THE „LIMPING‰ ALLERGIC MARCH Budiman Gunawan, M.D.,* Evelyn O. Salido, M.D.* and Jonah Amora, M.D.* 279 Phil. J. Internal Medicine, 47: 279-286, Nov.-Dec., 2009 *Department of Internal Medicine, De La Salle University Medical Center. Reprint request to: Budiman Gunawan, M.D., Department of Internal Medicine, De La Salle University and Medical Center, Dasmariñas, Cavite, Philippines. ABSTRACT Background: Churg-Strauss syndrome (CSS), also referred to as allergic granulomatous vasculitis, is a rare systemic vasculitis that affects small to medium sized arteries and veins. Characteristic features include the presence of asthma, paranasal sinusitis, eosinophilia, pulmonary infiltrates, and mononeuritis multiplex. Histopathologic examination confirms the disease. 1 Setting: Dela Salle University Medical Center (DLSUMC), a tertiary training hospital in Dasmariñas, Cavite, Philippines The Case : A 59 year old female with a history of poorly controlled asthma and allergic rhinitis manifested with palpable purpura and hemorrhagic bullae, anemia, gastrointestinal bleeding, pansinusitis, lumbar radiculopathy, and eosinophilia. Notable was the past occurrence of peripheral neuropathy which resolved upon administration of prednisone. Skin biopsy revealed dense neutrophil and neutrophilic nuclear clusters in the dermis and within the blood vessel walls. There were numerous eosinophils within the infiltrates and extravasated erythrocytes in the dermis consistent with Churg-Strauss syndrome. During her incumbent hospitalization, the skin lesions, gastrointestinal hemorrhage, and asthma rapidly resolved on institution of high dose steroids. Gabapentin was used to control pain secondary to radiculopathy. Significance: This report highlights the importance of considering Churg-Strauss syndrome among asthmatics presenting with systemic vasculitis. INTRODUCTION Asthma is a chronic inflammatory disorder of the airways resulting in bronchospasm, which may be completely or partially reversed with or without specific therapy. Airway inflammation is the result of interactions between various cells, cellular elements, and cytokines. Asthma is one of the most common chronic diseases globally affecting about 300 million people. 2 This condition is likewise prevalent among Filipinos. International statistics by country for asthma showed that 5.5 million (15.6%) Filipinos are afflicted with asthma. 3 Asthma is usually associated with atopic diseases like allergic rhinitis and atopic dermatitis. Rarely, in 1-3 per million individuals, it may be one of the manifestations of a systemic inflammatory disease like Churg-Strauss syndrome. In addition to a prodrome of asthma and allergic rhinitis, this syndrome is characterized by hypereosinophilia, and necrotizing vasculitis, associated with extravascular eosinophilic granulomas. 1 This report is a case of a 59 year old Filipina that shows the evolution of persistent bronchial asthma into a multisystemic inflammatory disease. Clinical Summary A 59 year old Filipino female was admitted at the Dela Salle University Medical Center in Dasmariñas, Cavite, Philippines due to progressive dyspnea. The admitting impression was bronchial asthma in acute exacerbation with community-acquired pneumonia. The patient has had asthma for 14 years. She managed her monthly exacerbations with salbutamol and ipratropium bromide nebulizations. She was maintained on fluticasone nasal spray for allergic rhinitis. Nine years prior to admission, she developed inability to dorsiflex the right ankle (foot drop). This was associated with elevation of the erythrocyte sedimentation rate (ESR= 45) with nerve conduction velocity (NCV) study findings of peripheral neuropathy, affecting all extremities with the lower more than the upper extremities. There is a significant affectation of the right tibial and peroneal nerves, an axonopathy more than myelinopathy. The impression then was systemic vasculitis and this was managed with prednisone 1 mg/kg/day initially then gradually tapered over six months. Response was good; she was able to ambulate independent of any walking aid after a few weeks. Physical therapy was also initiated for muscle strengthening. She was then lost to follow-up.
