Polyangiitis microscopic cutaneous M30.0

Lindberg 1931; Fisher and Orkin 1964

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.

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

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.

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.

Mainly located on the extensor sides of the lower extremities.

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.

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.

• Polyangiitis microscopic (classic form): severe p-ANCA-positive systemic disease.
• Livedovasculopathy: no nodularity, described Livedo Raceoma with painful, splashy ulcers
• Erythema induratum Bazin : the extent to which identical disease patterns are present is not yet clear.
• Erythema nodosum: acute recurrent painful nodules on the extensor sides of the lower legs

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.

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

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

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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