See discussions, stats, and author profiles for this publication at: https://www.researchgate.

net/publication/272512703

Vesiculobullous Disorders in Children

Article  in  The Indian Journal of Pediatrics · February 2015

DOI: 10.1007/s12098-015-1708-4 · Source: PubMed

CITATION READS

1 1,000

3 authors, including:

Sahana M Srinivas
Indira Gandhi Institute of Child Health
73 PUBLICATIONS   159 CITATIONS   

SEE PROFILE

All content following this page was uploaded by Sahana M Srinivas on 18 November 2015.

The user has requested enhancement of the downloaded file.

Indian J Pediatr (September 2015) 82(9):805–808
DOI 10.1007/s12098-015-1708-4
ORIGINAL ARTICLE
Vesiculobullous Disorders in Children
Sahana M. Srinivas & Preeti K. Sheth & Ravi Hiremagalore
Received: 14 July 2014 / Accepted: 21 January 2015 / Published online: 19 February 2015
# Dr. K C Chaudhuri Foundation 2015
Abstract
Objective To study the frequency and clinical pattern of
vesiculobullous disorders in children.
Methods A retrospective chart review of all children diag-
nosed with vesiculobullous disorders over a period of 36 mo
from January 2011 through December 2013 was performed.
All children 18 y and below were included in the study.
Results A total of 213 children presenting with vesiculo-
bullous lesions were examined during the study period.
Vesiculobullous disorders constituted 3.6 % of the total 5889
dermatoses seen during this period. The most common
vesiculobullous disorder in children was infections (129,
60.6 %), followed by genodermatoses (35, 16.4 %), inflamma-
tory disorders (33, 15.5 %), drug reaction (7, 3.3 %) and trauma
(5, 2.3 %). Autoimmune and metabolic disorders constituted
1.4 % (three children) and 0.5 % (one child) respectively.
Conclusions This study highlights the varied spectrum of
vesiculobullous disorders seen in the pediatric population.
Cutaneous infections and inherited disorders were the most
common disorders observed in the present study.
Keywords Vesiculobullous . Infections . Genodermatoses .
Pediatric age group
Introduction
Vesiculobullous disorders are common in children. Primary
vesiculobullous disorders include vesicles, bullae and pus-
tules. It can either be very benign or potentially fatal in some
cases. They can be either inherited or acquired depending on
the etiology [1]. There are however no studies in the Indian
S. M. Srinivas (*) : P. K. Sheth : R. Hiremagalore
Department of Pediatric Dermatology, Indira Gandhi Institute of
Child Health, Bangalore, Karnataka 560029, India
e-mail: drsahanasrinivas@rediffmail.com
literature describing the epidemiology of vesiculobullous dis-
orders in children. Hence the authors proposed to study the
epidemiological pattern of vesiculobullous disorders in
children.
Material and Methods
This study is a descriptive study, with a retrospective chart
review, comprising of 213 children seen over a period of
36 mo from January 2011 through December 2013. All chil-
dren 18 y and below, with vesiculobullous disorders, attending
the outpatient department of pediatric dermatology at authors’
institution were included in the study. A detailed clinical his-
tory and examination was done in all children and data was
collected on a predesigned proforma and information included
age, gender, age at onset, diagnosis, associated cutaneous and
systemic conditions. Investigations like gram stain, tzanck
smear, KOH examination, skin biopsy, immunofluorescence
and antigen mapping was done when required for confir-
mation of diagnosis. Institutional review board approval
was taken.
Results
A total 5889 children were examined for various dermatolog-
ical problems. Among them 213 children presented with
vesiculobullous disorders thus constituting 3.6 % of the total
dermatoses. The age of presentation ranged from 1 d to 15 y,
mean age of onset being 4.5 y. There were 112(52.6 %) boys
and 101(47.4 %) girls with boys to girls’ ratio of 1.1:1. The
duration of the disease ranged from 1 to 15 d. The most com-
mon group was the infantile age group (53 children, 24.9 %).
The demographic profile of children is described in Table 1.
806 Indian J Pediatr (September 2015) 82(9):805–808

Table 1 Demographic profile of study children Table 2 Various patterns of vesiculobullous disorders in children

Age Boys (%) Girls (%) Total (%) Vesiculobullous disorder n(%)

Neonates (Birth to 28 d) 5(2.3) 8(3.8) 13(6.1) Cutaneous infections 129(60.6)

