Recurrent cutaneous necrotising eosinophilic vasculitis

Author(s): Charlie Wang, House Medical Officer, Monash Health, Victoria, Australia. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. October 2018.


What is recurrent cutaneous necrotising eosinophilic vasculitis?

Recurrent cutaneous necrotising eosinophilic vasculitis is a rare form of small vessel vasculitis consisting of pruritic papules, purpura, angioedema, and peripheral blood eosinophilia. Recurrent cutaneous necrotising eosinophilic vasculitis is an eosinophilic subtype of cutaneous small vessel vasculitis. It was first described in 1994 [1].

Who gets recurrent cutaneous necrotising eosinophilic vasculitis?

Recurrent cutaneous necrotising eosinophilic vasculitis has been described in patients aged 17 to 81 years and affects both men and women equally. No predilection for age, sex or ethnicity has been reported [1].

What causes recurrent cutaneous necrotising eosinophilic vasculitis?

Recurrent cutaneous necrotising eosinophilic vasculitis is thought to be idiopathic, as no triggers or causes have been identified. The pathogenic mechanism is not fully understood; however, it is likely a result of eosinophil-mediated vascular damage. Similar to other eosinophilic disorders, interleukin (IL-5), platelet activating factors, and C4 may have a role in the pathogenesis by increasing vascular permeability [2].

What are the clinical features of recurrent cutaneous necrotising eosinophilic vasculitis?

Dermatologic manifestations can occur anywhere on the body, and include:

Most patients report a long history of recurrent pruritic papules, typically over the lower limbs.

Systemic features such as fevers, weight loss arthralgias, and visceral involvement typically associated with systemic vasculitides, are not usually present [3].

How is recurrent cutaneous necrotising eosinophilic vasculitis diagnosed?

On peripheral blood examination, the eosinophil count is often elevated.

Histopathological examination of recurrent cutaneous necrotising eosinophilic vasculitis shows a dermal small vessel vasculitis with fibrinoid necrosis of the vessel walls, but no leukocytoclasia is present. Throughout the dermis, there is exclusive eosinophilic infiltration. Tryptase staining shows increased mast cells in the perivascular areas of the dermis [3].

Indirect immunofluorescence reveals extensive intracellular and extracellular deposition of major basic protein and eosinophil-derived neurotoxin around the dermal vessels [3].

A thorough workup should be performed to rule out underlying systemic conditions and may include full blood examinations, biochemical and immunological tests, especially antineutrophil cytoplasmic antibodies (ANCA), peripheral blood film and bone marrow biopsy if indicated.

What are the differential diagnoses?

What is the treatment of recurrent cutaneous necrotising eosinophilic vasculitis?

Recurrent cutaneous necrotising eosinophilic vasculitis responds to oral corticosteroids as initial and maintenance therapy [2–4].

Long-term, patients may require low dose or alternate daily glucocorticoids, as recurrence after discontinuation of therapy is common. Other agents that have been used in case reports include:

  • Hydroxyurea (as maintenance therapy)
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Tacrolimus
  • Suplatast tosilate [4].

What are the outcomes and complications?

There is little data about the long-term outcome of recurrent cutaneous necrotising eosinophilic vasculitis. However, a benign course is assumed given the steroid-responsiveness of the condition [4].

Some patients may require long-term glucocorticoid therapy.

Potential complications include steroid-related side-effects, ulceration and secondary infection [1].

 

Related information

 

References

  1. Levell N, Mukhtyar C. Chapter 102 - Cutaneous Vasculitis. In: Griffiths C, Barker J, Bleiker T. Rook’s Textbook of Dermatology 9th edition. Wiley Blackwell; 2016. 102.10-102.11
  2. Li W, Cao W, Song H et al. Recurrent cutaneous necrotizing eosinophilic: a case report and review of the literature. Diagnostic Pathology 2013; 8: 185–9. PubMed
  3. Chen K, Pittelkow M, Su D et al. Recurrent cutaneous necrotizing eosinophilic vasculitis. Archives of Dermatology 1994; 130: 1159–66. PubMed
  4. Riyaz N, Sasidharanpillai S, Hazeena C et al. Recurrent cutaneous eosinophilic vasculitis: a rare entity. Indian Journal of Dermatology 2016; 61(2): 235. PubMed

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