.o. or glucocorticoids such as prednisolone (e.g. Decortin H) in low doses, initial 20 mg/day p.o., maintenance dose according to clinic.
  • In severe cases glucocorticoids in medium dosage, e.g. prednisolone (e.g. Decortin H) 40-60 mg/day. In case of resistance to therapy, immunosuppressive drugs such as azathioprine (e.g. Imurek) 100 mg/day, possibly in combination with low-dose glucocorticoids.
  • Alternatively: methotrexate (e.g. MTX) 7.5-15 mg/week or cyclophosphamide (e.g. endoxane), DADPS (e.g. Dapson-Fatol) and sulfapyridine
  • Alternative: IVIG therapy
  • Alternative: Etanercept
  • Alternative: anticoagulatory therapy with warfarin
  • Since the disease usually lasts for years, the medication should be carefully selected with regard to benefit/side effects.

Remember! Long-term control of the patient, since in (few) individual cases transition to systemic polyarteritis nodosa is known.

Progression/forecast
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Cheap. An eminently chronic, intermittent course is typical. Own cases show a course that lasts for years.

Note(s)
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There is neither evidence nor proof that cutaneous polyarteritis nodosa can develop into systemic PAN.

Case report(s)
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Medical history: The multimorbid, 78-year-old obese patient reported recurrent painful nodules and plaques. These occurred for the first time after implantation of a hip joint prosthesis and healed spontaneously after 2-3 months. 5 years later recurrence of the skin changes lasting several months. 10 years later recurrence of painful nodules and plaques on both lower legs, which persist 2 years later. The patient suffers from permanent atrial fibrillation (I48.9) which is treated with direct oral anticoagulants (DOAK) and a pacemaker. She also has type 2 diabetes mellitus (E11.90) which is treated with oral antidiabetics.

Findings: On both lower legs painful red plaques and nodules of 2-3 cm in size are found. Next to them flat non-irritating scars and hyperpigmentation.

Histology: Inflammatory changes in arterioles and arteries in the subcutaneous tissue and at the border between cutis and subcutis. Here also described necroses and focal calcifications.

Laboratory: Inflammation parameters (BSG; CRP) in the normal range. Blood count: o.B. HbA1c: 6.1%; antiphospholipid antibodies +; ANA 1:80; Yersinia AK elevated with 66 U/ml; urine: nitrite++; bacteria+; leukocytes ++;

chest: o.B.; no indication of old or fresh tuberculosis.

Therapy: local treatment with a 0.1% triamcinolone ointment. No stem therapy was applied.

Literature
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  1. Akhter A et al (2015) Cutaneous manifestations of viral hepatitis. Curr Infect Dis Rep 17:452
  2. Bauza A et al (2002) Cutaneous polyarteritis nodosa. Br J Dermatol 146: 694-699
  3. Gushi A et al (2000) Three cases of polyarteritis nodosa cutanea and a review of the literature. J Dermatol 27: 778-781
  4. Jorizzo L et al (1991) Low-dose weekly methotrexate for unusual neutrophilic vascular reactions: cutaneus polyarteritis nodosa and Behçet's disease. J Am Acad Dermatol 24: 973-978
  5. Kato A et al (2018) Clinical and Laboratory Markers Associated With Relapse in Cutaneous Polyarteritis Nodosa. JAMA Dermatol 154:922-926.
  6. Kawakami T et al (2011) Correlation of livedo racemosa, cutaneous inflammatory plaques, and antiphospholipid antibodies in patients with cutaneous polyarteritis nodosa. Medicine (Baltimore) 90:119-124.
  7. Lindberg K (1931) A contribution to the knowledge of periarteritis nodosa. Acta Med Scand 76: 183-225
  8. Mimouni D et al (2003) Cutaneous polyarteritis nodosa in patients presenting with atrophie blanche. Br J Dermatol 148: 789-794
  9. Mondal R et al (2014) Childhood Polyarteritis Nodosa: a prospective multicentre study from eastern India. Indian J Pediatr 81:371-374
  10. Müller CSL et al (2016) Diagnostic and histological features of cutaneous vasculitis/vasculopathies. Act Dermatol 42: 286-301
  11. Okazaki C et al (2011) A case of cutaneous protothecosis in a polyarteritis nodosa patient and review of cases reported in Japan. Dermatol Online J 17:20
  12. Pagnoux C et al (2010) Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis rheum 62:616-626.
  13. Ratzinger G et al (2015) The Vasculitis Wheel-an algorithmic approach to cutaneous vasculitis. JDDG 1092-1118
  14. Schlitz M et al (1991) Periarteritis nodosa cutanea benigna-a frequently misunderstood disease. Act Dermatol 17: 162-165
  15. Schneider SW (2016) Poyarteriitis nodosa - a mystery? Very close 32: 24-29
  16. Sunderkötter C et al (2015) Cutaneous symptoms of various vasculitides. dermatologist 66: 589-598
  17. Trueb RM et al (1995) Periarteritis nodosa cutanea. dermatologist 46: 568-572
  18. Zoshima T et ak. (2013) A case of refractory cutaneous polyarteritis nodosa in a patient with hepatitis B carrier status successfully treated with tumor necrosis factor alpha blockade. Mod Rheumatol 23:1029-1033

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020