Kawasaki disease

What is Kawasaki disease?

Kawasaki disease is an acute febrile illness with inflammation of small- and
medium-sized blood vessels throughout the body, in particular, the coronary arteries
(blood vessels around the heart).
Kawasaki disease was previously known as Mucocutaneous Lymph Node
Syndrome. It was first described in Japan in 1967 by Dr Tomisaku Kawasaki, a
paediatrician.
Without treatment Kawasaki disease is usually self-limiting illness and resolves
spontaneously within 4-8 weeks. However, about 20% of untreated cases develop
coronary artery damage and approximately 2% of patients will die, most commonly
from a heart attack. This outlook is improved significantly with appropriate
treatment.
The hallmarks of KD are fever of unknown origin for more than five days,
generalized erythema and desquamation of skin, cervical nonsuppurative
lymphadenopathy, and swelling of the hands and the feet.
Who gets Kawasaki disease?
Eighty percent of cases occur in children younger than 5 years of age with a peak
incidence between 1 to 2 years. The disease is very uncommon in those over 14
years old and in adults. Overall it occurs more commonly in boys than girls.
Although cases of Kawasaki disease have been reported in children of all ethnic
origins, the highest incidence is in children of Asian descent especially Japanese.
There are 5000-6000 cases each year in Japan.
What causes Kawasaki disease?
The cause of Kawasaki disease is unknown. Evidence suggests an abnormal
inflammatory response triggered by a neoantigen or a conventional antigen from
one or more etiologic agents. Several infectious causes of KD have been the orized;
these include Epstein-Barr virus; retroviruses; Streptococcus pyogenes;
Streptococcus viridans; Staphylococcus species; Chlamydia infections;

Propionibacterium, and Pseudomonas species. However, conventional bacterial and

viral cultures and serologic studies have not confirmed an infectious cause. Other
postulated etiologic agents are immunization; medications; and environmental
agents, such as house dust mites.
Clinical Features: The vast majority of cases have occurred in children between
three months and 12 years of age, although it has also been reported in adults. KD
occurs more often in boys than in girls, with a ratio of about 1.4 : 1. The most
frequent symptoms of the disease are:
Fever for five days or more, with no response to antibiotics.
Bilateral congestion of ocular conjunctiva.
Changes in the extremities including indurative edema,erythema of palms and
soles and membranous desquamation of fingers and toes.( Red, swollen and
indurated palms)
Changes in lips and mouth including dryness, redness and fissuring of lips,
strawberry-like reddening and swelling of tongue papillae and diffuse
reddening of oral and pharyngeal mucosa, sometimes with gingival ulceration.
Polymorphous exanthema of torso without vesicles or crusts.
Acute, nonpurulent, unilateral swelling of cervical lymph nodes of 1.5 cm or
more.
Other less common findings include diarrhea, arthralgia, proteinuria,
leukocytosis, inc reased sedimentation rate and positive C-reactive protein.
Deterioration of the mood ( extremely misery)
One of the unfortunately common complications of the disease is cardiac
abnormality. While the vast majority of cases are self-limiting and nonfatal,
occasional deaths do occur, almost invariably a result of the cardiac complications,
usually a coronary thrombosis or vascular damage related to infantile periarteritis
nodosa.

How is Kawasaki disease diagnosed?

There is no specific lab test that establishes the diagnosis of Kawasaki disease. definitively. The
diagnosis is considered established when the following diagnostic criteria are met:

 Fever for at least 5 days AND

At least 4 of the 5 cardinal signs listed bellow:
1. Rash the rash of Kawasaki disease may be morbilliform (measles-like), maculopapular
(red patches and bumps), erythematous (red skin) or target-like and may be persistent
over days or evanescent. Skin peeling may occur in the convalescent stage of the illness.
2. Oral signs the typical changes include redness within the mouth or on the pharynx,
strawberry tongue, and red or cracked lips.
3. Eye signs redness of the bulbar conjunctivae (whites of the eyes) without exudate or
stickiness.
4. Peripheral limb signs including firm swelling of the hands and feet, sometimes
including the fingers and toes, with redness of the palms and soles. Periungual
desquamation (peeling of skin around the fingernails) may occur during the convalescent
stage of the illness.
5. Lymphadenopathy swollen lymph glands can occur, often on one side of the neck. One
lymph gland of at least 1.5cm in length is considered diagnostically enlarged.
The absence of any other illness to account for the signs and symptoms.