Infants (1 mo to 1 y) 24(11.3) 29(13.6) 53(24.9) Viral infections 99(46.5)
Toddlers (1 to 3 y) 33(15.5) 17(8) 50(23.5) HFMD 41(19.2)
Preschool (3–5 y) 12(5.6) 14(6.6) 26(12.2) Varicella 25(11.7)
School age (5–11 y) 26(12.2) 25(11.7) 51(24) Herpes labialis 14(6.6)
Adolescents (11–18 y) 12(5.6) 8(3.2) 20(9.4) Herpes zoster 11(5.2)
Herpetic whitlow 2(0.9)
Herpetic gingivostomatitis 2(0.9)
The various vesiculobullous disorders were categorized in- Herpetic genitalis 1(0.5)
to eight groups as shown in Table 2. The most common group Ocular herpes simplex 1(0.5)
was infections (129, 60.6 %) followed by genodermatoses Eczema herpeticum 1(0.5)
(35, 16.4 %) and inflammatory disorders (33, 15.5 %). Drug Chickunguniya rash 1(0.5)
reactions constituted about seven children (3.3 %). Trauma Bacterial infections 29(13.6)
was seen in five children (2.3 %). The least common disorder Bullous impetigo 24(11.3)
seen in the present study belonged to autoimmune (3, 1.4 %)
SSSS 4(1.9)
and metabolic conditions (1, 0.5 %). The various patterns of
Blistering dactylitis 1(0.5)
vesiculobullous disorders in children in the different age
Fungal infections 1(0.5)
groups are described in Table 3.
Congenital cutaneous candidiasis 1(0.5)
Genodermatoses 35(16.4)
Epidermolysis bullosa simplex 21(9.9)
Discussion Junctional epidermolysis bullosa 9(4.2)
Dystrophic epidermolysis bullosa 2(0.9)
Vesiculobullous disorders in children form a challenging do-
LOC syndrome 1(0.5)
main for treating clinicians due its varied presentation. The
Bullous ichthyosiform erythroderma 1(0.5)
pattern of blistering disorders varies in different age groups.
Incontinentia Pigmentii 1(0.5)
The etiopathogenesis of bullous disorders in children differs
Inflammatory dermatoses 33(15.5)
as compared to adults. It is categorized into inherited disor-
Pompholyx 26(12.2)
ders, infectious, inflammatory, autoimmune, reactive and met-
Bullous papular urticaria 3(1.4)
abolic disorders [2]. Majority of children in the present study
Bechets disease 2(0.9)
belonged to infantile (24.5 %) and school going age group (5–
Erythema toxicum neonatorum 2(0.9)
11 y; 24 %). Many studies have shown that skin disorders are
Drug reaction 7(3.3)
more commonly seen in this age group [3, 4].
Steven Johnson syndrome 5(2.3)
In the present study the most common vesiculobullous dis-
Bullous fixed drug eruption 1(0.5)
order was infections as seen in other studies. Infections are the
Bullous drug reaction 1(0.5)
most common pattern documented in various studies ranging
Traumatic 5(2.3)
from 35.6 to 85.2 % [5]. Among infections, viral infections
(46.5 %) were most common followed by bacterial infections Burns 3(1.4)
(36.6 %). Hand foot and mouth disease, varicella and bullous Extravasation injuries 2(0.9)
impetigo accounted 19.2, 11.7 and 11.3 % of the total infec- Autoimmune disorders 3(1.4)
tions. It is well known that children are susceptible to a host of Bullous SLE 1(0.5)
infections. Hand foot mouth disease (HFMD) is a common Bullous pemphigoid 1(0.5)
viral illness and small outbreaks occur more in spring and CBDC 1(0.5)
rainy season at different places. There have been only few Metabolic disorders 1(0.5)
studies in Indian literature regarding the epidemiology of Acrodermatitis enteropathica 1(0.5)
HFMD. Studies have shown HFMD to occur more in children
HFMD Hand foot mouth disease; SSSS Staphylococcal scalded skin syn-
less than 5 y as seen in the index study [6–8]. Varicella is a drome; LOC syndrome Laryngo-onycho-cutaneous syndrome; SLE Sys-
common viral infection seen among children. Herpes zoster temic lupus erythematosus; CBDC Chronic bullous dermatoses of
was seen in 11 children (5.2 %) in the present study. Although childhood
herpes zoster is seen more in adults it is not uncommon in
children. Pediatric zoster is seen in immunocompromised
Indian J Pediatr (September 2015) 82(9):805–808 807