Transcript
The „Limping‰ Allergic March Asthma, Allergic Rhinitis, Vasculitis and Motor Weakness 279
THE „LIMPING‰ ALLERGIC MARCH
Budiman Gunawan, M.D.,* Evelyn O. Salido, M.D.* and Jonah Amora, M.D.*
279
Phil. J. Internal Medicine, 47: 279-286, Nov.-Dec., 2009
*Department of Internal Medicine, De La Salle University Medical Center.
Reprint request to: Budiman Gunawan, M.D., Department of Internal Medicine, De La Salle University and Medical Center, Dasmariñas, Cavite, Philippines.
ABSTRACT
Background: Churg-Strauss syndrome (CSS), also referred to as allergic granulomatous vasculitis, is a rare systemic vasculitis that affects small to medium sized arteries and veins. Characteristic features include the presence of asthma, paranasal sinusitis, eosinophilia, pulmonary infiltrates, and mononeuritis multiplex. Histopathologic examination confirms the disease.1
Setting: Dela Salle University Medical Center (DLSUMC), a tertiary training hospital in Dasmariñas, Cavite, Philippines
The Case: A 59 year old female with a history of poorly control led asthma and al lergic rhinit is manifested with palpable purpura and hemorrhagic bullae, anemia, gastrointestinal bleeding, pansinusitis, lumbar radiculopathy, and eosinophilia. Notable was the past occurrence of peripheral neuropathy which resolved upon administration of prednisone. Skin biopsy revealed dense neutrophil and neutrophilic nuclear clusters in the dermis and within the blood vessel walls. There were numerous eosinophils within the infiltrates and extravasated erythrocytes in the dermis consistent with Churg-Strauss syndrome. During her incumbent hospital ization, the skin lesions, gastrointestinal hemorrhage, and asthma rapidly resolved on institution of high dose steroids. Gabapentin was used to control pain secondary to radiculopathy.
Significance: This report highlights the importance of cons ider ing Churg-Strauss syndrome among asthmatics presenting with systemic vasculitis.
INTRODUCTION
Asthma is a chronic inflammatory disorder of the airways resulting in bronchospasm, which may be completely or partially reversed with or without specific therapy. Airway inflammation is the result of interactions between various cells, cellular elements,
and cytokines. Asthma is one of the most common chronic diseases globally affecting about 300 million people.2 This condition is likewise prevalent among Filipinos. International statistics by country for asthma showed that 5.5 million (15.6%) Filipinos are afflicted with asthma.3 Asthma is usually associated with atopic diseases like allergic rhinitis and atopic dermatitis. Rarely, in 1-3 per million individuals, it may be one of the manifestations of a systemic inflammatory disease like Churg-Strauss syndrome. In addition to a prodrome of asthma and allergic rhinitis, this syndrome is characterized by hypereosinophilia, and necrotizing vasculitis, associated with extravascular eosinophilic granulomas.1 This report is a case of a 59 year old Filipina that shows the evolution of persistent bronchial asthma into a multisystemic inflammatory disease.
Clinical Summary
A 59 year old Filipino female was admitted at the Dela Salle University Medical Center in Dasmariñas, Cavite, Philippines due to progressive dyspnea. The admitting impression was bronchial asthma in acute exacerbation with community-acquired pneumonia. The patient has had asthma for 14 years. She managed her monthly exacerbations with salbutamol and ipratropium bromide nebulizations. She was maintained on fluticasone nasal spray for allergic rhinitis.
Nine years prior to admission, she developed inability to dorsiflex the right ankle (foot drop). This was associated with elevation of the erythrocyte sedimentation rate (ESR= 45) with nerve conduction velocity (NCV) study findings of peripheral neuropathy, affecting all extremities with the lower more than the upper extremities. There is a significant affectation of the right tibial and peroneal nerves, an axonopathy more than myelinopathy. The impression then was systemic vasculitis and this was managed with prednisone 1 mg/kg/day initially then gradually tapered over six months. Response was good; she was able to ambulate independent of any walking aid after a few weeks. Physical therapy was also initiated for muscle strengthening. She was then lost to follow-up.
