Indian Journal of Dermatology
CASE REPORT
Year
: 2009  |  Volume : 54  |  Issue : 5  |  Page : 76--77

Churg-strauss syndrome


BK Brar, Vivek Mehta, Dimple Chopra, Asha Kubba 
 Department of Dermatology, Venereology and Leprology, GGS Medical College and Hospital, Faridkot, Punjab, India

Correspondence Address:
B K Brar
C/o Brar Eye Hospital, Lajpat Nagar, Kotkapura - 151 204, Punjab
India

Abstract

We report a case of 29-year-old man who presented with cutaneous vasculitis and was subsequently diagnosed as a case of Churg-Strauss syndrome. The patient fulfilled five out of the six criteria of the syndrome developed by American College of Rheumatology.



How to cite this article:
Brar B K, Mehta V, Chopra D, Kubba A. Churg-strauss syndrome.Indian J Dermatol 2009;54:76-77


How to cite this URL:
Brar B K, Mehta V, Chopra D, Kubba A. Churg-strauss syndrome. Indian J Dermatol [serial online] 2009 [cited 2019 Oct 15 ];54:76-77
Available from: http://www.e-ijd.org/text.asp?2009/54/5/76/45465


Full Text

 Introduction



Wegener granulomatosis (WG), CSS, and the microscopic form of periarteritis (i.e. microscopic polyangiitis) are 3 vasculitic syndromes involving medium- and small-sized vessels that are closely related and are associated with antibodies to neutrophil cytoplasmic antigens (ANCA). Churg-Strauss syndrome is a systemic vasculitis and allergic granulomatosis that was first described in 1951. [1] The syndrome is characterized by three distinct and progressive phases. The initial features are asthma and allergic rhinitis, which may precede systemic disease by many years. The second phase is heralded by fever, weight loss, peripheral eosinophilia, and eosinophilic infiltration of the lungs, upper respiratory tract, and gut. The third phase is manifested by systemic vasculitis, leading to cardiac failure, myocardial infarction, cutaneous disease, peripheral neuropathy, hypertension, renal diseases, and arthralgias. [2]

 Case History



A 29-year-old patient presented with chief complaints of intermittent dry cough, sneezing, rhinorrhea and fever since last six years and hyperpigmented plaque studded with papulo-nodular lesions on trunk and high grade fever since last one month. On examination, single large hyperpigmented infiltrated plaque was seen over chest extending to back on both sides studded with multiple papulo-nodular lesions surrounded by satellite lesions [Figure 1a],[Figure 1b]. Routine investigations revealed Hb-12.0 gm%, DLC as 20% Neutrophils, 5% Lymphocytes, 2% Monocytes, 73% Eosinophils [Figure 2] and ESR was raised (60mm). Platlet count was normal General physical examination was normal. Auscultation of chest revealed expiratory rhonchi. Sputum examination revealed occasional eosinophils. Chest X-ray revealed patchy pulmonary infiltrates in right lung. Histopathology of skin revealed acanthotic epidermis and moderately dense mixed cellular superficial perivascular infiltrate. The infiltrate consisted predominantly of eosinophils admixed with neutrophils and lymphocytes [Figure 3]. X-ray of Paranasal Sinuses showed thickening of mucosa. Patient responded to high dose of steroids in a period of two weeks.

 Discussion



Churg and Strauss [1] histopathologically investigated a group of 13 patients who first developed asthma, followed by fever, hypereosinophilia and systemic small vessel vasculitis noted that it was characterized by necrotizing vasculitis and extravascular granuloma with hypereosinophilia, which distinguished it from classic PAN. The following year, Zeek [3],[4] referred to this vasculitis as allergic granulomatous angitis and distinguished it from hypersensitivity vasculitis as a type of necrotizing vasculitis. The syndrome is characterized by three distinct and progressive phases. The initial features are asthma and allergic rhinitis, which may precede systemic disease by many years. The second phase is heralded by fever, weight loss, peripheral eosinophilia, and eosinophilic infiltration of the lungs, upper respiratory tract, and gut. The third phase is manifested by systemic vasculitis, leading to cardiac failure, myocardial infarction, cutaneous disease, peripheral neuropathy, hypertension, renal diseases, and arthralgias. [5] The vasculitic phase usually develops within three years of the onset of asthma, although it may be delayed for several decades.

Asthma is the central feature of CSS and precedes the systemic manifestations in almost all cases, whereas 70% of the patients have maxillary sinusitis, allergic rhinitis, and/or sinus polyposis. General symptoms are frequent, and associated with pulmonary infiltrates in 38 to 77% of the patients; peripheral neuropathy, usually mononeuritis multiplex, in 64 to 75%; skin involvement in 40 to 70%; and gastrointestinal tract symptoms in 37 to 62%. Cardiac involvement is common, with pericarditis in 23% of the patients and myocarditis in 13%, and represents the primary cause of mortality. [6]

The American College of Rheumatology (ACR) has proposed six criteria for diagnosis of CSS. These criteria are (1) asthma (wheezing, expiratory rhonchi), (2) eosinophilia of more than 10% in peripheral blood, (3) paranasal sinusitis, (4) pulmonary infiltrates (may be transient), (5) histological proof of vasculitis with extravascular eosinophils, and (6) mononeuritis multiplex or polyneuropathy. Masi et al, reported that the existence of four or more of these six criteria yields a sensitivity of 85% and a specificity of 99.7%. [7] The present patient did not suffer from mononeuropathy or polyneuropathy, but fulfilled the other five criteria.

References

1Churg J, Strauss L. Allergic granulomatosis, allergic angiitis and periarteritis nodosa. Am J Pathol 1951;27:277-301.
2Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: A clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984;63:65-81.
3Zeek PM. Periarteritis nodosa and other forms of necrotizing anditis. N Engl J Med 1953;248:764-72.
4Zeek PM. Periarteritis nodosa: A critical review. Am J Clin Pathol 1952;22:777-90.
5Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: A clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984;63:65-81.
6Guillevin L, Pagnoux C, Mouthon L. Churg-strauss syndrome. Semin Respir Crit Care Med 2004;25:535-45.
7Masi A, Hunder G, Lie J, Mchel B, Bloch D, Areend W, et al . The American college of rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angitis). Arthritis Rheum 1991;33:1094-100.