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179 VASCULITIS
E.J. Mayeaux, Jr., MD
Richard P. Usatine, MD
PATIENT STORY
A 21-year-old woman presented with a 3-day history of a painful
purpuric rash on her lower extremities (Figure 179-1 and Figure
179-2). The lesions had appeared suddenly, and the patient had expe-
rienced no prior similar episodes. The patient had been diagnosed
with a case of pharyngitis earlier that week and was given a course of
clindamycin. She had not experienced any nausea or vomiting, fever,
abdominal cramping, or gross hematuria. Urine dipstick revealed
blood in her urine, but no protein. The typical palpable purpura on
the legs is consistent with Henoch-Schnlein purpura (HSP).
INTRODUCTION
Vasculitis refers to a group of disorders characterized by inammation
and damage in blood vessel walls. They may be limited to skin or may
be a multisystem disorder. Cutaneous vasculitic diseases are classied
according to the size (small versus medium to large vessel) and type
of blood vessel involved (venule, arteriole, artery, or vein). Small-
and medium-size vessels are found in the dermis and deep reticular
dermis, respectively. The clinical presentation varies with the inten-
sity of the inammation, and the size and type of blood vessel
involved.
1
SYNONYMS
Hypersensitivity vasculitis is also known as leukocytoclastic vasculitis.
HSP is a type of leukocytoclastic vasculitis.
EPIDEMIOLOGY
HSP (Figure 179-1 to Figure 179-3) occurs mainly in children
with an incidence of approximately 1 in 5000 children annually.
2

It results from immunoglobulin (Ig) A-containing immune com-
plexes in blood vessel walls in the skin, kidney, and GI tract. HSP
is usually benign and self-limiting, and tends to occur in the
springtime. A streptococcal or viral upper respiratory infection
often precedes the disease by 1 to 3 weeks. Prodromal symptoms
include anorexia and fever. Most children with HSP also have joint
pain and swelling with the knees and ankles being most commonly
involved (Figure 179-3). In half of the cases there are recur-
rences, typically in the rst 3 months. Recurrences are more
common in patients with nephritis and are milder than the original
episode. To make the diagnosis of HSP, establish the presence of 3
or more of the following:
3
~ Palpable purpura
~ Bowel angina (pain)
FIGURE 179-1 Henoch-Schnlein purpura presenting as palpable
purpura on the lower extremity. The visible sock lines are from lesions
that formed where the socks exerted pressure on the legs. (Courtesy of
Richard P. Usatine, MD.)
FIGURE 179-2 Close-up of palpable purpura from the patient in
Figure 179-1. Some lesions look like target lesions but this is Henoch-
Schnlein purpura and not erythema multiforme. (Courtesy of Richard
P. Usatine, MD.)
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~ GI bleeding
~ Hematuria
~ Onset 20 years
~ No new medications
Some patients with systemic lupus erythematosus (SLE)
(Figure 179-4 and Figure 179-5), rheumatoid arthritis (RA),
relapsing polychondritis, and other connective tissue disorders
develop an associated necrotizing vasculitis. It most frequently
involves the small muscular arteries, arterioles, and venules.
The blood vessels can become blocked leading to tissue necrosis
(Figure 179-4 and Figure 179-5). The skin and internal organs
may be involved.
Leukocytoclastic vasculitis (Figure 179-6 to Figure 179-8) is
the most commonly seen form of small vessel vasculitis. Prodro-
mal symptoms include fever, malaise, myalgia, and joint pain. The
palpable purpura begins as asymptomatic localized areas of
cutaneous hemorrhage that become palpable. Few or many dis-
crete lesions are most commonly seen on the lower extremities
but may occur on any dependent area. Small lesions itch and are
painful, but nodules, ulcers, and bullae may be very painful.
Lesions appear in crops, last for 1 to 4 weeks, and may heal with
residual scarring and hyperpigmentation. Patients may experience
1 episode (drug reaction or viral infection) or multiple episodes
(RA or SLE). The disease is usually self-limited and conned to
the skin. To make the diagnosis, look for presence of 3 or more of
the following:
4
~ Age older than 16 years
~ Use of a possible offending drug in temporal relation to the
symptoms
~ Palpable purpura
~ Maculopapular rash
~ Biopsy of a skin lesion showing neutrophils around an arteriole or
venule
Systemic manifestations of leukocytoclastic vasculitis may include
kidney disease, heart, nervous system, GI tract, lungs, and joint
involvement.