Differential Diagnosis. Unfortunately, there are no laboratory tests available for

confirmation of the diagnosis of the disease. Therefore, its diagnosis is based
entirely on clinical manifestations. It must be carefully distinguished from scarlet
fever, erythema multiforme or Stevens-Johnson syndrome.
Histologic Findings. Sparse perivascular lymphocytic and histiocytic inflammatory
infiltrates are seen. Marked papillary dermal edema, dilatation of blood vessels, and
exocytosis of lymphocytes are observed. Evidence of vasculitis is most severe in
medium-sized arteries.
Treatment: The diagnosis usually depends on the clinical and, particularly,
electrocardiographic findings. There is no specifi c treatment, but aspirin, despite
the possible risk of Reyes syndrome, is frequently given to lessen vascular damage
and thromboses. However, a single large dose of intravenous gammaglobulin, given
with aspirin and sometimes corticosteroids, in the early acute stages appears to be
the most effective means of reducing the risk of coronary artery damage. Coronary
artery aneurysms respond well to thrombolytic therapy.

Reiter syndrome
What is Reiter syndrome?

Reiter syndrome is a collection of symptoms but generally has three main features:
arthritis, genitourinary tract symptoms and conjunctivitis. Lesions of the skin and
mucous membranes also develop in some patients.

Some or all of these symptoms usually occur 1-3 weeks after an infection of the
genitourinary tract or bacterial gastrointestinal infection causing diarrhoea. The
arthritis is sometimes referred to as reactive arthritis, which means that the arthritis
occurs as a reaction to an infection that started somewhere else in the body.

Reiter syndrome generally lasts several months. The symptoms are the same
regardless of the origin of the triggering infection.

What causes Reiter syndrome?

The exact cause of Reiter syndrome remains unknown but it has been identified that
people with a particular genetic type called HLA-B27 have an increased chance of
developing the syndrome (about 80% of people with Reiter syndrome carry this
gene). Having this gene does not mean you will develop Reiter syndrome but it may
predispose you to it if you have certain infections.

Genitourinary Reiter syndrome is often a reaction to urethral infection that has been
passed from one person to another by sexual intercourse. The infection is most
commonly Chlamydia.

Gastrointestinal or enteric Reiter syndrome may develop after acute bacterial

diarrhoea caused by eating food contaminated with bacteria such as salmonella,
shigella or campylobacter.
Clinical Features: Reiters syndrome is more prevalent in
young adult men, usually between 20 and 30 years of age. The
male to female ratio is 9:1. There is a typical tetrad of manifestations: nongonococcal urethritis, arthritis, conjunctivitis, and
mucocutaneous lesions. In any given case, however, the full
tetrad is often not present.
Urethritis may be the first sign. The urethral discharge
is usually associated with itching and burning sensation.
The arthritis is often bilaterally symmetrical and usually
polyarticular. Conjunctivitis is often so mild as to be
overlooked. The skin lesions are similar to those seen in
keratoderma blennorrhagica and consist of red or yellow
keratotic macules or papules which eventually desquamate. A
possible relationship between Reiters syndrome and psoriasis
has been discussed by Perry and Mayne.
Oral Manifestations. Oral lesions occur in reported series
of cases in less than 5 to about 50% of patients with the
disease. The lesions, described by Pindborg and associates,
appear as painless, red, slightly elevated areas, sometimes
granular or even vesicular, with a white circinate border on
the buccal mucosa, lips, and gingiva. They may be mistaken

for recurrent aphthous ulcers. The palatal lesions appear as

small, bright red purpuric spots which darken and coalesce,
while the lesions on the tongue closely resemble geographic
tongue. Clinically, similar lesions occur on the glans penis,
producing a circinate balanitis. The temporomandibular joints are probably not
involved. Oral
manifestations are uncommon and consist of scalloped or circinate
white lines somewhat resembling one type of migratory
glossitis but involving any part of the mouth. In other cases
there may be shallow erosions. Lesions are typically painless
and frequently unnoticed
Histologic Features. The microscopic findings are not diagnostic. They consist of parakeratosis, acanthosis and polymorphonuclear leukocyte infiltration of epithelium, sometimes
with microabscess formation similar to psoriasis. The connective tissue shows a lymphocyte and plasma cell infiltrate.
Laboratory Findings. The patients usually have a mild
leukocytosis, an elevated sedimentation rate, and pyuria.
Treatment and Prognosis. The disease may undergo
spontaneous remission but has been treated by antibiotics and
corticosteroids.