A case of rash a harbinger of more serious illness
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A case of rash…a harbinger of more serious illness?. Cassie Hajek, MD Sanford Adult Medicine September 13 th , 2013. 32-year-old woman with rash. CC: Rash 32-year-old woman who presented with a rash that started 5 days earlier.

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A case of rash…a harbinger of more serious illness?

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A case of rash a harbinger of more serious illness

A case of rash…a harbinger of more serious illness?

Cassie Hajek, MD

Sanford Adult Medicine

September 13th, 2013


32 year old woman with rash

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.


A case of rash a harbinger of more serious illness

ROS

  • 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

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

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:

    • Upper arms, shoulders, upper back and chest had several pink, pseudo-vesicular lesionswith some that were slightly lighter centrally with erythema at the periphery but no targetoid lesions

    • Bilateral lower extremities: numerous dull, erythematous, tender, indurated papulesand nodules. No ulceration is appreciated. No surrounding induration or impressive erythema


A case of rash a harbinger of more serious illness

Rash


Diagnostic testing

Diagnostic testing

  • 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

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

Sweet’s SyndromeDefinition

  • 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

Sweet’s SyndromeThree 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 trimethoprim-sulfamethoxazole


Sweet s syndrome diagnostic criteria

Sweet’s SyndromeDiagnostic Criteria

Major Criteria

  • Abrupt onset of tender or painful erythematous or violaceous plaques or nodules

  • Predominantly neutrophilic infiltration in dermis without leukocytoclastic vasculitis

    Minor Criteria

  • Preceding fever or infection

  • Association with malignant lesion, pregnancy, inflammatory bowel disease, upper respiratory or gastrointestinal infection

  • Good response to systemically administered corticosteroids and not to antibiotics

  • Abnormal lab values: elevated ESR, leukocytosis

Presence of 2 Major and 2 Minor criteria establish the diagnosis


Sweet s syndrome treatment and prognosis

Sweet’s SyndromeTreatment and Prognosis

  • Classical

    • 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

Back to our Patient…

  • CT Chest/Abdomen/Pelvis:Inflammatory bowel changes involving the terminal ileumwith 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

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

Any Questions?


References

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

Sweet’s SyndromeExtracutaneous Manifestations

  • 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 (culture-negative)

  • 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 upper- airway obstruction, and chest roentgenogram abnormalities: corticosteroid-responsive culture-negative infiltratives, pulmonary tissue with neutrophilicinflammation

  • 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


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