Page 90 - The Netter Collection of Medical Illustrations - Integumentary System_ Volume 4 ( PDFDrive )

Plate 4-5 Integumentary System

Sweet’s syndrome. Edematous papules and plaques, often
associated with an underlying infection or systemic illness

Diagnostic Criteria for Sweet’s Syndrome*
Major criteria
ACUTE FEBRILE
NEUTROPHILIC DERMATOSIS Abrupt onset of rash—various morphologies
(SWEET’S SYNDROME) Histological evaluation shows diffuse neutro-
philic infiltrate with papillary edema
Minor critieria
Acute febrile neutrophilic dermatosis is an uncommon
rash that most often is secondary to an underlying Preceding infection or pregnancy or malignancy
infection or malignancy. The diagnosis is made by ful- Fever 38 C
filling a constellation of criteria. Both clinical findings Sedimentation rate 20 or elevated C-reactive
and pathology results are required to make the diagno- protein level or leukocytosis with left shift
sis in a patient with a consistent history. Rapid resolution with systemic steroids
Clinical Findings: Acute febrile neutrophilic derma-
tosis is often associated with a preceding infection. The *For the diagnosis, both major criteria and one minor
infection can be located anywhere but most commonly criterion must be present. Adapted from Odom RB,
is in the upper respiratory system. Females appear to James WD, Berger T. Andrews’ Diseases of the Skin:
be more likely to be afflicted, and there is no race pre- Clinical Dermatology. 10th ed. Philadelphia: Saunders,
dilection. Patients present with fever and the rapid 2006.
onset of juicy papules and plaques. Because the papules
can look as if they are fluid filled, they are given the
descriptive term juicy papules. They can occur anywhere
on the body and can be mistaken for a varicella infec-
tion. Patients also have neutrophilia and possibly arthri-
tis and arthralgias. If this condition is associated with a
preceding infection, it is usually self-limited and heals
without scarring, unless the papules and plaques are
excoriated or ulcerated by scratching. Variable amounts
of pruritus and pain are associated with this skin disease.
When one is evaluating a patient with this condition,
a thorough history is required. A skin biopsy must be
performed. A chest radiograph, throat culture, and uri-
nalysis should be performed to assess for the possibility
of bacterial infection.
Lymphoproliferative malignancies have also been
seen in association with Sweet’s syndrome. The malig-
nancy often precedes the rash, and the skin disease is
believed to be a reaction to the underlying malignancy.
It is important to obtain specimens from these patients
for histological evaluation and culture for aerobic,
anaerobic, mycobacterial, and fungal organisms. The
main differential diagnosis is between an infection and
Sweet’s syndrome in cases associated with a malignancy.
The most common malignancy associated with acute
febrile neutrophilic dermatosis is acute myelogenous
leukemia. The prognosis in these cases is directly
related to the underlying malignancy. Often, the skin
disease continues to recur unless the malignancy is put
into remission.
A few medications have also been shown to induce
Sweet’s syndrome, including granulocyte colony- Sweet’s syndrome on the dorsal hand.
stimulating factor (G-CSF), lithium, all-trans-retinoic This can be difficult to differentiate
acid, minocycline, and oral contraceptives. Diffuse neutrophilic infiltrate throughout the dermis from pyoderma gangrenosum.
Pathogenesis: The pathomechanism of Sweet’s syn-
drome is theorized to involve the secretion of a neutro-
philic chemoattractant factor, which causes massive
amounts of neutrophils to migrate into the skin. The of neutrophils. Varying amounts of leukocytoclasis are postinfectious Sweet’s syndrome. Topical and oral ste-
exact molecule responsible for the recruitment of neu- present. Subepidermal bulla formation is possible roids can dramatically shorten the course of the disease.
trophils into the skin is unknown. Reports of exogenous because of the extensive dermal edema. Special stains Sweet’s syndrome that develops as a paraneoplastic
use of G-CSF have led to the theory that it is respon- for microorganisms must be negative to exclude an process secondary to underlying leukemia should be
sible for the chemoattraction of neutrophils. Other infectious process, and these must be backed up with treated with oral or intravenous steroids once an infec-
chemoattractants are possible players in the pathogen- cultures to help disprove an infection, because the his- tious process has been ruled out. This can result in a
esis, including interleukin-8. tological picture can mimic an infectious process. rapid response, but it is short lived once the steroids are
Histology: Histological examination shows massive Treatment: Treatment should be directed at the caus- removed. True remission occurs only if the cancer is
dermal edema with a dense infiltrate composed entirely ative agent. Supportive care is needed for those with treated and put into remission.

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