280 Gunawan B, Salido EO and Amora J
Five months prior to admission, the patient noticed minute painless, erythematous macules on the dorsum of her hand and fingers. These lesions resolved spontaneously after three weeks.
Two weeks prior to admission, she developed severe dyspnea, temporarily relieved by nebulization. A week later, there was note of pain and tingling sensations on both lower extremities, more prominent on the right leg. This was temporarily alleviated by tramadol 50 mg three times a day. Four days prior to admission, she developed high grade fever, persistent dyspnea, pleuritic chest pain, palpitations and cough productive of greenish-yellow sputum, Two days before admission, she consulted at another health facility where she was found to be hypotensive. Intravenous ceftriaxone and inotropics were started. Once stable, the patient was transferred to the De La Salle University Medical Center (DLSUMC) for further management.
Regional examination showed a pale, tachypneic and febrile patient with hypotension (BP 80/60 mmHg). Auscultation of the lungs showed fine crackles and wheezes over both lung fields but more prominent on the right hemithorax. Cardiac and abdominal examinations were within normal. Motor strength testing revealed grade 3/5 weakness on the right leg. No sensory deficit was noted. Initial laboratory investigation included complete blood count, which showed mild anemia (Hgb of 113 G/L), leukocytosis (WBC of 15.1 X 109 / L), and eosinophilia (0.35). Arterial blood gas analysis revealed respiratory alkalosis with hypoxemia. There were reticulohazed densities on the lower lobe of the right lung on chest roentgenogram ( Fig 1 Appendix 4). Blood culture and sensitivity studies revealed no aerobic growth after five days of incubation. Creatinine, sodium, potassium, SGOT and SGPT were within normal limits (Appendix 1). Initial management consisted of piperacillin-tazobactam 4.5 g IV q8h, ultrasonic nebulization with salbutamol / ipratropium and intravenous hydrocortisone 100 mg every 8 hours. Hypotension resolved after the first hospital day and inotropic support was discontinued. On the second hospital day, she developed palpable purpura. These appeared initially on both hands, progressing to involve the arms, shoulders, both ears, and both lower extremities in the said order. Some lesions were noted to be tender, while several coalesced and became hemorrhagic bullae. No lesions were noted on the oral mucosa. Simultaneous with this, the patient had melena and was noted to be weak and anemic. The appearance of the skin lesions and gastrointestinal hemorrhage in an asthmatic
patient with a peripheral neuropathy suggested the diagnosis of Churg-Strauss syndrome (CSS). Other findings, notably of pansinusitis on paranasal sinus roentgenograms ( Fig 2,3,4 - appendix 4 ), and skin histopathology supported the CSS diagnosis. Skin biopsy revealed dense neutrophil and neutrophilic nuclear clusters in the dermis and within blood vessel walls, numerous eosinophils within the infiltrates, and extravasated erythrocytes in the dermis. NCV findings were consistent with bilateral lumbosacral radiculopathy, right more affected than the left, with signs of demyelination, axonopathic, acute, and chronic denervation changes. ( Appendix 2 )
Hydrocor tisone was continued and packed red blood cell transfusion was given. Gabapentin was used to control neuropathic pain. The patient responded well to treatment; the skin lesions rapidly improved, asthma was controlled, gastrointestinal hemorrhage resolved, and neuropathic pain gradually subsided. Hydrocortisone was shifted to prednisone for possible eventual tapering once disease activity is fully controlled.
