Rash Cold/cough Then Cough Again Month Later

Clin Pract Cases Emerg Med. 2019 Feb; 3(ane): 1–5.

38-year-erstwhile Woman with a Cough and a Rash

Megan P. Donohue, Md, MPH,^* Elizabeth P. Clayborne, MD, MA,^† Zachary D.W. Dezman, Md, MS, MS, ^† and Laura J. Bontempo, MD, MEd^†

Megan P. Donohue

^*University of Maryland Medical Center, Department of Emergency Medicine, Baltimore, Maryland

Elizabeth P. Clayborne

^†Academy of Maryland School of Medicine, Department of Emergency Medicine, Baltimore, Maryland

Zachary D.W. Dezman

^†University of Maryland School of Medicine, Department of Emergency Medicine, Baltimore, Maryland

Laura J. Bontempo

^†Academy of Maryland School of Medicine, Department of Emergency Medicine, Baltimore, Maryland

Received 2018 Aug x; Revised 2018 Oct 11; Accepted 2018 Nov seven.

Instance PRESENTATION (Dr. Donohue)

A 38-year-old female presented to the emergency department (ED) with rash, dyspnea, odynophagia, and nasal congestion for the prior two weeks. During that time, she sought medical intendance twice. The first dr. to evaluate the patient started her on antibiotics for a presumed upper respiratory infection (URI). Her symptoms did not improve subsequently completing a ten-day course of amoxicillin; then a second medical provider prescribed her ciprofloxacin. She was on her eighth solar day of ciprofloxacin (i.e., total 18^thursday solar day of handling) when she presented to our ED with rash and dyspnea. She decided to come to the ED because her cough had worsened and become productive of sputum. She likewise complained of one month of fevers, chills, night sweats, and malaise. She denied whatever complaints of headaches, breast pain, palpitations, intestinal pain, genitourinary or neurologic symptoms.

Her past medical history was significant for adult-onset asthma and allergic rhinitis. Medications included fluticasone, ipratropium, and her contempo courses of amoxicillin and ciprofloxacin. She had no known medication allergies but reported gastrointestinal intolerance to fish oil. Her family history was pregnant for a sister with multiple sclerosis. She was an Iranian immigrant who had moved to Baltimore six months prior to presenting in our ED. She was married with no children and denied e'er using tobacco, alcohol or illicit drugs.

On physical examination, she was alert but appeared uncomfortable as she hobbled into triage that dark. She was afebrile (36.7° Celsius) and mildly tachycardic (eye rate of 110 beats per minute). Her blood pressure was 102/68 millimeters of mercury, she was mildly tachypneic with a respiratory rate of xx breaths per minute, and her oxygen saturation was 97% while breathing room air. She was well developed and well nourished, with an estimated body mass index of 22. Her head was normocephalic and atraumatic. Her oropharynx was articulate; her cervix was supple and no lymphadenopathy was detected. On auscultation, she was tachycardic with a normal S1 and S2, without whatever murmurs, gallops or rubs. She was mildly tachypneic without whatever accessory muscle use, retractions, or increased work of breathing. She was able to speak in full sentences without difficulty. The patient'due south lungs were clear to auscultation bilaterally without wheezes, rhonchi, or rales. Her abdomen was soft and not-tender, and no lower extremity edema was nowadays. She was alert, oriented and appropriately interactive.

On closer examination of the patient's peel, her rash appeared to have three unlike morphologies. The first was located on her forehead and consisted of sub-centimeter papulovesicular eruptions with petechiae that were pruritic but not tender (Paradigm i). The second rash consisted of scattered hemorrhagic vesicles with purpuric macules and was located on her distal upper and lower extremities (Image 2). The third rash was an erythematous and indurated plaque at the base of the left foot, which was tender and made it painful for her to walk.

Papulovesicular eruptions with petechiae on the forehead of the patient (arrow).

Scattered hemorrhagic vesicles (arrow) with purpuric macules on the patient's anxiety.

Initial laboratory results are shown in Tables 1 ​ –3. The patient's electrocardiogram showed sinus tachycardia with normal intervals and without ST-segment or T-wave abnormalities. Bilateral multilobar infiltrates were revealed on breast radiograph (Image iii). A computed tomography (CT) of her breast confirmed the presence of bilateral multilobar infiltrates and a CT of her sinuses showed mucosal thickening throughout. An echocardiogram revealed a normal ejection fraction and no valvular pathology. No vegetations were seen. The patient was admitted to the infirmary for farther evaluation. A test and then was performed, which confirmed the diagnosis.

