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166 M. Papi and E. Fiscarelli
Fig. 12 Multiple painful leg and feet ulcers in 73 years old female with cryoglobulinaemic
vasculitis
Atypical Wounds and Wounds Resulting from Infection 167
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Fig. 13 Necrotic and ulcerative lesions in a livedoid leg of a patient with panarteritis nodosa
168 M. Papi and E. Fiscarelli
Pyoderma Gangrenosum
It is a cutaneous inflammatory disease of unknown etiology at ulcerative evolution,
included in the group of “neutrophilic dermatoses”. It has also been considered as a
cutaneous marker for some autoinflammatory diseases (Marzano et al. 2016). The
initial lesion is characterized by a small purplish papule-pustule that increases in the
peripheral area with the formation of a violaceous skin ulcer (Papi et al. 1997). It
typically affects adults and tends to recur. The ulcer is very painful and, sometimes,
grows on one side and decreases on the opposite. The active area shows an
undermined purplish margin. It tends to heal leaving a characteristic atrophic-scar
outcomes (cribriform) (Fig.14). The lesion can be single but, more often, is multiple
and localized at the lower limbs (Maverakis et al. 2020). No skin area may be
excluded. The localization on previous surgical scars is common.
Several clinical forms are known: superficial, granulomatous, panniculiticsuppurative (Fig. 15).
About 50% of patients present an associated disease (intestinal inflammatory
pathologies, seronegative arthritis, rheumatoid arthritis, chronic hepatitis, monoclonal gammopathy) (Table 2). In the atypical cases (multifocality) or severe,
haematological diseases (myelofibrosis, leukaemia) and solid tumours should be
excluded (Croitoru et al. 2020; Janowska et al. 2020).
The non-specific histological exam shows a diffuse dermal infiltration of neutrophil leukocytes responsible, sometimes, of non-specific signs of leukocytoclastic
vasculitis. There is no specific diagnostic test and the diagnosis is usually obtained
from exclusion. Its early recognition and proper management with prompt initiation
of immunosuppressive therapy are essential to improve the quality of life and the
prognosis of patients.
In the forms without concomitant pathologies, therapy is based on corticosteroids (1 mg/prednisone/kg/die), cyclosporine (3–5 mg/kg/die), clofazimine and
dapsone. Good results have been reported using anti-TNFalpha drugs (etanercept
and infliximab) and new biological molecules
Diagnosis: it is an exclusion diagnosis. A biopsy and the histological examination may help in many cases in which a clinical suspicion is present.
,
(Papi and PapiC. 2018).
Occlusive Non Vasculitic Vasculopathies
Occlusive cutaneous small vessel vasculopathies cause several cutaneous lesions
which may result in AW, which are difficult to distinguish from inflammatory
ulcerative disorders and clinically atypical. Embolization due to cholesterol and
oxalate emboli, cutaneous intravascular metastasis from visceral malignancies,
atrial myxomas, intravascular angiosarcoma, intralymphatic histiocytosis,
intravascular lymphomas, endocarditis, crystal globulin vasculopathy, hypereosinophilic syndrome, and foreign material have been described to have a
pathogenetic role in these disturbances. (Velasco et al. 2017 ). When platelet
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Fig. 14 Typical lesion of PG tending to extend with purplish edges and ulcerated central area
cribriform like
170 M. Papi and E. Fiscarelli
Fig. 15 Suppurative PG in a 72 year old male with recurrent septic arthritis
Table 2 Systemic diseases
associated with PG (50% of
patients)
Seronegative arthritis Myelodysplastic syndrome
Rheumatoid arthritis Myelocytic
Chron’s disease Lymphoproliferative disorders
Ulcerative colitis Autoimmune hepatitis
leukaemia
pugging is involved, heparin necrosis, thrombocytosis secondary to myeloproliferative disorders, paroxysmal nocturnal haemoglobinuria, and thrombotic thrombocytopenic purpura may be responsible for the occlusion. A special role may be
played by cryoproteins (cryofibrinogenemia, cryoglobulinemia) which may cause
multiple and extremely painful atypical ulcers. Systemic coagulopathies due to
defects in C and S proteins, coumarin/warfarin-induced skin n
ecrosis, disseminated
intravascular coagulation, and antiphospholipid antibody/lupus anticoagulant syndrome may also result in occlusive non vasculitic vasculopathy. Finally, livedoid
vasculopathy is a distinct entity which may also cause occlusion of the vessels and
result in recurrent skin ulcers on the legs with ambiguous etiology and clinical
interpretation (Jorizzo
1998).Though many of the above conditions can cause skin
ulcers which may become chronic and atypical, we will limit our description to
livedoid vasculopathy and antiphospholipid antibodies-associated leg ulcers.
