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A CASE OF RASH…A HARBINGER OF
MORE SERIOUS ILLNESS?
CASSIE HAJEK, MD
SANFORD ADULT MEDICINE
S E P T E M B E R 1 3 TH, 2 0 1 3
32-YEAR-OLD WOMAN WITH RASH
• CC: Rash
• 32-year-old woman who presented with a rash that started 5 days earlier.
• She noted a tender bump on the right lower leg and thought she had
bumped her leg. Over the next couple of days, she began to acquire more
lesions on the lower legs.
• 24-48 hours after the initial tender nodule on the leg, she started to notice
lesions on her shoulders and upper trunk. These had more of a sunburn kind of
quality to them.
• She was out on the deck a few nights before she developed the lesions on the
upper trunk, but did not recall getting any insect bites, and the lesions were
not itchy
• The rash progressed for 2 days, then stabilized
• The lesions on the lower legs remained tender, but this improved with
ibuprofen.
• 2 days after the onset of the rash, she was seen in acute care and started on
triamcinolone 0.5% cream which she applied to the lesions on the upper trunk
with some relief
• She was recently exposed to mycoplasma, but denies any other exposures.
ROS
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Constitutional: No unexpected change in weight, no fatigue, no fevers, sweats or chills.
HEENT: Eye: No recent significant change in vision, no eye pain, redness, or discharge. Nose: She had a
mild runny nose and cold-type symptoms approximately 6 weeks earlier Ear: No ear pain, no tinnitus or
vertigo, no recent change in hearing. Mouth/Throat: No sore throat, no difficulty swallowing, no recent
change in voice or hoarseness. Neck: No lumps or masses, no swollen glands, no recent swelling in
thyroid area, no significant pain in neck.
Pulmonary: No chronic cough, sputum, or hemoptysis, no dyspnea on exertion, no wheezing, no
shortness of breath
Cardiovascular: No orthopnea, no chest pain, no diaphoresis, no edema, no palpitations, no
claudication symptoms.
Gastrointestinal: chronic on/off diarrhea - seems to be correlated with stress and nervousness, no
abdominal pain, no melena, no hematochezia, no significant change in appetite, no nausea or
vomiting
Musculoskeletal/Extremities: mild generalized arthralgias, no noted joint swelling or redness.
Heme/Allergy/Immune: No abnormal bleeding, no bruising, no night sweats, no history of DVT.
Skin/Integumentary: as mentioned in HPI
Neurologic: No chronic headaches, no seizures, no weakness, no numbness or tingling.
Psychiatric: No depression, no anxiety, no psychosis.
Endocrine: Negative for polydipsia, dry mouth, polyuria, and heat or cold intolerance.
GU: Denies abnormal vaginal bleeding, discharge or unusual pelvic pain, no dysuria, frequency or
hematuria
ADDITIONAL HISTORY
Past Medical History: negative
Past Surgical History: negative
Medications: Nuvaring –discontinued one week prior to presentation
Allergies: NKDA
Family Medical History:
• Mother – gastric adenocarcinoma
• There is no known family history of any other cancer, connective tissue disease,
inflammatory bowel disease
Social History
• Married
• Works as a nurse in the intensive care unit
• Occasional alcohol use and no tobacco or illicit drug use
PHYSICAL EXAM
• Vital signs: T = 97.8, P = 76, R = 14, BP = 104/72
• General: well-appearing woman in no acute distress
• HEENT: Pupils are round and react to light. There is no eyelid pallor. Tympanic
membranes are clear. Pharynx is moist and non-erythematous. Neck: Trachea is
midline. There is no thyromegaly.
• Lymph: There is no cervical, supraclavicular, groin or axillary adenopathy
• Heart: Regular rate, no obvious murmur. PMI is not displaced.
• Lungs: Lungs are clear to auscultation and percussion.
• Abdomen: Abdomen is soft. Positive bowel sounds. No bruits, no masses.