DISCUSSION
Diagnosis Churg and Strauss first described the syndrome in a series of thirteen patients who had asthma, eos inoph i l ia , g ranu lomatous in f lammat ion , necrotizing systemic vasculitis, and necrotizing glomerulonephritis.4
The precise pathogenesis of this disease is still uncertain, although its strong association with asthma and its clinicopathologic manifestations point to aberrant immunologic processes. The presence of a marked peripheral eosinophilia and secretory products of eosinophils in blood and tissues implicates a pathogenetic role of eosinophil granulocytes. One contributing factor for the prominent eosinophilia appears to be the prolonged survival of eosinophils due to inhibition of CD95-mediated apoptosis by soluble CD95. Eosinophil-activating cytokines secreted from T lymphocytes have also been implicated.5
Recent investigations have arisen regarding the possible relation of the use of leukotriene receptor antagonists for asthma and Churg-Strauss syndrome. One study, however, did not note any pathogenetic role.6 Another series postulated that leukotriene modifiers may unmask an underlying vasculitic syndrome that is initially clinically recognized as moderate to severe asthma and treated with corticosteroids.7
The „Limping‰ Allergic March Asthma, Allergic Rhinitis, Vasculitis and Motor Weakness 281
The clinical elements of this syndrome occur in several sequential phases, namely the prodromal, eosinophilic, and vasculitic phases. The prodromal phase is characterized by atopic disease, allergic rhinit is, and asthma. The eosinophil ic phase features peripheral eosinophilia and eosinophilic infiltration of multiple organs, notably the lungs and gastrointestinal tract. The vasculitic phase constitutes a life-threatening systemic vasculitis of small vessels. Asthma usually precedes the vasculitic phase by approximately eight to ten years. This patient had five years history of asthma before she manifested with CSS.
In one retrospective study of 96 patients with CSS, asthma was the most frequently observed presenting manifestation (97%). This was followed by mononeuritis multiplex (77%) and allergic rhinitis (61%).8 This patient had all these three symptoms at the onset.
Necrotizing vasculitis mediated by cytotoxic T cells leading to ischemia, appears to be the major cause of the neuropathy. This may progress into asymmetrical polyneuropathy, restricted to the limbs.9 Electromyography and nerve conduction velocity studies were performed in our patient. The findings showed bilateral lumbosacral radiculopathy, right more affected than the left, with signs of demyelination, axonopathic, acute, and chronic denervation changes.
Skin manifestations, such as nodules, urticarial rash, necrotic bullae, and digital ischemia occur in 49 percent of affected patients. This patient had hemorrhagic bullae at the upper and lower extremities but these rapidly improved with steroid treatment. Gastrointestinal symptoms have been shown to occur in 31 percent of CSS patients; bleeding in 18 percent.8 Notably, our patient developed melena necessitating blood transfusion (Hgb 85 G/L) on the fourth hospital day. This was initially thought to be from steroid-induced gastritis, but was eventually attributed to gastrointestinal vasculitis. There were no signs of bleeding three days after steroid initiation. Diagnostic endoscopy was planned, but the patient refused the procedure. Using the American College of Rheumatology criteria for the diagnosis of CSS (Appendix 3), the patient fulfills five of the six criteria (namely, asthma, eosinophilia >10%, paranasal sinusitis, histological proof of vasculitis, mononeuritis multiplex or polyneuropathy). This set of criteria has a sensitivity of 85 percent and a specificity of 99.7percent.10 The presence of anti-neutrophil cytoplasmic autoantibodies (ANCA) has been documented
in patients with Churg-Strauss syndrome, at approximately 48 percent.1 Testing for ANCA is not helpful in establishing or excluding a diagnosis because of this low frequency of ANCA positive tests in patients with this syndrome. However, its presence can be correlated with certain manifestations of the disease. Positive ANCA status at diagnosis was associated with renal involvement, peripheral neuropathy, and biopsy-proven vasculitis, whereas negative ANCA status was associated with heart disease and fever.11 The ANCA test was not done in this patient. Lung biopsy is the gold standard for the diagnosis of Churg-Strauss syndrome.12 Biopsy of skin lesions can also be performed, preferably extending to the subcutis and taken from the most tender, reddish or purpuric lesional skin to obtain a significant diagnostic result.13 In one retrospective case series involving 90 patients with Churg-Strauss syndrome, 29 patients who underwent skin biopsy showed extravascular necrotizing granuloma (15 specimens) and leukocytoclastic vasculitis (16 specimens).14 Results of skin biopsy in our patient showed leukocytoclastic vasculitis as described.