ETIOLOGY AND PATHOPHYSIOLOGY
Vasculitis is dened as inammation of the blood vessel wall. The
mechanisms of vascular damage consist of either a humoral
response, immune complex deposition, or cell-mediated
T-lymphocyte response with granuloma formation.
5
Vasculitis induced injury to blood vessels may lead to increased
vascular permeability, vessel weakening, aneurysm formation,
hemorrhage, intimal proliferation, and thrombosis that result in
obstruction and local ischemia.
5
Small-vessel vasculitis is initiated by hypersensitivity to various
antigens (drugs, chemicals, microorganisms, and endogenous anti-
gens), with formation of circulating immune complexes that are
deposited in walls of postcapillary venules. The vessel-bound
immune complexes activate complement, which attracts polymor-
phonuclear leukocytes. They damage the walls of small veins by
release of lysosomal enzymes. This causes vessel necrosis and local
hemorrhage.
FIGURE 179-4 Necrotizing vasculitis in a young Asian woman with
systemic lupus erythematosus. The circulation to the ngertips was
compromised and the woman was treated with high-dose intravenous
steroids and intravenous immunoglobulins to prevent tissue loss.
(Courtesy of Richard P. Usatine, MD.)
A
FIGURE 179-3 Henoch-Schnlein purpura in an 11-year-old girl. A. In
addition to the palpable purpura, this patient also had abdominal pain.
Note how the seam of her jeans is visible in the purpuric pattern. B. She
also had knee pain and swelling and was walking with a limp. (Courtesy
of Richard P. Usatine, MD.)
B
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FIGURE 179-5 Vasculitis ulcer on the leg of a woman with systemic
lupus erythematosus. (Courtesy of Everett Allen, MD.)
FIGURE 179-6 Leukocytoclastic vasculitis on the leg of a woman.
(Courtesy of Richard P. Usatine, MD.)
FIGURE 179-7 Very palpable purpura on the leg of a middle-aged
woman with leukocytoclastic vasculitis. (Courtesy of Eric Kraus, MD.)
FIGURE 179-8 Vasculitis on the abdomen of a middle-aged woman
who also has the vasculitis on her legs. (Courtesy of Everett Allen, MD.)
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Small-vessel vasculitis most commonly affects the skin and rarely
causes serious internal organ dysfunction, except when the kidney is
involved. Small-vessel vasculitis is associated with leukocytoclastic
vasculitis, HSP, essential mixed cryoglobulinemia, connective tissue
diseases or malignancies, serum sickness and serum sickness-like
reactions, chronic urticaria, and acute hepatitis B or C infection.
Hypersensitivity (leukocytoclastic) vasculitis causes acute inam-
mation and necrosis of venules in the dermis. The term leukocyto-
clastic vasculitis describes the histologic pattern produced when leu-
kocytes fragment.
RISK FACTORS
Viral infections
Autoimmune disorders
Drug hypersensitivity
Cocaine (adulterated with levamisole) (Figure 179-9) (Chapter 239,
Cocaine for additional images and information)
DIAGNOSIS
Initially, determining the extent of visceral organ involvement is
more important than identifying the type of vasculitis, so that
organs at risk of damage are not jeopardized by delayed or inad-
equate treatment. It is critical to distinguish vasculitis occurring
as a primary autoimmune disorder from vasculitis secondary to
infection, drugs, malignancy, or connective tissue disease such as
SLE or RA.
5
CLINICAL FEATURES
Small-vessel vasculitis is characterized by necrotizing inammation
of small blood vessels, and may be identied by the nding of
palpable purpura. The lower extremities typically demonstrate
palpable purpura, varying in size from a few millimeters to sev-
eral centimeters (Figure 179-2, Figure 179-6, Figure 179-7,
and Figure 179-10). In its early stages leukocytoclastic vasculitis
may not be palpable.
The clinical features of HSP include nonthrombocytopenic palpable
purpura mainly on the lower extremities and buttocks (Figure
179-1, Figure 179-2, and Figure 179-3), GI symptoms, arthral-
gia, and nephritis.
TYPICAL DISTRIBUTION
Cutaneous vasculitis is found most commonly on the legs, but may
be seen on the hands and abdomen (Figure 179-3, Figure 179-8,
and Figure 179-10).
