Page 194 - The Netter Collection of Medical Illustrations - Integumentary System_ Volume 4 ( PDFDrive )

Plate 6-19 Integumentary System

ACUTE ADRENAL INSUFFICIENCY (WATERHOUSE-FRIDERICHSEN SYNDROME)

Circulatory collapse,
marked hypotension

MENINGOCOCCEMIA
Meningococci from
blood, spinal fluid,
Meningococcemia can cause a wide range of clinical and/or throat
diseases, of which neisserial meningitis is the most
severe and life-threatening. The bacteria, Neisseria men-
ingitidis, is capable of causing septicemia, pneumonia,
and meningitis. These are all relentless diseases that
are universally fatal if not promptly treated. The bac-
teria has been known to cause severe disseminated
intravascular coagulation (DIC) and the Waterhouse-
Friderichsen syndrome. The latter syndrome, also
known as acute adrenocortical insufficiency, is directly
caused by hemorrhagic destruction of both adrenal
glands. This syndrome can result from a wide range of
conditions, including infections, and N. meningitidis is
one of the more frequent infectious causes.
Clinical Findings: Children younger than 1 year Extensive purpura, shock,
of age are those most likely to develop disease from prostration, cyanosis
N. meningitidis infection. Boys are more apt to develop
this infection than girls, and there is no race predilec-
tion. One risk factor appears to be the presence of a
smoker in the household. It is theorized that the sec-
ondhand smoke damages the child’s respiratory epithe- Hemorrhagic
lium just enough to allow the bacteria to penetrate the destruction of
mucous membranes and enter the bloodstream. Other adrenal gland
risk factors include a deficiency of the complement
components C5, C6, C7, and C8. Asplenia also increases
one’s risk, because the spleen is extremely important in
removing encapsulated bacteria from the bloodstream.
Chronic immunosuppression increases the risk, as Characteristic fever chart
does living in crowded conditions. This is why military
barracks and college dormitories are often sources 105
of outbreaks.
Patients who develop meningitis have fever, head-
ache, vomiting, stiff neck, and meningeal physical signs,
including Kernig’s sign and Brudzinski’s sign. Kernig’s
sign is positive when placing a patient’s hips and knees
in 90-degree flexion and extending the knee joint elicits Temperature (°F)
pain. Brudzinski’s sign is more sensitive for meningitis
and is positive when flexing of the patient’s neck causes 100
flexion of the hips and knees. These signs have long
been used to help diagnosis meningitis clinically. As the
disease progresses, seizures or coma may occur.
Cutaneous findings include palpable purpura, ecchy-
mosis, widespread macular purpura, and necrosis of the
skin with secondary vesiculopustules. The purpura can 1 2
be angulated with an irregular border. Centrally within Days
the purpuric region, there is often a dusky gray discol-
oration of the skin. Patients often complain of skin pain.
Necrosis may progress to cause gangrene of the digits
or distal extremities. In severe cases, entire limbs can
become gangrenous. If DIC sets in, the clinical skin syndrome is seen in fewer than 5% of patients with extravasates through the damaged endothelial walls
findings of DIC may be seen on top of the initial N. meningitidis septicemia, but it occurs in more than and produces massive purpura. The more extensive
skin findings. The presence of DIC is a poor prognostic 50% of the fatal cases. Patients present with skin find- the cutaneous purpura in meningococcal septicemia,
indicator. ings of widespread purpura and cyanosis. They have the higher the incidence of Waterhouse-Friderichsen
Fulminant meningococcal septicemia may lead to signs and symptoms of hemodynamic collapse, hypo- syndrome.
hemorrhagic necrosis of the adrenal glands; this is tension, acute renal failure, and a biphasic fever. The Laboratory testing can be used to diagnosis the
termed the Waterhouse-Friderichsen syndrome. It skin findings are caused by small-vessel embolization or disease, but one should not wait for the results to
leads ultimately to acute adrenal dysfunction. This endothelial destruction from the septicemia. Blood begin therapy if there is a high clinical suspicion of

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