Treatment Therapy of Churg-Strauss syndrome consists mainly of systemic glucocorticoids, which appear to be effective in many patients.1 In this patient, intravenous hydrocortisone was started as treatment for asthma and continued for the vasculitis as well. The skin lesions responded well to corticosteroids alone. (Fig 1,2 - Appendix 5) There was no need to add other immunosuppressants, like cyclophosphamide which is preferred in cases of glucocorticoid failure or in fulminant multisystem disease.1 A multicenter therapeutic trial involving 48 patients with poor prognostic factors showed better disease control with the 12-monthly over the 6-monthly cyclophosphamide pulse regimen.15 Other treatments like interferon-alpha, TNF-alpha blockers, and intravenous immunoglobulin, have been repor ted to have beneficial effects in a few patients.16,17, 18
Most deaths result from complications of the vasculitic phase, commonly due to cardiac failure or myocardial infarction, cerebral hemorrhage, renal failure, gastrointestinal bleeding, or status asthmaticus.8 Several clinical features correlate strongly with subsequent mortality. In one large series, the presence of at least one of five of the clinical factors score (FFS) was found to have significant prognostic value.19 These five factors are namely, cardiac involvement, gastrointestinal disease, renal insufficiency (plasma creatinine > 1.6 mg /
282 Gunawan B, Salido EO and Amora J
dL, proteinuria > 1 g / day, central nervous system involvement. A FFS of 0 connotes a 12 percent five-year mortality rate; FFS of 1, a 26 percent five-year mortality rate; FFS >3, 46 percent five-year mortality rate. Based on this sensing system, this patient had only one factor (gastrointestinal disease) and thus a fairly good prognosis. This patient was discharged after eight hospital days with controlled asthma, resolving skin lesions, and an acceptable hemoglobin level (110 G/L). She was maintained on prednisone at 1 mg/kg/day, budesonide 160 mg + formoterol 4.5 mcg turbuhaler 1 puff twice daily, omeprazole 20 mg once daily, gabapentin 300 mg once daily. Two weeks later on follow-up, the skin lesions were almost completely resolved (Fig 3,4,5,6 - Appendix 5) with diminished leg pains. Prednisone was subsequently tapered.
CONCLUSION
The case of a 59 year old Filipina with 14 years history of asthma and allergic rhinitis subsequently showing manifestations of a systemic vasculitis was presented. In this patient, vasculitis presented five years after the onset of asthma as a neuropathy, with first symptom of foot drop at the right. This bout of vasculitis was controlled but flared up after nine years in the form of hemorrhagic bullae, gastrointestinal bleeding, and radiculopathy. Skin biopsy established the diagnosis of CSS in this patient. She had good response to corticosteroid treatment, with symptoms starting to resolve four days after initiation of steroids. Physicians who encounter cases of asthma have to consider the possibility of CSS when other clinical manifestations and hypereosinophilia are present.
REFERENCES
1. Fauci, A, Braunwald E, Kasper, D, Hauser S, Longo, D, Jameson, J, Loscalzo, J. HarrisonÊs Principles of Internal Medicine, 17th Ed. 2008. Mc-Graw Hill.
2. Beasley DM, et al: Prevalence and Ethiology of Asthma, J Allergy Clin Immunol S 466, 2000.
3. US Census Bureau, International Data Base, 2004. www.cueresearch.com
4. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. Mar-Apr 1951;27(2):277-301.