Table 3 Vesiculobullous disorders in different age groups

Diagnosis 0–28 d Upto 1 y 1 to 3 y 3 to 5 y 5 to 11 y 11 to 18 y Total

Hand foot and mouth disease – 16 15 4 5 1 41

Epidermolysis bullosa 8 9 6 5 3 2 33
Pompholyx – 3 8 4 9 2 26
Varicella 1 3 3 3 10 5 25
Bullous impetigo 2 5 7 3 7 24
Herpes labialis – 1 2 3 4 4 14
Herpes zoster – 1 1 7 2 11
Stevens Johnson syndrome – 2 – 1 – 2 5
Staphylococcal scalded skin syndrome – 4 – – – – 4
Bullous papular urticaria – 1 1 – 1 – 3
Burns – 3 – – – – 3
Behcet’s disease – – – – 2 – 2
Erythema toxicum neonatorum 2 – – – – – 2
Extravasation injury – 1 1 – – – 2
Herpetic whitlow – – – – 2 – 2
Herpetic gingivostomatitis – – – 1 – 1 2
Acrodermatitis enteropathica – – 1 – – – 1
Blistering dactylitis – – – – – 1 1
Bullous fixed drug eruption – – 1 – – – 1
Bullous pemphigoid – – 1 – – – 1
Bullous ichthyosiform erythroderma – – 1 – – – 1
Bullous systemic lupus erythematosus – – – – 1 – 1
Chronic blistering disease of childhood – – 1 – – – 1
Chikungunya rash – 1 – – – – 1
Congenital cutaneous candidiasis – 1 – – – – 1
Drug reactions – – 1 – – – 1
Eczema herpeticum – – 1 – – – 1
Herpes genitalis – – – 1 – – 1
Incontinentia pigmenti – 1 – – – – 1
Ocular herpes simplex – 1 – – – – 1

