**4.4 Erythromelalgia**

Erythromelalgia is characterized by vasodilation of the extremities, especially in male children, and is usually associated with limb warm-up, pain and burning. It is believed that there is some change in calcium channels, so therapy is directed toward this focus.

In the case of primary erythromelalgia, anesthetics, antiarrhythmics, anticonvulsants and even oral magnesium may be used. In the case of secondary erythromelalgia, besides the treatment already discussed, the underlying disease needs to be controlled [27].

Its treatment includes topical drugs like 5% lidocaine and 0.075% capsaicin. For oral medications, we have amitriptyline 10 mg/day, gabapentin 900–1800 mg/ day, pregabalin 75 mg/day, flecainide 200 mg/day and buflomedil 200–330 mg/day. Tricyclic antidepressants, selective serotonin reuptake inhibitors and, in selected cases, acetylsalicylic acid, beta blockers and calcium channel antagonists may be excellent associations. In refractory cases, we may use epidural infusions of opioids, bupivacaine and, in the latter case, sympathectomy. However, responses are quite variable and complete remission of symptoms is rarely observed [27, 33].

#### **4.5 Livedo reticularis**

Reticular livedo is a common dermatological manifestation in which the limb in question suffers a vasospasm and has a cyanotic, erythematous and erythematousviolet coloration. When the blood plot does not have a confluent pattern, it is called a racemose livedo. In clinical practice, it may be isolated by cold or trauma or may be associated with some systemic diseases such as lupus erythematosus, scleroderma and HIV. Although it is more common in the limbs, it can also affect the trunk and there may be ulcerations. Cold stimulates vasospasm, when the cause is removed, however, over time, vessels may become permanently dilated and become permanently telangiectatic [27, 34].

The treatment of livedo, primarily, is protection against the cold. Vasodilators may be an alternative and corticosteroids should be avoided as much as possible. If there are ulcerations or a racemose livedo associated with antiphospholipid antibody syndrome, anticoagulation is recommended. When livedo is associated with some underlying disease, the management of the disease usually improves its manifestation. Other medications may be tried such as danazol, tissue plasminogen activator (tPA), pentoxifylline and antiplatelets. Immunosuppressants such as azathioprine and sympathectomy are reserved for refractory cases [27, 34, 35].

#### **4.6 Raynaud's phenomenon**

It is a paroxysmal vasospastic disorder characterized by the simultaneous alternation of pallor (vasoconstriction), cyanosis (blood stasis) and redness (compensatory vasodilation).

We consider Raynaud's disease (20% of the cases) when it occurs primarily and not associated with other acne. And we consider Raynaud's phenomenon (80%) when it occurs secondary to another disease [27].

Treatment consists of treating the basic disease and quitting smoking. Protection against the cold is necessary to avoid triggering the frame. In idiopathic forms, the use of nifedipine 30–120 mg/day can be used. Other treatment modalities may also be employed such as topical nitroglycerin, iloprost, losartan, serotonin receptor inhibitors, phosphodiesterase inhibitors, n-acetylcysteine, botulinum toxin, bosentan, platelet inhibitors and fibrinolytics. In refractory cases, sympathectomy may be performed [27].

#### **4.7 Erythema pernio or perniosis**

Perniosis is a rare panniculitis that develops with painful erythematousviolaceous nodules in young people more susceptible to cold. There is also a certain uncertain relationship with tobacco. Treatment, like other cold-related conditions, requires protection from the cold such as appropriate clothing, gloves, socks, boots and smoking cessation. Behavior changes and topical corticoid creams can help to heal the lesions. Some vasodilators may also be applied as nicotinic acid, nifedipine and pentoxifylline. In refractory cases, sympathectomy and UVB phototherapy may also be considered [36].

#### **4.8 Livedoid vasculitis or Millian's white atrophy**

Livedoid vasculitis is one of the most common vasculopathies, described in 1929, and can affect up to about 5% of the healthy population, reaching 70% in patients

**149**

used [27].

**4.9 Atherosclerosis obliterans**

and vascular surgery may be used [38].

two systems can be fatal [40].

**4.10 Thromboangiitis obliterans (Buerger's disease)**

**4.11 Malignant atrophic papulosis (Köhlmeier-Degos disease)**

*Vasculitis and Vasculopathies*

*DOI: http://dx.doi.org/10.5772/intechopen.92778*

established link is with chronic venous insufficiency [35].

associated or not with heparin and aspirin [27, 35].

line and buflomedil hydrochloride [27, 35].

• Vasodilators such as nifedipine [27, 35].

with venous ulcers. Some authors differentiate livedoid vasculitis from Millian's white atrophy in relation to etiology. Livedoid vasculitis is persistent livedoid reticularis associated with an ulcer [37]. White atrophy is a white atrophy scar with a stellar pattern most common in the lower limbs of women aged 30–60 years that may or may not be associated with collagenases and neoplasia; however, the best-

The treatment for this disease is divided into some of the following groups:

• Drugs stimulating endogenous fibrinolytic activity such as danazol, the

activating factor of recombinant tissue plasminogen, rt-PA, low dose alteplase,

• Drugs such as dipyridamole, cilostazol, the thienopyridine group (clopidogrel, ticlopidine hydrochloride, whether associated with aspirin) and sarpogrelate.