5. Hellmich B, Ehlers S, Csernok E, Gross WL. Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol. Nov-Dec 2003;21(6 Suppl 32):S69-77.
6. Keogh KA, Specks U. Churg-Strauss syndrome: cl in ical presentat ion, ant ineutrophi l cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. Sep 2003;115(4):284-90.
7. Wechsler ME, Finn D, Gunawardena D, et al.: Churg-Strauss Syndrome in Patients Receiving Montelukast as Treatment for Asthma. Chest. Mar 2000;117(3):708-13.
8. Guillevin L, Cohen P, Gayraud M, et al.: Churg-Strauss Syndrome. Clinical Study and Long-term Follow-up of 96 Patients. Medicine (Baltimore). Jan 1999;78(1):26-37.
9. Hattori N, Ichimura M, Nagamatsu M, Li M, Yamamoto K, Kumazawa K, Mitsuma T, Sobue G: Clinicopathological Features of Churg-Strauss Syndrome-Associated Neuropathy. Brain.; 122 (Pt3 ):427, 1999.
10. Masi AT, Hunder GG, Lie JT, et al.: The American College of Rheumatology 1990 Criteria for the Classification of Churg-Strauss Syndrome (Allergic Granulomatosis and Angiitis). Arthritis Rheum. Aug 1990; 33(8):1094-100.
11. Sablé-Four tassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L , Jayne D, B lockmans D, Cord ie r JF, Delaval P, Puechal X, Lauque D, Viallard JF, Zoulim A, Guillevin L; Ann Intern Med.; 143(9): 632, 2005.
12. Allen JN, Davis WBL Eosinophilic Lung Diseases. Am J Respir Crit Care Med.; 150 (5 Pt 1): 1423, 1994.
13. Chen KR, Carlson JA: Clinical Approach to Cutaneous Vasculitis. Am J Clin Dermatol.; 9(2): 71, 2008.
14. Davis MD, Daoud MS, McEvoy MT, Su WP: Cutaneous Man i f es ta t i ons o f Churg-S t rauss Syndrome: A Clinicopathologic Correlation. J Am Acad Dermatol.; 37(2 Pt 1):199, 1997.
15. Cohen P, Pagnoux C, Mahr A, Arène JP, Mouthon L, Le Guern V, André MH, Gayraud M, Jayne D, Blöckmans D, Cordier JF, Guillevin L. Arthritis Rheum.; 57(4): 686, 2007.
16. Reissig A, Förster M, Mock B, Schilder C, Kroegel C. [ Inter feron-Alpha Treatment of the Churg-Strauss Syndrome]. Dtsch Med Wochenschr.; 128(27):1475, 2003.
17. Arbach O, Gross WL, Gause A: Treatment of Refractory Churg-Strauss Syndrome by TNF-Alpha Blockade. Immunobiology; 206(5):496, 2002.
18. Taniguchi M, Tsurikisawa N, Higashi N, Saito H, Mita H, Mori A, Sakakibara H, Akiyama K: Treatment for Churg-Strauss Syndrome: induction of Remission and Efficacy of Intravenous Immunoglobulin Therapy. Allergol Int.; 56(2): 97, 2007.
19. Guillevin L, Lhote F, Gayraud M, et al.: Prognostic Factors in Polyarteritis Nodosa and Churg-Strauss Syndrome. A Prospective Study in 342 Patients. Medicine (Baltimore). Jan 1996;75(1):17-28
20. Katzenstein AL: Diagnostic Features and Differential Diagnosis of Churg-Strauss Syndrome in the Lung. A Review. Am J Clin Pathol.; 114(5):767, 2000.