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Livedoid Vasculopathy
Livedoid vasculopathy (LV) is a chronic, painful, thrombo-occlus ive cutaneous
vasculopathy that involves the distal lower extremities and feet. Typical clinical
features include livedoid skin changes (linear or angular, erythematous nodules),
atrophie blanche (smooth, ivory-white plaques), and painful atypical ulceration.
The diagnosis needs to be confirmed by a histological exam which shows characteristic vascular abnormalities, including intraluminal thrombosis, endothelial
proliferation, and subintimal hyaline degeneration and no signs of vasculitis
et al. 1998)
For several years the confusing term “livedo vasculitis” has been used to identify
a characteristic and an autonomous clinical entity. It presented focal livedoid
lesions, purplish ridge-like (livedo reticularis) and a treelike aspect (livedo race-
mosa), often associated to painful ulcerations and starred scars (Figs. 16 and 17.)
which are located in the lower third of the legs and in the dorsum of the feet. It
preferentially affects young women and has a chronic course with frequent worsening in winter (livedo reticularis with winter ulcerations) or in summer (livedo
with summer ulcerations) (Bilgic et al. 2021) (Table 3).
The histological feature, also very specific, includes thrombosis of the “ candelabrum artery”, hyalinization of the vessel walls, swelling of the endothelial cells
and poor infiltration of lymphocytes. These aspects suggest a thrombotic pathogenesis of LV, as it is also confirmed by several studies which indicate the presence
of various thrombotic defects associated to LV patterns and the clear presence of
platelet activation (Papi et al. 1998; Alavi et al. 2013).
An incisional surgical skin biopsy and the histological confirm of the diagnosis
is mandatory. An extensive investigation for inherited or acquired thrombophilic
disorders, immune-related ulcerating diseases and metabolic disturbances must be
performed (Table 4). Therapeutic options: antiplatelet agents have been extensively
used for a long period as a first-line therapy. They did not demonstrate to be
effective in many cases. Anticoagulation by hepari n was the most successful
treatment as reported in some case-series study (Gardette et al. 2018). Recent
studies reported good results with the use of rivaroxaban, a direct factor Xa inhibitor that prevents thrombus formation. (Weishaupt et al. 2016; Lee and Kim 2016).
It may be increasingly recommended in the near future. In some cases with
recurrences the therapy is a real challenge and the clinical lesions are respectively
worsened by hot or cold environmental temperatures or other physical variants
(Papi 2006).
Diagnosis: histological examination, clinical aspects, young age, pain.
,
(Papi
Antiphospholipid Antibodies-Associated Leg Ulcers
Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which
autoantibodies are produced against a variety of phospholipids and phospholipidbinding proteins. They are called antiphospholipid antibodies (APLAs). APL share
172 M. Papi and E. Fiscarelli
Fig. 16 Multiple ulcerative lesions with branch morphology in a 35 years old woman
anticoagulant properties in vitro, while associate to a higher incidence of thrombotic
phenomena in vivo (Knight and Kanthi 2022). In fact, the venous and arterial
thromboses and the repeated abortions are the main clinical features of the syndrome that sometimes precedes or is connected to systemic lupus erythematosus.