Breast radiograph of a 38-year-old patient with rash and dyspnea, showing bilateral multi-lobar infiltrates (arrows).

Tabular array 1

Initial laboratory results of a 38-year-old female patient presenting with a rash and dyspnea.

	Values
Complete claret cell count
White blood cell count (K/μL)	12.ane
Hemoglobin (g/dL)	xi.5
Hematocrit (%)	33
Platelets (M/μL)	237
Differential
Granulocytes (%)	64.four
Lymphocytes (%)	15.3
Monocytes (%)	eight.3
Eosinophils (%)	10.half dozen
Serum chemistries
Sodium (mmol/L)	141
Potassium (mmol/50)	3.5
Chloride (mmol/L)	100
Bicarbonate (mmol/L)	26
Blood urea nitrogen (mg/dL)	6
Creatinine (mg/dL)	0.54
Total protein (g/dL)	eight.iv
Albumin (g/dL)	3.viii
Aspartate aminotransferase (u/Fifty)	26
Alanine aminotransferase (u/L)	21
Element of group i phosphatase (u/50)	58

Table ii

Additional laboratory results of 38-year-onetime patient presenting with a rash and dyspnea.

Additional labs	Values
Human immunodeficiency virus screen	Non-reactive
Erythrocyte sedimentation rate (mm/hr)	70
C-reactive protein (mg/L)	iii.3
Immunoglobulin Eastward (KU/L)	6,266

Tabular array iii

Urinalysis of 38-year-old patient presenting with a rash and dyspnea.

Urinalysis	Values
pH	seven
Specific gravity	1.003
Glucose	Negative
Ketones	Negative
Protein	Negative
Nitrile	Negative
Leukocyte esterase	Negative
White claret cells	None
Red blood cells	Trace

Example Give-and-take (Dr. Clayborne)

My arroyo to cases that incorporate a lot of non-specific information is to kickoff expect at the big picture. I like to place highlights from the history of present illness (HPI), past medical history, social history, review of symptoms (ROS) and physical examination to isolate what stands out almost and may requite insight into the differential. Start impressions of the HPI were the story of a young female with a history of asthma and allergic rhinitis, who recently had a URI that was unresponsive to two different antibiotics. That patient and so presented to the ED with a rash later on taking ciprofloxacin. First impressions of the ROS highlighted that she had one month of constitutional symptoms and fatigue, nasal congestion, sore throat, a cough that was productive without hemoptysis and a new rash with pruritis. Commencement impressions of social history included her status as an Iranian immigrant who did not drink, smoke or use drugs. Finally, first impressions from her physical examination included an afebrile, stable-appearing patient with tachycardia and tachypnea whose lungs were clear to auscultation bilaterally, who also had rashes of three unlike morphologies on her face, extremities, and the sole of ane pes.

Immediately after my offset impressions, the item that raised my business organisation the most was the rash. I notice that many emergency physicians can be uncomfortable with rashes since we often find them hard to properly describe, breaking the link between identifying a rash and making the diagnosis. In this example, the rash'due south location and characteristics were not specific to an etiology with which I was familiar. Only I was able to combine the clarification of the rash with my first impressions to generate a preliminary differential diagnosis.

I subdivided my differential diagnosis into the 3 broad areas: infectious (bacterial, viral, or fungal); allergic; and autoimmune. Based on these three categories I began to use the data collected in the ED to help narrow my focus.

Pertinent positives from the lab piece of work included a mild leukocytosis of 12.1 kilo/microliter with eosinophilia of 10.half dozen%, urinalysis with trace blood, erythrocyte sedimentation charge per unit of 70 millimeters per hour, a C-reactive protein of 3.three milligrams per liter, and her screen for caused human immunodeficiency virus was nonreactive. These are the labs that I would look to result during the patient'south ED visit. In the ED I would as well encounter her chest radiograph and CT chest showing bilateral multilobar infiltrates. With this information, if I were the treating doc, I would order antibiotics and acknowledge the patient for an inpatient workup.