• Extremities: Strong pulses without edema.
• Neurologic: Range of motion in the neck is appropriate. Peripheral sensation
appropriate to light touch.
• Musculoskeletal: Mild tenderness to palpation in bilateral elbows, no joint swelling or
erythema; other joints WNL
• Skin:
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Upper arms, shoulders, upper back and chest had several pink, pseudo-vesicular lesions with
some that were slightly lighter centrally with erythema at the periphery but no targetoid
lesions
Bilateral lower extremities: numerous dull, erythematous, tender, indurated papules and
nodules. No ulceration is appreciated. No surrounding induration or impressive erythema
RASH
DIAGNOSTIC TESTING
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CBC: WNL
CMP: WNL
ESR: WNL
CRP: WNL
Rapid strep with reflex culture: negative
ASO titer: negative
ANA screen: negative
HIV: negative
Mycoplasma titers: negative
Peripheral smear: normal
UA: negative
BIOPSY RESULTS
• Leg:
• Dermatitis with minimal spongiosis, focal lichenoid
change and vacuolar cell change with
extravasated red blood cells
• Biopsy lacked subcutaneous tissue component to
evaluate for erythema nodosum.
• Back:
• Neutrophilic dermatitis
• Consistent with Sweet’s syndrome
SWEET’S SYNDROME
DEFINITION
• Acute febrile neutrophilic dermatosis
• Described by Robert Sweet in 1964
• Characterized by erythematous plaques,
nodules, or papules accompanied by fever,
malaise, or arthralgias
• Biopsy contains diffuse infiltrate of
neutrophils in the papillary dermis
• Female predominance
SWEET’S SYNDROME
THREE SUBTYPES
• Classic (idiopathic)
• Sudden onset of the typical skin findings and histopathology without
associated vasculitis
• Associated symptoms may include fever, preceding infection,
arthralgia, or conjunctivitis
• Lab findings can include leukocytosis or elevated ESR
• Malignancy-associated
• Acute myelogenous leukemia – most common hematologic
malignancy
• Genitourinary tumors – most common solid tumor
• Suspected with consistent constitutional symptoms or family history
• Drug-induced
• Temporal relationship between drug ingestion and onset of symptoms
• Granulocyte-colony stimulating factor and trimethoprimsulfamethoxazole
SWEET’S SYNDROME
DIAGNOSTIC CRITERIA
Major Criteria
1. Abrupt onset of tender or painful erythematous or violaceous
plaques or nodules
2. Predominantly neutrophilic infiltration in dermis without
leukocytoclastic vasculitis
Minor Criteria
1. Preceding fever or infection
2. Association with malignant lesion, pregnancy, inflammatory
bowel disease, upper respiratory or gastrointestinal infection
3. Good response to systemically administered corticosteroids
and not to antibiotics
4. Abnormal lab values: elevated ESR, leukocytosis
Presence of 2 Major and 2 Minor criteria establish the diagnosis
SWEET’S SYNDROME
TREATMENT AND PROGNOSIS
• Classical
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Systemic corticosteroids for diffuses disease
Intralesional/topical corticosteroids for limited disease
Improvement begins within 48 hours of treatment
Resolution in 1-2 weeks
May require taper over several weeks
Drug-induced – discontinue offending agent
Malignancy-associated – treat underlying malignancy
Spontaneous resolution possible in weeks to months
Skin lesions generally resolve without scarring
Recurrence occurs in ~30% of patients
BACK TO OUR PATIENT…
• CT Chest/Abdomen/Pelvis: Inflammatory bowel
changes involving the terminal ileum with two areas
of stricture present, mild diffuse fatty infiltration of
the liver with no large lymph nodes seen, minimal
mesenteric fat stranding present
• Colonoscopy with ileal biopsy: ileocolonic mucosa
demonstrated architectural distortion, mixed
lymphoplasmacytic inflammation with ulceration,
and acute inflammation that was felt consistent
with idiopathic inflammatory bowel disease
• EGD: negative
FINAL DIAGNOSIS AND OUTCOME
• Sweet’s Syndrome secondary to underlying Crohn’s
disease
• Patient initially refused systemic steroids as she did
not want them to affect her colon biopsy
• After colon biopsy, she was started on budesonide
and azathioprine with significant improvement of
her chronic diarrhea
• Skin manifestations gradually resolving
ANY QUESTIONS?