LABORATORY TESTING
Laboratory evaluation is geared to nding the antigenic source of the
immunologic reaction. Consider throat culture, antistreptolysin-O
titer, erythrocyte sedimentation rate, platelets, complete blood
count (CBC), serum creatinine, urinalysis, antinuclear antibody,
A
FIGURE 179-9 A. Cutaneous vasculitis of the ear caused by levami-
sole-adulterated cocaine. (Courtesy of Jonathan Karnes, MD.) B. Cuta-
neous vasculitis in a retiform (net-like) pattern caused by the use of
levamisole-adulterated cocaine. This is called retiform purpura.
(Courtesy of John M. Martin IV, MD.)
B
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serum protein electrophoresis, circulating immune complexes, hep-
atitis B surface antigen, hepatitis C antibody, cryoglobulins, and
rheumatoid factor. The erythrocyte sedimentation rate is almost
always elevated during active vasculitis. Immunouorescent studies
are best done within the rst 24 hours after a lesion forms. The most
common immunoreactants present in and around blood vessels are
IgM, C3, and brin. The presence of IgA in blood vessels of a child
with vasculitis suggests the diagnosis of HSP.
Basic laboratory analysis to assess the degree and types of organs
affected should include serum creatinine, creatinine kinase, liver
function studies, hepatitis serologies, urinalysis, and possibly chest
x-ray and ECG.
BIOPSY
The clinical presentation is so characteristic that a biopsy is gener-
ally unnecessary. In doubtful cases, a punch biopsy should be taken
from an early active (nonulcerated) lesion or, if necessary, from the
edge of an ulcer (Figure 179-4).
DIFFERENTIAL DIAGNOSIS
Pigmented purpuric dermatosis is a capillaritis characterized by
extravasation of erythrocytes in the skin with marked hemosiderin
deposition. It is not palpable. Schamberg disease is a type of pig-
mented purpuric dermatosis found most often on the lower legs in
older persons (Figure 179-11 and Figure 179-12). It is described
as a cayenne pepper-like appearance. Lichen aureus is a localized
pigmented purpuric dermatosis seen in younger persons that may
occur on the leg or in other parts of the body (Figure 179-13).
The color may be yellow brown or golden brown. There is also a
pigmented purpuric dermatosis of the Majocchi type that has an
annular appearance with prominent elevated erythematous borders
that may have telangiectasias (Figure 179-14). A dermatoscope can
help to visualize the red or pink dots that represent inamed capil-
laries in these conditions.
Meningococcemia that presents with purpura in severely ill patients
with central nervous system symptoms (Figure 179-15 and
Figure 179-16).
Rocky Mountain spotted fever is a rickettsial infection that presents
with pink to bright red, discrete 1- to 5-mm macules that blanch
with pressure and may be pruritic. The lesions start distally and
spread to the soles and palms (Figure 179-17).
Malignancies, such as cutaneous T-cell lymphoma (mycosis fungoi-
des) (Chapter 176, Mycosis Fungoides).
Stevens-Johnson syndrome and toxic epidermal necrolysis (Chapter 177,
Erythema Multiforme, Stevens-Johnson Syndrome, and Toxic
Epidermal Necrolysis).
Idiopathic thrombocytopenia purpura can be easily distinguished
from vasculitis by measuring the platelet count. Also, the purpura
is usually not palpable and the petechiae can be scattered all over
the body (Figure 179-18).
Wegener granulomatosis is an unusual multisystem disease charac-
terized by necrotizing granulomatous inammation and vasculitis of
the respiratory tract, kidneys, and skin.
FIGURE 179-11 Schamberg disease (pigmented purpuric dermatosis)
of the lower leg showing hemosiderin deposits and a cayenne pepper
capillaritis. (Courtesy of Richard P. Usatine, MD.)
A
FIGURE 179-10 Leukocytoclastic vasculitis in a 26-year-old man.
A. Palpable purpura on the lower leg. B. Involvement of the lower
abdomen. (Courtesy of Richard P. Usatine, MD.)
B
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FIGURE 179-12 Schamberg disease with prominent petechiae and
hemosiderin deposits. Note that this condition is not palpable.
(Courtesy of Richard P. Usatine, MD.)
FIGURE 179-14 Pigmented purpuric dermatosis of the Majocchi type.