• Hemorheological drugs that decrease blood viscosity, increase red blood cell flexibility and improve circulation. From this group, the example is pentoxifyl-

• Modulating lymphocyte response therapy as systemic PUVA phototherapy [27].

• Other drugs such oral corticosteroids, intravenous immunoglobulin, cyclospo-

It is a chronic disease associated with diabetes that gradually occludes the vessel

The priority treatment is smoking cessation and rest. Iloprost IV may be an alternative to retard evolution. In many cases, amputation is required, with excision of the gangrenous fingers, sympathectomy and surgeries such as arterialization of the venous arch of the foot. The vascular surgeon is extremely important for follow-up [27, 39].

Malignant atrophic papulosis (MAP) is an obliterating endovasculitis of smalland medium-sized arteries that produces tissue infarction as its main feature. It is considered an uncommon disease of unknown cause and can affect the skin, gastrointestinal tract and central nervous system, and the involvement of these last

light. It affects the feet more than the hands in patients over 50 years. There is no specific treatment, but oral corticosteroids may be employed associated with diabetes with strict disease control. Behavioral change with diabetes mellitus improvement and antibiotic therapy, if secondary injuries, is the therapy of choice. Vasodilators do not have their proven efficiency. In severe cases, sympathectomy

For patients with any associated thrombophilia, warfarin, unfractionated heparin, low molecular weight heparin and even heparin minidoses may also be

rine, hyperbaric oxygen therapy and intravenous iloprost [27].

### *Vasculitis and Vasculopathies DOI: http://dx.doi.org/10.5772/intechopen.92778*

*Vascular Biology - Selection of Mechanisms and Clinical Applications*

**4.5 Livedo reticularis**

permanently telangiectatic [27, 34].

**4.6 Raynaud's phenomenon**

thectomy may be performed [27].

**4.7 Erythema pernio or perniosis**

also be considered [36].

when it occurs secondary to another disease [27].

**4.8 Livedoid vasculitis or Millian's white atrophy**

tory vasodilation).

bupivacaine and, in the latter case, sympathectomy. However, responses are quite

Reticular livedo is a common dermatological manifestation in which the limb in question suffers a vasospasm and has a cyanotic, erythematous and erythematousviolet coloration. When the blood plot does not have a confluent pattern, it is called a racemose livedo. In clinical practice, it may be isolated by cold or trauma or may be associated with some systemic diseases such as lupus erythematosus, scleroderma and HIV. Although it is more common in the limbs, it can also affect the trunk and there may be ulcerations. Cold stimulates vasospasm, when the cause is removed, however, over time, vessels may become permanently dilated and become

The treatment of livedo, primarily, is protection against the cold. Vasodilators may be an alternative and corticosteroids should be avoided as much as possible. If there are ulcerations or a racemose livedo associated with antiphospholipid antibody syndrome, anticoagulation is recommended. When livedo is associated with some underlying disease, the management of the disease usually improves its manifestation. Other medications may be tried such as danazol, tissue plasminogen activator (tPA), pentoxifylline and antiplatelets. Immunosuppressants such as azathioprine and sympathectomy are reserved for refractory cases [27, 34, 35].

It is a paroxysmal vasospastic disorder characterized by the simultaneous alternation of pallor (vasoconstriction), cyanosis (blood stasis) and redness (compensa-

We consider Raynaud's disease (20% of the cases) when it occurs primarily and not associated with other acne. And we consider Raynaud's phenomenon (80%)

Treatment consists of treating the basic disease and quitting smoking. Protection against the cold is necessary to avoid triggering the frame. In idiopathic forms, the use of nifedipine 30–120 mg/day can be used. Other treatment modalities may also be employed such as topical nitroglycerin, iloprost, losartan, serotonin receptor inhibitors, phosphodiesterase inhibitors, n-acetylcysteine, botulinum toxin, bosentan, platelet inhibitors and fibrinolytics. In refractory cases, sympa-

Perniosis is a rare panniculitis that develops with painful erythematousviolaceous nodules in young people more susceptible to cold. There is also a certain uncertain relationship with tobacco. Treatment, like other cold-related conditions, requires protection from the cold such as appropriate clothing, gloves, socks, boots and smoking cessation. Behavior changes and topical corticoid creams can help to heal the lesions. Some vasodilators may also be applied as nicotinic acid, nifedipine and pentoxifylline. In refractory cases, sympathectomy and UVB phototherapy may

Livedoid vasculitis is one of the most common vasculopathies, described in 1929, and can affect up to about 5% of the healthy population, reaching 70% in patients

variable and complete remission of symptoms is rarely observed [27, 33].

**148**

with venous ulcers. Some authors differentiate livedoid vasculitis from Millian's white atrophy in relation to etiology. Livedoid vasculitis is persistent livedoid reticularis associated with an ulcer [37]. White atrophy is a white atrophy scar with a stellar pattern most common in the lower limbs of women aged 30–60 years that may or may not be associated with collagenases and neoplasia; however, the bestestablished link is with chronic venous insufficiency [35].

The treatment for this disease is divided into some of the following groups:


For patients with any associated thrombophilia, warfarin, unfractionated heparin, low molecular weight heparin and even heparin minidoses may also be used [27].