The skin is often site of necrotizing or gangrenous lesions at ulcerative evolution
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Fig. 17 Livedoid aspects and racemose necrotizing lesion in a 23 years-old female
Table 3 Diagnostic
screening in occlusive
non-vasculitic vasculopathies
Immunologic exams
ANA, antibodies anti ENA,
glycoprotein 1, C3,C4, CH50, CIC,
ß
2
cryoglobulinemia, cryo-blood fibrinogen
Screening for thrombophilia
Haplotypes of MTHFR, PAI-1, factor II, Factor V
Leiden, LAC, antithrombin III, factors C and S of
the coagulation, PT, PTT; fibrinogen
Metabolic screening
Homocystinemia, vit B12, folic acid, homocysteine
dosing and oxalic acid in urines
Instrumental exams
Ecocolordoppler of lower limbs, ecocardiodoppler
anticardiolipin and anti-
and, sometimes, of less evident manifestations characterized by small irregular and
branched ulcerations that result in atrophic-cicatricial areas resembling “white
atrophy” (Fig. 18) (Flores et al. 2021). In these cases the histological exam shows a
picture of thrombotic microangiopathy of the dermal and subcutaneous vessels,
lacking real signs of vasculitis. However, severity of deep venous-arterial thrombotic events often causes serious systemic complications. The relation between
174 M. Papi and E. Fiscarelli
Fig. 18 Antiphospholipid antibodies chronic atypical ulcer and residual scars of previous ulcers
in a 45 years-old female
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APL immunity and the associated thrombotic events is not clear, yet. From time to
time APLs have been considered responsible for a reduced production of prostacyclin, thus interfering with the anticoagulant proteins of protein C and inhibiting
the fibrinolytic process.
Lifelong anticoagulation with vitamin K antagonists remains the cornerstone of
the therapy for thrombotic APS and its
complication (warfarin) (Cohen et al. 2020).
Rivaroxaban and antiplatelets (acetylsalicylic acid) are considered second line
drugs. However, contradictory guidelines have been published in the last decade.
Immunosuppressive (corticosteroids, rituximab) and antimalarial synthesis treatments (hydroxyquinolines), if associated to lupus erythematosus, may be advised.
Diagnosis: positivity for APS, no histological aspects of vasculitis, pain.
Drug-Induced
Hydroxyurea
Hydroxyurea (HU) is an antiproliferative molecule mainly used by haematologists
to treat chronic myeloid leukaemia and polycythaemia vera. In the case of prolonged therapies, dermatomyositis-like lesions, cutaneous atrophy, spread alopecia
(baldness), linear hyperpigmentation of the nails, dyskeratosis and multiple
epitheliomas of the light-exposed areas can appear on the skin (Papi et al. 1993).
The possible onset of ulcers on the legs has been known for several years. They are
mainly supra-malleolar ulcers, almost always very painful, roundish shape, covered
with a constant yellowish fibrinous type material (Fig. 19). The preferred location is
an area that covers a bone protuberance and that is often the site of ulcers which
may be confused with other type of wounds (Sirieix et al. 1999). Patients sometimes
refer to a trauma as a triggering factor. The drug antiproliferative action makes
difficult it to repair the ulcer damage. The cutaneous atrophy and the thinning out of
the capillary bed responsible for ischemia condition are other reasons that confirm
HU direct role. The increase in the absolute number of platelets and/or their
increased clustering in some of the haematological diseases treated with HU can
constitute a favouring factor. Recent studies have demonstrated a significant volume increase of blood cells and the reduction of their deformability in patients
treated with HU. The largest and most rigid erythrocyte can induce microcirculatory
obstructions and, therefore, tissue hypoxia. HU interruption or substitution is
generally followed by a significant improvement of the ulcer. Good results have
been obtained by using vasodilators, pentoxifylline and elastic compression (Bulte
et al. 2021).
Diagnosis: HU therapy, lower extremities, over bone prominences location, pain.
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