Note the annular appearance and the prominent elevated erythema-
tous borders. (Courtesy of Suraj Reddy, MD.)
A
FIGURE 179-13 Lichen aureus. A. On the leg of a 27-year-old woman. B. On the leg of a 16-year-old girl. (Courtesy of
Richard P. Usatine, MD.)
B
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FIGURE 179-16 Petechiae, purpura, and acrocyanosis in a severely ill
patient with meningococcemia. (Courtesy of the University of Texas
Health Sciences Center Division of Dermatology.)
FIGURE 179-17 Rocky Mountain spotted fever with many petechiae
visible around the original tick bite. This rickettsial disease looks similar
to vasculitis. (Courtesy of Tom Moore, MD.)
FIGURE 179-15 Petechiae of meningococcemia on the trunk of a
hospitalized adolescent. (Courtesy of Tom Moore, MD.)
Churg-Strauss syndrome (allergic granulomatosis) that presents
with a systemic vasculitis associated with asthma, transient pulmo-
nary inltrates, and hypereosinophilia.
Cutaneous manifestations of cholesterol embolism, which are leg
pain, livedo reticularis (blue-red mottling of the skin in a net-like
pattern), and/or blue toes in the presence of good peripheral pulses.
MANAGEMENT
NONPHARMACOLOGIC
The offending antigen should be identied and removed whenever
possible. With a mild hypersensitivity vasculitis is due to a drug,
discontinuing the offending drug may be all the treatment that is
necessary.
SOR
C
MEDICATIONS
An antihistamine might be used for itching.
SOR
C
Oral prednisone is used to treat visceral involvement and more severe
cases of vasculitis of the skin. Short courses of prednisone (60 to
80 mg/day) are effective and should be tapered slowly.
6,7

SOR
B
Colchicine (0.6 mg twice daily for 7 to 10 days) and dapsone (100 to
150 mg/day) may be used to inhibit neutrophil chemotaxis.
SOR
B
They are tapered and discontinued when lesions resolve. Azathioprine,
cyclophosphamide, and methotrexate have also been studied.
SOR
C
In HSP and prolonged hypersensitivity vasculitis, treatment with
nonsteroidal antiinammatory drugs is usually preferred. Treatment
with corticosteroids may be of more benet in patients with more
severe disease such as more pronounced abdominal pain and renal
involvement.
8

SOR
B Adding cyclophosphamide to the steroids
may also be effective.
SOR
C Azathioprine also may be used.
9
REFER OR HOSPITALIZE
Refer or hospitalize with signicant internal organ involvement or
prolonged disease course.
PROGNOSIS
In leukocytoclastic (hypersensitivity) vasculitis, the cutaneous
lesions usually resolve without sequelae. Visceral involvement (such
as kidney and lung) most commonly occurs in HSP, cryoglobuline-
mia and vasculitis associated with SLE.
10
Extensive internal organ
involvement should prompt an investigation for coexistent
medium-size vessel disease and referral to a rheumatologist.
FOLLOW-UP
Relapses may occur, especially when the precipitating factor is an
autoimmune disease. Regular monitoring is necessary.
PATIENT EDUCATION
Reassure patients and parents that most cases of acute cutaneous
vasculitis resolves spontaneously.
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PATIENT RESOURCES
MedicineNet. Vasculitis (Arteritis, Angiitis)http://www.
medicinenet.com/vasculitis/article.htm.
National Kidney and Urologic Diseases Information Clearing-
house. Henoch-Schnlein Purpurahttp://kidney.niddk.nih.
gov/kudiseases/pubs/HSP/.
National Heart Blood and Lung Institute. What Is Vasculitis?
http://www.nhlbi.nih.gov/health/dci/Diseases/vas/
vas_whatis.html.
PROVIDER RESOURCES
Roane DW, Griger DR. An approach to diagnosis and initial
management of systemic vasculitis. Am Fam Physician
1999:60:1421-1430http://www.aafp.org/afp/
991001ap/1421.html.
Sharma P, Sharma S, Baltaro R, Hurley J. Systemic vasculitis.
Am Family Physician 2011;83(5):556-565http://www.aafp.
org/afp/2011/0301/p556.html.
FIGURE 179-18 Petechiae and purpura in a patient with idiopathic
thrombocytopenic purpura and a platelet count of 3000. Note that this
purpura is not palpable. (Courtesy of Richard P. Usatine, MD.)