Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Review Article
Review Articles
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Observation Letter
88 (
); 392-395

Spontaneous splenic rupture: A rare complication in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Department of Dermatology, Venereology and Leprology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India
Department of Medicine, Christian Medical College and Hospital, Vellore, Tamil Nadu, India
Department of Pathology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India
Corresponding author: Dr. Dharshini Sathishkumar, Department of Dermatology, Venereology and Leprology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Shajil C, Sathishkumar D, George K, Thomas M. Spontaneous splenic rupture: A rare complication in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Indian J Dermatol Venereol Leprol 2022;88:392-5.


Eosinophilic granulomatosis with polyangiitis is a necrotising vasculitis affecting small- to medium-sized vessels of the skin, usually presenting with palpable purpura or subcutaneous nodules and rarely as urticaria, livedo reticularis or papulonecrotic lesions.1,2 The extracutaneous manifestations are commonly seen in the respiratory tract, peripheral nerves, gastrointestinal tract, kidneys and heart.2,3 Although splenic involvement is rare, we were unable to find any reports of spontaneous splenic rupture in eosinophilic granulomatosis with polyangiitis.3 Here, we report a case of eosinophilic granulomatosis with polyangiitis who had spontaneous splenic rupture 12 days after developing skin lesions.

A 33-year-old lady presented with painful blisters on the extremities for three days associated with fever and paraesthesia involving the feet. She had history of wheezing which improved with steroids and bronchodilators. There was no other history suggestive of connective tissue disease, loss of weight/appetite or drug intake. On examination, she had pedal oedema and multiple dull erythematous papules/plaques on the limbs with a dusky centre and a few also had central vesiculation [Figures 1a-d]. The differentials of cutaneous vasculitis, erythema multiforme and Sweet syndrome were considered and she was initiated on injection hydrocortisone and oral dicloxacillin. Histopathological examination of the skin lesion was consistent with small-vessel vasculitis with perivascular eosinophilic infiltrate [Figures 2a and b] and direct immunofluorescence showed immunoglobulin M deposition in a few superficial dermal blood vessels. The relevant laboratory investigations and imaging findings are shown in Table 1. A day after upper gastrointestinal endoscopy was done for the evaluation of trace blood in stools, she developed diffuse abdominal pain with swelling, tachycardia, cold peripheries, hypotension and persistent oozing from the site of intravenous cannulation.

Figure 1a:: Multiple dull erythematous papules and plaques with dusky necrotic centre and a few with central vesicle/bulla on legs
Figure 1b:: Multiple dull erythematous papules and plaques with dusky necrotic centre and a few with central vesicle/bulla on dorsum of hand
Figure 1c:: Multiple dull erythematous papules and plaques with dusky necrotic centre and a few with central vesicle/bulla on dorsa of feet
Figure 1d:: Multiple dull erythematous papules and plaques with dusky necrotic centre and a few with central vesicle/bulla on right forearm
Figure 2a:: Medium-vessel vasculitis with organising thrombi in deep dermis (haematoxylin and eosin, ×40)
Figure 2b:: Perivascular scattered eosinophils (black arrow) (haematoxylin and eosin, ×200)
Figure 2c:: Vasculitis with fibrinoid necrosis (black arrow) involving red pulp of spleen and perivascular eosinophilic infiltrate (blue arrow) (haematoxylin and eosin, ×100)
Table 1:: Relevant laboratory investigations and imaging
Investigations Results (normal range)
C-reactive protein 109 mg/L (<6)
Total count and differential count 1 2,400 cells/mm3 (4000-12,000), neutrophils 59%, lymphocytes 16%, eosinophils 21%
Platelet count (at presentation) 137,000 cells/mm3 (150,000–450,000)
Platelet count (day seven) 44,000 cells/mm3
Creatinine 0.57 mg% (0.5–1.4)
Urine microscopy Normal
Stool occult blood (two numbers) Trace
Immunoglobulin E 12,673.3 U/ml (5–100)
ASO <50 IU/ml (<300)
Cold agglutination Negative
Blood culture No growth
ANA Negative
C3/C4 118/38.3 mg/dl (90–180/10–40)
Anti-PR3§ (c-antineutrophil cytoplasmic antibody)/anti-myeloperoxidase (p-antineutrophil cytoplasmic antibody) <2 U/ml (<20)/ 4.2 U/ml (<20)
Rheumatoid arthritis factor <8.8 IU/ml (<20)
Cryoglobulins Not detected
Anti-prothrombin phosphatidylserine complex antibody 4 U/ml (<16)
Anticardiolipin antibody 2 GPL (<20)
Lupus anticoagulant Negative
Antibodies to β2 glycoprotein complex immunoglobulin M/immunoglobulin G 6/2 Ru/ml (<20/<20)
Anti-SSA antibodies 1 Ru/ml (<20)
Homocysteine estimation 6.1 umol/L (3.7–13.9)
Chest X-ray and computed tomography thorax Well-defined 4×3 mm calcified soft-tissue nodule in the left lung (sequela of past infection/inflammation)
Nerve conduction study Sensorimotor axonal neuropathy involving the lower limbs
ASO: Antistreptolysin O, ANA: Antinuclear antibody,
Anti-PR3: Antiproteinase 3