The results that would more likely be done during the inpatient stay demonstrated sinusitis, a normal cardiac ejection fraction, and an elevated Immunoglobulin E (IgE) of 6,266 kilounits per liter. Based on this additional information, I went back to my wide differential diagnosis to come across what fit and did not fit each category. Allergic etiologies would include a rash, respiratory symptoms, and perhaps mild elevation of inflammatory markers merely do not account for the constitutional symptoms or positive findings on radiograph and CT. Autoimmune etiologies would account for the constitutional symptoms, rash, and infiltrates but I would question why but a few of the inflammatory markers were elevated. Infectious etiologies, peculiarly fungal infections, could account for the constitutional symptoms, pulmonary affliction, eosinophilia, and elevated IgE.

My top two diagnoses based on the patient'southward presentation coupled with her laboratory results are eosinophilic granulomatous polyangiitis (EGPA), also known as Churg-Strauss syndrome, and aspergillosis. Invasive pulmonary aspergillosis starts when the patient becomes colonized by inhaling fungal spores. The patient'due south symptoms are often ramble (weakness, fatigue, and depression-form fevers) and non-specific (shortness of breath and cough that is productive but not responsive to antibiotics). Patients often present with hemoptysis, which did not occur in this case. The infection can spread from the lower respiratory tree to multiple organs, most often the encephalon. These patients will then develop abnormalities on head CT (infarcts, band-enhancing lesions, hemorrhage, abscess) and begin to suffer from seizures.

Patients with aspergillosis will oft present with eosinophilia, elevated IgE levels, abnormal chest radiographs and CTs, sinusitis, and a feature rash. The rash begins as either solitary or multiple erythematous or violaceous, indurated papules or plaques. It can exist tender and evolve quickly into pustules, hemorrhagic vesicles, or eschars.1 Notwithstanding, this patient does non have any of the risk factors for aspergillosis (prolonged neutropenia, transplant [peculiarly lung], prolonged high-dose corticosteroid therapy, hematological malignancy, cytotoxic therapy, or advanced acquired immune deficiency syndrome. This patient has sinusitis and no hemoptysis, both of which are inconsistent with aspergillosis.

EGPA was first described in 1951 by Churg and Strauss. They described a syndrome in 13 patients who had asthma, eosinophilia, granulomatous inflammation, necrotizing systemic vasculitis, and necrotizing glomerulonephritis. In 1990 the American College of Rheumatology (ACR) proposed the following half dozen criteria for the diagnosis of EGPA:2

• Asthma (wheezing, expiratory rhonchi)

• Eosinophilia of more than 10% in peripheral claret

• Paranasal sinusitis

• Pulmonary infiltrates (may be transient)

• Histological proof of vasculitis with extravascular eosinophils

• Mononeuritis multiplex or polyneuropathy

This patient met iv of these criteria (asthma, eosinophilia, sinusitis and pulmonary infiltrates), which is consistent with a diagnosis of EGPA (sensitivity of 85%, specificity of 99.vii%).

If I were the treating physician, I would empirically treat with antifungals due to the concern for aspergillosis. For this case practise, nonetheless, I believe a punch biopsy would confirm a diagnosis of EGPA.

CASE Consequence (Dr. Donohue)

The diagnostic study performed was a punch biopsy of a hemorrhagic vesicle on the right foot. It revealed 2 key findings: 1) eosinophils surrounding a central granuloma; and two) the cross-department of a blood vessel with key necrosis. These findings confirmed the diagnosis of EGPA, also known as Churg-Strauss syndrome.

Our patient was admitted to an inpatient medical service, but the diagnostic punch biopsy was actually performed in the ED prior to access. While on the inpatient service, numerous consultants participated in her intendance including dermatology, rheumatology, pulmonology, and communicable diseases. She underwent bronchoscopy, broncho-alveolar lavage and lung biopsy. Ultimately, based on the ACR EGPA criteria, she was diagnosed with EGPA and started on high-dose prednisone 60 milligrams (mg) daily.2 On outpatient follow-upwardly, she was transitioned to azothioprine and at one year follow-upward was doing well.

