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.
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
15. General examination
Alert , active and interacting well with mother
HEAD to TOE examination
Examination of the skin
16.
17.
18.
19.
20.
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
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
44.
45.
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
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