Laboratory investigations showed a drop in haemoglobin (from 11 g/dl to 4.2 g/dl) and prolonged coagulation parameters, suggesting disseminated intravascular coagulation. The patient was resuscitated and rushed to the operation theatre considering the possibility of an intraperitoneal bleed. Intraoperative upper gastrointestinal endoscopy ruled out gastrointestinal perforation and emergency laparotomy revealed massive haemoperitoneum with mild splenomegaly and stellate rupture of the spleen. Splenectomy was done and histopathological examination of the splenic tissue showed vasculitis with eosinophilic infiltrate [Figure 2c]. After surgery, the patient gradually recovered with antibiotics (meropenem, metronidazole) and other supportive measures. The diagnosis of eosinophilic granulomatosis with polyangiitis was made according to the American College of Rheumatology criteria.1 Her skin lesions showed remarkable improvement [Figure 3] on treatment with prednisolone (0.6 mg/kg) and mycophenolate mofetil. The disease has been in remission for the past 2.5 years and she is currently on low-dose prednisolone (5 mg) and mycophenolate mofetil.

Figure 3:: Lesions have completely healed with post-inflammatory hyperpigmentation (2.5 years follow-up)

Eosinophilic granulomatosis with polyangiitis is an antineutrophil cytoplasmic antibody-associated vasculitis and its natural course is divided into three phases. The first phase is characterised by allergic rhinosinusitis and adult-onset asthma followed by peripheral blood eosinophilia with end-organ eosinophilic infiltrate in phase two. Systemic vasculitis with granulomatous inflammation in phase three can lead to complications such as myocardial infarction, heart failure, gastrointestinal perforation and venous thromboembolism.1,2

The American College of Rheumatology criteria proposed in 1990 include asthma, eosinophilia, neuropathy, sinusitis, non-fixed pulmonary infiltrate and eosinophilic infiltrate in the biopsy. The presence of four out of six criteria diagnoses the condition with 85% sensitivity and 99.7% specificity.1,2 Our patient had cutaneous small-vessel vasculitis with eosinophilic infiltrate, asthma, peripheral eosinophilia and peripheral neuropathy, thus fulfilling the criteria. Her raised immunoglobulin E level also favoured the diagnosis. Although extravascular red granulomas are described in eosinophilic granulomatosis with polyangiitis, it is neither a pathognomonic feature nor mandatory for diagnosis.2,3 Antineutrophil cytoplasmic antibody positivity, absent in this case, is not a prerequisite for diagnosis, as it is seen in only 40–60% eosinophilic granulomatosis with polyangiitis cases.1,2 The prognosis depends on the severity of the disease, with studies demonstrating five-year survival rate of 88.9% and ten-year relapse-free survival rate of 54.4%.1,2

The occurrence of spontaneous splenic rupture makes this case unique. Spontaneous splenic rupture is a rare morbid condition, causes of which fall into six major groups: neoplastic, infectious, inflammatory, structural, iatrogenic and idiopathic.4,5 It is reported in various rheumatologic conditions such as rheumatoid arthritis, systemic lupus erythematosus, polyarteritis nodosa with greater frequency and in granulomatosis with polyangiitis infrequently.5 It presents with left upper abdominal pain, abdominal distension, tenderness and guarding. Ultrasound helps in rapid diagnosis with exploratory laparotomy being both therapeutic and diagnostic. Although the predisposing factors for spontaneous splenic rupture have not been clearly defined, McCain et al. have proposed splenic vasculitis, vessel destruction and necrosis as the possible triggers.4,5 In our case, spontaneous splenic rupture was probably secondary to splenic vasculitis as evident on histopathological examination. She was vaccinated to prevent overwhelming post-splenectomy infections, a fatal long-term complication following splenectomy.

Even though spontaneous splenic rupture is a rare complication of vasculitis, it should be considered in a patient with vasculitis presenting with features of with acute abdomen, as timely diagnosis and intervention improve the overall outcome as evident in our case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


  1. . Cutaneous vasculitis. J Am Acad Dermatol. 2003;48:311-44.
    [CrossRef] [PubMed] [Google Scholar]
  2. , , , . Eosinophilic granulomatosis with polyangiitis: An overview. Front Immunol. 2014;5:549.
    [CrossRef] [PubMed] [Google Scholar]
  3. , , , , . Eosinophilic granuloma and necrotizing vasculitis (Churg-Strauss syndrome) Involving a parotid gland, lymph nodes, liver and spleen. Scand J Rheumatol. 1989;18:171-5.
    [CrossRef] [PubMed] [Google Scholar]
  4. , , , , . Life-threatening spontaneous splenic rupture with systemic lupus erythematosus: Case report and literature review. Clin Rheumatol. 2012;31:1019-25.
    [CrossRef] [PubMed] [Google Scholar]
  5. , , , , , , et al. Splenic rupture as the presenting manifestation of vasculitis. Semin Arthritis Rheum. 2002;31:311-6.
    [CrossRef] [PubMed] [Google Scholar]

Fulltext Views

PDF downloads
View/Download PDF
Download Citations
Show Sections