RESIDENT Discussion

EGPA is a rare vasculitis with an estimated incidence of i to 3 cases per million people. Early on in the history of its recognition, reliable data on incidence were unavailable due to its similarities with other heterogeneous disease processes and lack of diagnostic criteria.3 EGPA was first described in 1951 by two young pathologists in New York City who were studying vasculitis. As pathologists, their case study information was obtained from autopsies in which they recognized "the occurrence of a clinical syndrome of astringent asthma, fever and hypereosinophilia."4 Keep in mind, this was in addition to the fact that these patients already had known vasculitis and were already deceased. However, information technology was "the finding of granulomatous lesions, both within vessel walls and in connective tissue" that made it singled-out from other allergic syndromes and vasculitides.5 Churg and Strauss theorized that 11 of the 13 sentry cases had died due to this syndrome to which they applied their eponym.

Since 1951 the underlying pathology and pathogenesis of EGPA has become better understood. As a result, the name was changed from Churg-Strauss syndrome to EGPA, which improve describes the underlying disease procedure and the phases of its clinical manifestations. EGPA has three singled-out clinical phases. The first, or prodromic, is characterized past onset of asthma in the 2d or third decade of life. It often is associated with allergic rhinitis and recurrent sinusitis. The second, eosinophilic, phase is marked by peripheral eosinophilia with organ infiltration. Peripheral eosinophilia may be masked by steroid therapy.5 Eosinophilic organ infiltration occurs almost commonly in the lungs, peripheral nerves and pare, but tin can occur in any organ system,half dozen creating an assortment of clinical presentations.vii This phase of the disease is difficult to distinguish from other hypereosinophilic conditions. The third phase of EGPA is vasculitic; this phase, which is unique to EGPA, makes it fatal if untreated.

Patients with pulmonary EGPA tin develop pulmonary infarcts, nodules or lengthened alveolar hemorrhage. The virtually common CT findings include bilateral ground-glass opacities, airspace consolidation, centrilobular nodules and bronchial wall thickening.8,9 Infiltration of the heart can cause myocardial infarction, pericarditis or congestive centre failure. Primal nervous system involvement may result in neuropathy, mononeuritis, seizure, stroke or blackout. One study establish that mononeuritis was the second about common presentation of EGPA, second only to asthma.viii In the gastrointestinal organization EPGA can cause cholecystitis, pancreatitis or gastroenteritis. Vasculitic involvement of the renal system may issue in proteinuria, hematuria, glomerulonephritis or renal insufficiency. Any of these complications may be a patient'south presenting symptoms; the case we highlighted is the most common presentation of EGPA. Organ organisation interest is important due to its prognostic value every bit calculated by the five-factor score. If two or more of the following organ systems are involved, cardiac, gastrointestinal, nervous system or kidneys, five-year mortality is 50% untreated.9

Diagnostic criteria have changed over fourth dimension. Churg and Strauss initially described a disease that merely was diagnosed on biopsy. It was felt that an accent on biopsy and pathological findings led to the illness beingness underdiagnosed.ten The ACR revised the diagnostic criteria in 1990 to include more mutual features: the presence of asthma; eosinophilia >10% on white blood cell count differential; mononeuropathy or polyneuropathy; non-fixed pulmonary infiltrates on imaging; parasinus aberration; or a characteristic biopsy. If iv or more criteria were present, sensitivity was 85% and specificity 99.7%. Alternatively, if a patient had asthma, eosinophilia and history of allergy or drug sensitivity, sensitivity was 95% and specificity 99.ii%.2 Now that biopsy is no longer needed to ostend the diagnosis, a provider's clinical index of suspicion portends significant impact in recognition and diagnosis of EGPA.

It has been suggested that anti-leukotriene medications may play a role in the development of EGPA, but this is controversial.eleven–14 The current understanding is that prolonged survival of eosinophils due to inhibition of CD95-mediated apoptosis plays a role in EGPA pathogenesis. Recent information suggest that cytokine release from T-lymphocyte may be an important step.15 Even though the verbal cause of EGPA has withal to be fully elucidated, handling guidelines are available. High-dose glucocorticoids (1mg/kg/day prednisone) for at least ii to three weeks is the cornerstone for handling to obtain remission. EGPA responds well to get-go-line treatments, just relapse has been shown to occur in upwards to 25% of patients.10 Cyclophosphamide is the main pharmacotherapy for remission induction. Azothioprine and methotrexate are used for maintenance therapy for patients with life- or organ-threatening illness involvement. Intravenous immunoglobulin is considered 2d-line treatment for refractory disease. New handling modalities currently being studied include plasma exchange and use of monoclonal antibodies.16 Treatment of EGPA should ever include a multidisciplinary team, including rheumatology.

