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Abdel-Halim et al. Vessel Plus 2022;6:8 https://dx.doi.org/10.20517/2574-1209.2021.40 Page 11 of 14

The presence of associated skin necrosis, ulceration, and gangrenes in the context of palpable purpuric
lesions warrants exclusion of other conditions such as hepatitis C virus-induced vasculitis other causes of
cryoglobulinemic vasculitis and vasculitis associated with underlying vasculopathic disorders such as
[43]
protein C and S deficiency or factor V Leiden mutation . Livedo reticularis/racemosa and skin nodules can
also be a manifestation of polyarteritis nodosa .
[4]
Localized cases of GPA involving mainly the face, nose, ears, and throat should be differentiated from
levamisole induced vasculopathy associated with levamisole-adulterated cocaine abuse. History taking, skin
[44]
biopsy findings, and negative ANCA can help in verifying this condition .
PG-like lesions of GPA should be differentiated from classic PG. The presence of cytoplasmic/anti-PR3-
ANCA, the involvement of internal organs (mainly the lungs), the presence of palisaded neutrophilic and
granulomatous dermatitis, necrotizing vasculitis and basophilic collagen degeneration on skin biopsy, and
not diffuse neutrophilic infiltrate helps in excluding classic PG in such cases . Cultures to exclude
[45]
infectious causes are also important in PG-like lesions or in cases presenting with ulcerated nodules/plaques
that show granulomatous inflammation on biopsy. Finally, many types of cutaneous lymphomas can
present as PG-like lesions , and a skin biopsy will easily establish the diagnosis.
[46]

It is important also to remember that lesions of PNGD can occur in association with a wide range of
systemic disorders other than AAV such as connective tissue disorders, arthritides, Behçet’s disease,
ulcerative colitis, lymphoproliferative disorders, and multiple sclerosis [15,47,48] . Careful clinical, laboratory, and
serological assessment is important in such cases.

Finally, care should be given not to confuse vasculitic lesions of AAV with hemorrhagic or occlusive
pseudovasculitis. Hemorrhagic pseudovasculitis presents with non-palpable petechiae, purpura, or
ecchymoses as a result of vessel wall dysfunction that can occur in many conditions such as metabolic
disorders, nutritional deficiencies, drug reactions, infections, thrombocytopenias, or simply with aging.
Occlusive pseudovasculitis on the other hand presents with livedo, acral cyanosis, or digital
necrosis/gangrene as a result of occlusion of vessel lumen by emboli, thrombi, or other materials. Examples
of occlusive pseudovasculitis include essential cryoglobulinemia, purpura fulminans, coumadin necrosis,
antiphospholipid syndrome, cardiac myxoma, calciphylaxis, cholesterol embolization, and radiation
arteritis . In all cases, a skin biopsy and laboratory investigations can easily verify such conditions.
[49]

MANAGEMENT OF CUTANEOUS LESIONS OF AAV
AAV patients presenting with skin manifestations should follow the same standard algorithms and
guidelines of management used in patients without cutaneous manifestations. These include measures to
induce remission of new-onset organ threatening (life threatening) or non-organ threatening disease, as
[50]
well as measures to induce remissions of relapses and measures to maintain remissions .

Although skin ulcers if present can become contaminated with different types of bacteria, routine
administration of topical or systemic antibiotics is not encouraged, as it was not found to be associated with
reduced bacterial colonization or better healing and can lead to emergence of resistant strains . Systemic
[51]
and non-topical antibiotics are recommended only in the presence of significant evidence of infection such
as increasing pain, erythema in the surrounding skin, progressive increase in ulcer size, pus discharge,
hotness, or edema . Proper management of ulcers should also include removal of necrotic tissue by
[52]
surgical or chemical debridement and the use of appropriate dressings such as hydrocolloid and hydrogel
sheets which absorb fluids from the wound and keep it moist [53,54] . Pain relief measures should also be