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Page 8 of 14 Abdel-Halim et al. Vessel Plus 2022;6:8 https://dx.doi.org/10.20517/2574-1209.2021.40

Erythema elevatum diutinum-like nodules and plaques mainly involving the extensor aspects of the limbs
[31]
with symmetric distribution have been described early in the course of MPA . Digital ischemia and
[4]
gangrene can also rarely develop in MPA .
Clinical presentation of PNGD which can develop with AAV
The classic clinical presentation of PNGD is erythematous or flesh-colored papules which may develop
central umbilication or crustations. They usually take a symmetrical distribution on the extremities, mainly
involving elbows and knees and but frequently other sites [15,32] [Figure 10].

Clinical presentation of non-specific skin lesions in AAV
Many non-specific skin lesions can occur in AAV patients. Non-specific maculopapular rash is the second
[2]
most frequently encountered skin manifestation in AAV occurring in 8% of cases . In GPA, non-specific
oral lesions develop in 10%-60% of patients and may be the presenting sign of the disease in 5% of cases [33,34] .
[3]
They take the form of oral erosions or ulcers [Figure 11], strawberry gingivitis with exophytic hyperplasia,
and mucosal petechial spots and erythematous granular appearance (with or without loss of alveolar bone
and loosening of teeth) [33-35] . Other non-specific lesions in GPA include skin ulcers (with no pathological
[24]
[7]
[3]
evidence of vasculitis or granulomas) , erythema nodosum-like lesions , xanthelasmas , sterile
pustules , acneiform lesions , and chronic eyelid edema and infiltration . EGPA patients commonly
[36]
[7]
[37]
develop urticarial-like, angioedema-like, erythema multiforme-like, and chronic itchy lichenified prurigo
nodularis-like lesions, as well as sterile pustules [19,38] . Allergic manifestations such as pruritus are also
frequently observed in EGPA [2,39] . Cases of EGPA presenting with Well’s syndrome (eosinophilic
panniculitis)-like picture have been reported . Urticarial lesions may also develop with MPA . Table 1
[40]
[4]
represents a summary of the clinicopathological spectrum of cutaneous lesions in AAV.
CUTANEOUS LESIONS IN RELATION TO DIFFERENT TYPES OF AAV AND DIFFERENT
TYPES OF ANCA
Certain skin lesions were noticed to develop more frequently in certain types of AAV. For example, livedo
reticularis/racemosa and segmentary edema develop significantly more frequently in MPA. Non-specific
manifestations such as pruritus, urticaria, and maculopapular rash are more likely encountered in EGPA.
Urticaria is uncommon in GPA and MPA. Painful skin lesions and skin ulcers occur more significantly in
GPA and EGPA when compared to MPA [2,39] . Oral ulcers are also more frequently associated with GPA as
[39]
[39]
compared to EGPA and MPA . PG-like lesions and palpebral xanthomas can only occur in GPA .
Cutaneous lesions were found to be the presenting manifestation of AAV in patients with positive
cytoplasmic/anti-PR3ANCA or in ANCA-negative patients when compared to patients with positive
perinuclear/anti-MPO ANCA. The same was observed as regards the development of the typical vasculitic
skin lesions of purpura/petechiae and splinter hemorrhages. Coinciding with their significantly higher
occurrence in MPA, livedo reticularis/racemosa were reported more often in patients with positive
perinuclear/anti-MPO ANCA than in those without. Non-specific manifestations such as pruritus and
urticaria were more frequently encountered in ANCA-negative patients. The presence of multiple types of
[2]
cutaneous manifestations was most frequently reported in patients who were ANCA-negative .

CUTANEOUS LESIONS IN RELATION TO SPECIFIC ORGAN INVOLVEMENT AND
SEVERITY OF SYSTEMIC MANIFESTATIONS OF AAV
GPA patients with skin lesions were more prone to have pulmonary, renal, neurologic, musculoskeletal, and
gastrointestinal involvement and more severe manifestations [2,39] . Similarly, EGPA patients with skin lesions
were more likely to suffer from renal, neurologic, musculoskeletal, and gastrointestinal involvement. They