children or those who had primary intrauterine infection or
acute varicella within first year of life or occasionally occurs
without any risk factors [9, 10]. Bullous impetigo is the most
common bacterial infection seen in children less than 10 y of
age as observed in the present study [11].
In the present cohort the second most common
vesiculobullous disorder was genodermatoses.
Epidermolysis bullosa (EB) had a frequency of 15.5 %
of the total dermatoses. Indira Gandhi Institute of Child
Health, Karnataka, being a tertiary care children hospital,
a large number of genetic cases were referred thereby
contributing to its increased frequency. Worldwide data
suggests that there is no gender, racial or geographical
predilection of EB. According to the National EB regis-
try project from USA, the incidence and prevalence rates
of EB simplex are 10.75 and 4.65, of junctional EB are
2.04 and 0.44, and dystrophic EB dominant type 2.86
and 0.99 and recessive dystrophic EB 2.04 and 0.92,
respectively [12, 13].
Among the inflammatory dermatoses, pompholyx consti-
tuted about 12.2 % of the total dermatoses. Banerjee et al. in
their study have reported 0.3 % of the children to have
pompholyx [14]. Pompholyx in children is uncommon in pre-
pubertal or pubertal age and rare in preschool children [15].
Bullous papular urticaria was seen in 1.4 % of total children.
Information regarding prevalence of insect bite reaction is
limited. Various studies have reported the prevalence of insect
bite reaction varying from 5.1 to 10.6 % [16, 17]. Among the
different morphological patterns of insect bite reaction, vesi-
cles and bullous lesions are less common. Kar et al. in his
study found only 5 % of insect bite reactions to have vesicles
and bullae [18].
Drug reactions comprised 3.3 % of the total dermatoses.
Adverse cutaneous drug reactions are quiet frequently
808
observed in children in both outpatient and inpatient settings.
Children are commonly prescribed with antibiotics and anti-
epileptic drugs thus predisposing them to drug reactions [19].
Among drug reactions, Steven Johnson syndrome (SJS) was
seen in 2.3 % cases. A review of world literature of SJS from
1975 to 2003 showed the incidence ranging from 1.1 to 7.1
per million per year [20].
In the present study autoimmune blistering disorders were
noted to occur in three children (1.4 %). Among them one
child had chronic bullous dermatoses of childhood, one had
bullous pemphigoid and the third had bullous SLE. It is a well
known that immune mediated blistering disorders are com-
mon in adults and rarer in children. There is limited data
worldwide regarding the epidemiology of immune mediated
blistering disorders in children. In most children the disease
begins under the age of 4 y [21].
Conclusions
Vesiculobullous disorders are common in children. Their
causes can be varied. Amongst them, cutaneous infections were
the most common cause in the index cohort. Genodermatoses
contributed an important cause of vesiculobullous lesions in
pediatric age group in the present study. Autoimmune disorders
that occur in adults are rarely seen in children.
Contributions SMS: Concept, design, literature search, manuscript
preparation; PKS: Data analysis; RH: Manuscript editing and review.
RH will act as guarantor for this paper.
Conflict of Interest None.
Source of Funding None.
References
1. Wu H, Schapiro B, Harrist TJ. Noninfectious vesiculobullous and
vesiculopustular diseases. In: Elder DE, editor. Lever’s histopatholo-
gy of the skin. Philadelphia: Lippincott, Williams and Wilkins; 2005.
p. 243–91.
2. Rico MJ. Differential diagnosis of vesiculobullous lesions. In: Harper
J, Oranje A, Prose N, editors. Textbook of pediatric dermatology.
Oxford: Blackwell Publishing; 2006. p. 823–30.
3. Sayal SK, Bal AS, Gupta CM. Pattern of skin diseases in pediatric
age group and adolescents. Indian J Dermatol Venereol Leprol.
1998;64:117–9.
View publication stats
Indian J Pediatr (September 2015) 82(9):805–808
4. Sardana K, Mahajan S, Sarkar R, Mendiratta V. The spectrum
of skin disease among Indian children. Pediatr Dermatol.
2009;26:6–13.
5. Karthikeyan K, Thappa DM, Jeevenkumar B. Pattern of pediatric
dermatoses in a referral centre in south India. Indian Pediatr.
2004;41:373–7.
6. KasHyap S, Verma GK. Hand-foot-mouth-disease: outbreak in
Shimla. Indian Pediatr. 2014;51:155.
7. Kar BR, Dwibedi B, Kar SK. Outbreak of hand foot and mouth
disease in Bhubaneswar, Odisha. Indian Pediatr. 2013;50:139–42.
8. Ghosh SK, Bandyopadhyay D, Ghosh A, Dutta A, Biswas S, Mandal
RK, et al. Mucocutaneous features of hand, foot, and mouth disease:
a reappraisal from an outbreak in the city of Kolkata. Indian J
Dermatol Venereol Leprol. 2010;76:564–6.
9. Fabiano V, Dilillo D, Mauri S, Vivaldo T, Gazzarri A, Zuccotti GV.
Herpes zoster in an immunocompetent boy following intrauterine
exposure to varicella-zoster virus. Cutis. 2013;91:127–8, 140.
10. Weinmann S, Chun C, Schmid DS, Roberts M, Vandermeer M,
Riedlinger K, et al. Incidence and clinical characteristics of herpes
zoster among children in the varicella vaccine era, 2005–2009. J
Infect Dis. 2013;208:1859–68.
11. Al-Ghamdi KM. A retrospective study of some clinical and epidemi-
ological features of impetigo patients seen in dermatology clinic in
the eastern province of Saudi Arabia. J Fam Community Med.
2006;13:31–4.
12. Fine JD, Johnson LB, Suchindran C, Moshell A, Gedde-Dahl T Jr.
The epidemiology of inherited epidermolysis bulllosa: findings in the
US, Canadian and European study populations. In: Fine JD, Bauer
EA, Mc Guire J, Moshell A, editors. Clinical, epidemiological and
laboratory advances, and the findings of the national epidermolysis
bullosa registry. Baltimore: John’s Hopkings University Press; 1999.
p. 101–13.
13. Sarkar R, Bansal S, Garg VK. Epidermolysis bullosa: where do we
stand? Indian J Dermatol Venereol Leprol. 2011;77:431–8.
14. Banerjee S, Gangopadhyay DN, Jana S, Chanda M. Seasonal varia-
tion in pediatric dermatoses. Indian J Dermatol. 2010;55:44–6.
15. Gelmetti CM. Pompholyx. In: Irvine AD, Hoeger PH, Yan AC, ed-
itors. Harper’s textbook of pediatric dermatology. Oxford: Blackwell
Publishing; 2011. p. 39.1–39.5.
16. Sacchidanand S, Sahana MS, Asha GS, Shilpa K. Pattern of
pediatric dermatosis at a referral centre. Indian J Pediatr.
2014;81:375–80.
17. Singh S, Mann BK. Insect bite reactions. Indian J Dermatol Venereol
Leprol. 2013;79:151–64.
18. Kar S, Dongre A, Krishnan A, Godse S, Singh N. Epidemiological
study of insect bite reactions from central India. Indian J Dermatol.
2013;58:337–41.
19. Sarkar R, Basu S, Patwari AK, Sharma RC, Dutta AK, Sardana K.
An appraisal of pediatric dermatological emergencies. Indian Pediatr.
2000;37:425–9.
20. Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR,
Foster CS. Steven-Johnson syndrome and toxic epidermal necrolysis
in children: a review of literature. Ann Allergy Asthma Immunol.
2005;94:419–36.
21. Lara-Corrales I, Pope E. Autoimmune blistering diseases in children.
Semin Cutan Med Surg. 2010;29:85–91.