FINAL DIAGNOSIS

Eosinophilic granulomatous polyangiitis, likewise known as Churg-Strauss syndrome

Central Pedagogy POINTS

1. EGPA is a rare only deadly vasculitis.

2. Typical features of EGPA are asthma with allergic rhinitis and recurrent sinusitis, peripheral eosinophilia and vasculitis.

3. High clinical suspicion is paramount equally it is a clinical diagnosis.

4. Differential diagnosis should exist broadened when a patient has bounced back and already failed initial medical therapy.

5. Diagnostic momentum in the ED tin can play a pivotal role in making the correct diagnosis.

Footnotes

Department Editor: Rick A. McPheeters, Practice

Full text available through open up access at http://escholarship.org/uc/uciem_cpcem

Documented patient informed consent and/or Institutional Review Board approval has been obtained and filed for publication of this case report.

Conflicts of Involvement: Past the CPC-EM article submission agreement, all authors are required to disclose all affiliations, funding sources and financial or management relationships that could be perceived every bit potential sources of bias. The authors disclosed none.

REFERENCES

1. Kosmidis C, Denning DW. Republished: the clinical spectrum of pulmonary aspergillosis. Postgrad Med J. 2015;91(1077):403–10. [PubMed] [Google Scholar]

ii. Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome. Arthritis Rheumatol. 1990;33(8):1094–100. [PubMed] [Google Scholar]

three. Watts RA, Carruthers DM, Scott DG. Epidemiology of systemic vasculitis: changing incidence or definition? Semin Arthritis Rheum. 1995;25(one):28–34. [PubMed] [Google Scholar]

4. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis and periarteritis nodosa. Am J Pathol. 1951;27(2):277–301. [PMC gratuitous article] [PubMed] [Google Scholar]

5. Churg A. Recent advances in the diagnosis of Churg-Struass syndrome. Modern Pathol. 2001;14(12):1284–93. [PubMed] [Google Scholar]

6. Chumbley LC, Harrison EG, Jr, De Remee RE. Allergic granulomatosis and angiitis (Churg-Strauss syndrome) Mayo Clin Proc. 1977;52(8):477–84. [PubMed] [Google Scholar]

seven. Churg A, Brallas M, Stephen R, et al. Formes frustes of Churg-Strauss syndrome. Chest. 1995;108(ii):320–3. [PubMed] [Google Scholar]

8. Guillevin L, Cohen P, Gayraud K, et al. Churg-Strauss syndrome: clinical study and long-term follow-upwards of 96 patients. Medicine (Baltimore) 1999;78(one):26–37. [PubMed] [Google Scholar]

9. Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. Medicine (Baltimore) 1996;75(1):17–28. [PubMed] [Google Scholar]

10. Lanham JG, Elkon KB, Pusey CD, et al. Systemic vasculitis with asthma and eosinophilia: A clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984;63(2):65–81. [PubMed] [Google Scholar]

eleven. Noth I, Strek ME, Leff AR. Churg-Strauss syndrome. Lancet. 2003;361(9357):587–94. [PubMed] [Google Scholar]

12. Bili A, Condemi JJ, Bottone SM, et al. Vii cases of complete and incomplete forms of Churg-Strauss syndrome not related to leukotriene receptor antagonists. J Allergy Clin Immunol. 1999;104(5):1060–5. [PubMed] [Google Scholar]

xiii. Wechsler ME, Garpestad E, Flier SR, et al. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA. 1998;279(half-dozen):455–seven. [PubMed] [Google Scholar]

14. Churg A, Churg J. Steroids and Churg-Strauss. Lancet. 1998;352(9121):32–three. [PubMed] [Google Scholar]

xv. Hellmich B, Ehlers South, Csernok E, et al. Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol. 2003;21(half dozen Suppl 32):S69–77. [PubMed] [Google Scholar]

xvi. Navarro-Mendoza EP, Tobon GJ. Eosinophilic granulomatosis with polyangitis: newer therapies. Curr Rheumatol Rep. 2018;20(five):23. [PubMed] [Google Scholar]

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Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6366375/

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