Polyangiitis microscopic cutaneous M30.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 20.03.2023

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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

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Lindberg 1931; Fisher and Orkin 1964

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

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

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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.

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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.

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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 onset with recurrent vasculitic plaques or nodules of 1.0 - 5.0 cm in size, coarse, usually very pressure-dolent, also spontaneously painful, reddish to livid in color (iceberg phenomenon).

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

Livedo racemosa (90%) of the legs, especially of the lower legs.

Petechial hemorrhages especially in the ankle area.

Mild extracutaneous symptoms (detectable in 50%): Arthralgias (often in the ankle joints); myalgias or peripheral neuropathies (lower legs) are less common.

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

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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 hemolytic pharyngeal streptococci are detected.

Mild cases respond well to anti-inflammatories such as diclofenac (e.g. Voltaren Drg.) 50-150 mg/day p.o. or glucocorticoids such as prednisolone (e.g. Decortin H) in low dosage, initially 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 therapy resistance, immunosuppressants such as azathioprine (e.g. Imurek) 100 mg/day, in combination with low-dose glucocorticoids if necessary.

Alternative: Methotrexate (e.g. MTX) 7.5-15 mg/week or cyclophosphamide (e.g. Endoxan), DADPS (e.g. dapsone-fatol) and sulfapyridine.

Alternative: IVIG therapy

Alternative: etanercept

Alternative: anticoagulant therapy with warfarin

Since the disease usually progresses over years, the medication should be carefully selected with regard to benefits/side effects.

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

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

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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.

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Last updated on: 20.03.2023