REFERENCES
• Cohen P. Sweet’s Syndrome – a comprehensive
review of an acute febrile neutrophilic dermatitis.
Orphanet Journal of Rare Disease. 2007; 2:34
• Rochet N, Chavan R, Cappel M, Wada D, Gibson L.
Sweet syndrome: Clinical presentation, associations
and response to treatment in 77 patients. J Am
Acad Derm. 2013; 10:1-8
SWEET’S SYNDROME
EXTRACUTANEOUS MANIFESTATIONS
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Bone - Acute sterile arthritis, arthralgias, focal aseptic osteitis, pigmented villonodular synovitis, sterile osteomyelitis
Central nervous system - Acute benign encephalitis, aseptic meningitis, brain SPECT abnormalities, brain stem lesions,
cerebrospinal fluid abnormalities, computerized axial tomography abnormalities, electroencephalogram abnormalities,
encephalitis, Guillain- Barre syndrome, idiopathic hypertrophic cranial pachymeningitis, idiopathic progressive bilateral
sensorineural hearing loss, magnetic resonance imaging abnormalities, neurologic symptoms, "neuro-Sweet disease", pareses
of central origin, polyneuropathy, psychiatric symptoms
Ears - Tender red nodules and pustules that coalesced to form plaques in the external auditory canal and the tympanic
membrane
Eyes - Blepharitis, conjunctival erythematous lesions with tissue biopsy showing neutrophilic inflammation, conjunctival
hemorrhage, conjunctivitis, dacryoadenitis, episcleritis, glaucoma, iridocyclitis, iritis, limbal nodules, ocular congestion,
periocular swelling, peripheral ulcerative keratitis, retinal vasculitis, scleritis, uveitis
Kidneys - Mesangiocapillary glomerulonephritis, urinalysis abnormalities (hematuria and proteinuria)
Intestines - Intestine with extensive and diffuse neutrophilic inflammation, neutrophilic ileal infiltrate, pancolitis (culturenegative)
Liver - Hepatic portal triad with neutrophilic inflammation, hepatic serum enzyme abnormalities, hepatomegaly
Heart - Aortic stenosis (segmental), aortitis (neutrophilic and segmental), cardiomegaly, coronary artery occlusion, heart
failure, myocardial infiltration by neutrophils, vascular (aorta, bracheocephalic trunk and coronary arteries) dilatation
Lung - Bronchi (main stem) with red-bordered pustules, bronchi with neutrophilic inflammation, pleural effusion showing
abundant neutrophils without microorganisms, progressive pharyngeal mucosal infiltration and edema resulting in upperairway obstruction, and chest roentgenogram abnormalities: corticosteroid-responsive culture-negative infiltratives,
pulmonary tissue with neutrophilic inflammation
Mouth - Aphthous-like superficial lesions (buccal mucosa, tongue), bullae and vesicles (hemorrhagic: labial and gingival
mucosa), gingival hyperplasia, necrotizing ulcerative periodontitis, nodules (necrotic: labial mucosa), papules (macerated:
palate and tongue), pustules (individual and grouped: palate and pharynx), swelling (tongue), ulcers (buccal mucosa and
palate)
Muscles- Magnetic resonance imaging (T1-weighted and T2-weighted) abnormalities: high signal intensities due to myositis
and fasciitis, myalgias (in up to half of the patients with idiopathic Sweet's syndrome), myositis (neutrophilic), tendinitis,
tenosynovitis
Spleen – Splenomegaly