This document describes the admission of a 3-year-old female patient presenting with skin lesions and fever for 5 months. On examination, she has eczematous lesions covering 90% of her body surface area, indicative of erythroderma. Initial tests show hyper-eosinophilia but rule out underlying infections. A working diagnosis of idiopathic hyper-eosinophilia syndrome is made. She shows significant clinical improvement on oral steroids, with resolution of fever and improvement of erythroderma. Further follow up is planned while tapering steroids over 2-3 months.

1. Admission Rounds
13 / 09/2011
Dr Saptharishi L G
Pediatric Allergy- Immunology

2. Patient details
 Name :S
 Age : 3 yrs
 Sex : female
 R/o : KARNAL , Haryana
 Father  Daily wage employee
 Mother  Housewife
Admitted in Pediatric Allergy Immunology

3. Chief complaints
Skin lesions  5 months
Fever  5 months

4. Skin Lesions

5. History of presenting illness
 Fever Most bothersome
to the parents
 Moderate grade
 Intermittent ; multiple spikes per day
 Documented up to 102 F
 Intermittently a/w chills and rigors
 No diurnal variation
 Responsive to anti-pyretics ; transiently
 Maximum fever free interval  10 days
 a/w decreasing activity / poor feeding
 a/w loss of weight / bed bound / stopped walking

6. History of presenting illness
 Loose stools
 During 3rd month of illness
 Duration – 14 days
 Hospital admission – 6 days
 Increased frequency / altered consistency
 No blood / mucus
 Responded to IV dehydration correction and IV
antibiotics
 Subsequently stool frequency / consistency – WNL

7. Relevant negative history
 No h/o rapid breathing / burning mictuirition / vomiting
 No h/s/o any focus of sepsis
 No h/o photosensitivity / malar rash
 No h/o any mucosal involvement
 No h/o any skin nodules / joint involvement
 No h/o similar illness/ skin lesions in the past
 No h/o any musculoskeletal / abdominal pain
 No recent travel / contact with animals
 No h/o clinical repsonse to antibiotics /anti-helminthics

8. PAST HISTORY
 No h/o skin rash / atopy-like illness in the first yr of life
 No known drug / other allergies
 No h/o similar illness in the past
 No h/o any other major illnesses / hospitalizations
 H/o one blood transfusion 15 days back

9. FAMILY HISTORY
30 yr old 33 yr old
Abortion 6 yr old 3 yr old

10. PERINATAL HISTORY
 Not contributory
 Born by Full term Normal vaginal delivery
at home
 Birth weight  Not known
 No adverse antenatal / perinatal events

11. Other relevant history
 Developmentally Normal
 Immunized appropriate for age
 Poor socio-economic status
 ENVIRONMENTAL HISTORY :
 No h/o contact with dogs/ cats
 No h/o poultry / cattle near home
 No h/o consumption of unpasteurized milk
 No definite allergen could be identified on history
 No h/o similar rash in other family members /
neighbors

12. Treatment History
 Treatment on OPD basis from multiple private
practitioners
 Received topical and indigenous oral
preparations
 h/o improvement in skin rash after topical
application
 No h/o addition or withdrawal of drugs in the
last 2 wks

13. Summary of the history

14. General &
Systemic
Examination

15. General examination
 Alert , active and interacting well with mother
HEAD to TOE examination
Examination of the skin

21. PALMS & SOLES – Involved
Desquamation present but not as
prominent as the rest of the body

22. Dermatological findings
 Eczematous lesion with oozing
 Areas showing secondary skin changes including
crusting, lichenification
 Associated with redness of skin – underlying /
surrounding
 Seborrheic dermatitis of scalp
 Total body surface area involved  90 %
ERYTHRODERMA
CAUSE  ? Atopic eczema

23. Anthropometry
 WEIGHT – 9.3 kg ( 66% of expected)
 Height - 86.5 cm (91% of expected)
 OFC - 47 cm (WNL)

24. Weight-for-age GIRLS
Birth to 5 years (z-scores)
30 30
3
28 28
2.5
26 26
2
24 24
22 22
20 20
Weight (kg)
18 0 18
16 16
14 -2 14
-2.5
12 -3 12
10 10
8 8
6 6
4 4
2 2
Months 2 4 6 8 10 2 4 6 8 10 2 4 6 8 10 2 4 6 8 10 2 4 6 8 10
Birth 1 year 2 years 3 years 4 years 5 years
Age (Completed months and years)
WHO Child Growth Standards

25. Systemic examination
 CVS  S1 S2 Normal / No murmurs
 RS  NVBS + ; No added sounds
 Abdominal  Liver – 4 cm below RCM
Soft, non tender, Span – 11 cm
Spleen not palpable , No LN mass
 CNS  Unremarkable

26. Database

28. Other possibilities (less likely)
 Nutritional deficiencies
 Acrodermatitis enteropathica
 Essential fatty acid deficiency
 Icthyosis + Erythroderma
 Netherton syndrome
 Conradi –Hunerman syndrome
 DRESS syndrome
 ? Drug induced