Hemoglobin 113 G/L 85 G / L 110 G / L Hematocrit 0.33 0.26 0.33 WBC 15.1 x 109 / L 5.8 X 109 / L Segmenters 0.52 0.83 Lymphosites 0.1 0.15 Eosynophils 0.35 0.02 Platelet Adequqte 450 X 109 / L Creatinine 46 u mol / L Sodium 134 mmol / L Potassium 3.9 mmol / L SGOT 44.00 u / L SGPT 30.00 u / L A B G:
pH 7.4 7.47 PCO2 29.1 mmHg 29.3 mmHg PO2 55 mmHg 116 mmHg HCO3 14.6 mEq / L 21.3 mEq / L BE ( - ) 8.1 (-)1.4 mEq / L O2 Sat 99.40% 98.90% Urinalysis:
Color light yellow Specific Gravity Clear pH 6.5 Albumin Negative Sugar Negative WBC 0 3 / LPF RBC 1 2 / LPF Blood C/S No growth after 24 hour No Aerobic growth after 5 day Occult blood positive
APPENDIX 2
EMG & NCV findings:
1. No response obtained upon stimulation of right peroneal and tibial compound muscle action. 2. Delayed onset latency decreased amplitude noted on left tibial compound muscle action potential. 3. Decreased amplitude noted on left peroneal compound muscle action. 4. Normal H reflex studies obtained for both gastrocnemius muscles. 5. Positive sharp wave potentials noted on both upper, middle and lower paralumbar muscles. 6. Positive sharp wave potentials noted on both medial gastronemeus and right anterior tibialis muscles. 7. Complex repetitive discharges noted on middle paralumbar.
284 Gunawan B, Salido EO and Amora J
8. Reduced recruitment pattern noted on right medial gastrocnemius muscles. 9. The rest of muscle tested were electrically silent at rest.
Impression:
Lumbosacral radiculopathy, bilateral ( right more affected than left ), incomplete affecting both L4-5 and S1 nerve roots, with signs of demyelination, axonopathic, acute and chronic denervation changes.
DERMOPATHOLOGY REPORT
Gross description :
Cylindreal skin segment measyred of 0.4 cm in diameter X 0.3 cm in thickness entirely submitted.
Microscopic description :Sections show dense neutrophils and neutrophilic nuclear clust in the dermis and within blood vessel walls.Fibrin deposition is present in endothelial wall. There are numerous eosinophils within the infiltrate and extravasated erythrocytes in the dermis.
APPENDIX 3
The American College of Rheumatology classification criteria for Churg Strauss Syndrome
• Asthma (a history of wheezing or the finding of diffuse high pitched wheezes on expiration)
• Eosinophilia of >10 percent on differential white blood cell count
• Mononeuropathy (including multiplex) or polyneuropathy
• Migratory or transient pulmonary opacities detected radiographically
• Paranasal sinus abnormality
• Biopsy containing a blood vessel showing the accumulation of eosinophils in extravascular areas
The presence of four or more of these criteria yields a sensitivity of 85 percent and a specificity of 99.7 percent for CSS
The „Limping‰ Allergic March Asthma, Allergic Rhinitis, Vasculitis and Motor Weakness 285
APPENDIX 4
Fig 1. Chest PA Showed Prominent of Bronchovascular Marking with Suspicious Densities in Both Upper Lobes. Reticulohazed Densities in the Right Lower Lobe. Heart is Top Normal in Size. Aortic Knob Calcification. Tenting of the Right Hemidiaphragm.
Fig 2, 3, 4. There is Haziness of the Sphenoid, Both Frontal, Ethmoid and Maxillary Sinuses, Suggestive of Pansinusitis
286 Gunawan B, Salido EO and Amora J
APPENDIX 54 DAYS OF HOSPITALIZATION
Fig. 1 Fig. 2
Fig 1, 2. Randomly Distributed Erythematous to Violaceous Macules, Papules, and Patches Over Both Upper Extremities
Fig. 3 Fig. 4
Fig. 5 Fig. 6
Fig 3,4,5 6. Resolving Skin Lesions Seen at All Extremities