29. Biochemistry
20-8-11 23-8-11 27-8-11 01-9-11
Na 133 137 130 137
K 4.9 4.5 4.0 4.2
Cl 99 100 103
Urea 12 15 10 16
Creat 0.3 0.3 0.3 0.4
Ca / P 8.2 / 3.8 8.8/2.9 8.1/4.1
TP/ alb 5.9/2.1 5.9/2.0 5.4/1.8
AST/ALT 102/41 75/37 107/38
ALP 157 161 138
Bil 0.7 0.7 0.7 0.7
Mg 2.3
CRP 49.7 51.73 35.44

30. Hemograms
20-8-11 23-8-11 24-8-11 1-9-11 2-9-11 8-9-11
Hb 9.9 10 9.6 8.1 8.6 7.2
TLC 16000 27700 17100 10200 29000
DLC 50 28 66 68
10 12 6 21
6 5 3 4
34 55 25 7
Plts 552,000 462,000 261000 852000
ESR 52 48 38 58 QNS
AEC 9418 9405 2550 2030

31. Database
HYPER -EOSINOPHILIA INFLAMMATION

32. Work-up to rule out
underlying infections
 Urine R/E (two samples)  WNL
 Stool R/E (two samples)  No ova / cyst
 Blood c/s & Urine c/s  Sterile
 Malaria card test and smears  Negative
 Filarial serology  Negative
 USG Abdomen  No collections
 Mantoux / CXR / GA (AFB)  Negative for TB

34. No evidence of lytic
lesions on skull X ray

36. To Rule out an underlying
Immunodeficiency
 NBT reduction test  Normal
 Immunoglobulin profile
 Ig G  1064 (490 – 1610)
 Ig A  59 (40 – 200)
 Ig M  176 (50 – 200)
 Ig E  1400 (<60)
 Retro test  Negative

37. Initial Treatment
 IV antibiotics ( Ceftriaxone / Amika / Clox)
 Trial of Albendazole
 Supportive care
 Nutritional rehabilitation & Supplements
PERSISTENT FEVER SPIKES
NO CLINICAL IMPROVEMENT
WORSENING ERYTHRODERMA

38. Possibility of Idiopathic
Hyper- Eosinophilia
 Dermatology consultation
 Skin biopsy
 Bone marrow biopsy
No evidence of underlying malignancy
Routine Parasitology work up negative
Serology ( Trichinella / Toxocara / Toxopl ) awaited

39. Approach to
Eosinophilia

40. Eosinophil Biology
•Role of IL 5 in Eosinophilopoeisis
•Most eosinophil specific cytokine
•Production / release / tissue accumulation
•Variety of cytokines involved
•Eotaxin 1,2 & 3  most important
•CCR 3 receptor
•Eosin staining granules
•MBP/ECP / EDN /EPO
• Lipid mediators  LT C4 D4 E4
• Chemoattractants and pro-fibrotic molecules

41. How do we define
Eosinophilia ?
 Normal frequency  1-3 %
 AEC > 500/cmm (or 450/cmm)  eosinophilia
 AEC > 1500/cmm  Hyper-eosinophilia

42. How do we classify
Eosinophilia?
 Arbitrary classification
 MILD  AEC 500/cmm to 1500/cmm
 MODERATE  AEC 1500/cmm to 5000/cmm
 SEVERE  AEC > 5000/cmm
Reference : WHO defined eosinophilic disorders –
2011 update on diagnosis, risk-stratification and
management

43. Whenever you face
eosinophilia,
 Always consider
 Degree of eosinophilia
 Location of eosinophilia ( blood / tissue / both)
 Clinical presentation
 Careful history reg
 Symptoms of atopy / gastro-intestinal disease
 Helminth endemicity information
 Drug ingestion
 Systemic symptoms suggesting malignancy

46. WORKING DIAGNOSIS in index case
Idiopathic hyper-Eosinophilia
syndrome
 Does it meet the criteria?
 Old criteria – Chusid et al (1975)
 Newer criteria – WHO
 Types
 Myeloproliferative (m-HES)
 Lymphocytic (L-HES)
 Familial ( f- HES)
 Lymphocytic variant of HES 
 only cutaneous involvement / skin plus one other organ
system
 Elevated Ig E levels
 Risk of subsequent malignancy – T cell lymphomas

47. Double negative T cells
(CD 3+ CD4- CD 8-)
 A clone of lymphocytes described in
lymphocytic variant of HES

48. Therapeutic decision making

49. Treatment strategies
 Etiology specific therapy
 Hyper-eosinophilic states
 Corticosteroids
 PREDNISOLONE  1 mg/kg  slow taper
 Hydroxyurea
 Imatinib ( m-HES)
 Interferon alfa
 Mepolizumab

50. Index Case
 Started on Oral steroids at 1 mg/kg/day
 Significant clinical improvement ; activity better
 Erythroderma improving
 No fever
 PLAN  Steroids for 2 wks  Taper over 2-3
months
 Routine follow up ; Decide need for step-up

51. TAKE HOME MESSAGE
 Try to approach eosinophilia systematically
 Always rule out secondary causes MOST IMPORTANT
parasitic infections
 BUT not every eosinophilia should be blindly given
Albendazole and sent home
 Keep malignancies / immunodeficiency states in
consideration if no other secondary cause found
 Lastly, consider HES and the tissue damage that
Eosinophils may be producing
 Decide regarding appropriate therapy

52. THANK YOU

53. QUERIES ?