Clinical dermatology X Concise report

Neonatal eosinophilic pustular folliculitis

D. A. Buckley, S. E. Munn and E. M. Higgins
Department of Dermatology, King's College Hospital, London, UK

Summary Eosinophilic pustular folliculitis (EPF) of infancy is a rare disorder which may
begin in the neonatal period and cause considerable parental anxiety. It must be
distinguished from other causes of a pustular eruption in neonates, including
infection and erythema toxicum neonatorum, and rare disorders such as transient
neonatal pustular melanosis, infantile acropustulosis and Langerhans' cell
histiocytosis. Skin smears and occasionally skin biopsy may be necessary to
reach a diagnosis. We report a case of a Caucasian child with an unusually early
onset of EPF in the first day of life. We wish to emphasize the importance of
recognizing this self-limiting condition in order to prevent inappropriate
antimicrobial treatment.

and eosinophils. A scalp biopsy was taken to exclude

Report
A female infant, the child of a Columbian mother
and an English father, was noted to have a pustular
eruption on the scalp, trunk and limbs shortly after
birth. Her full blood count was normal and she was
otherwise well. On day 4 a presumptive diagnosis of
folliculitis was made and intravenous penicillin was
administered for 48 h without response. At this time
a dermatological opinion was sought. On examina-
tion there were widespread crusted papules involving
the scalp (Fig. 1a), trunk (Fig. 1b) and limbs. The
flexures and acral sites were uninvolved and general
examination was normal.
Erythema toxicum neonatorum and bacterial
infection were thought unlikely in view of the
distribution and persistence of the rash and failure
to respond to antibiotic treatment, respectively.
Immediate Gram stain of a blister fluid smear was
negative. Cytology of a smear showed neutrophils
Correspondence: D. A. Buckley, Department of Dermatology, Royal Free
Hospital, Pond Street, London NW3 2QG, UK. Tel.:144 207 7940500, extn
4384. Fax: 144 207 8302247.
E-mail: dbuckley@doctors.org.uk
Accepted for publication 9 January 2001
Langerhans' cell histiocytosis (LCH). Histology
showed a dense inflammatory infiltrate around hair
follicles, composed mainly of eosinophils (Fig. 2a),
subcorneal pustules (Fig. 2a) and eosinophil infiltra-
tion of the epidermis with associated spongiosis
(Fig. 2b). These findings were consistent with eosi-
nophilic pustular folliculitis (EPF) of infancy. Full
blood count was not repeated to obtain a differential
white cell count as this was felt to be unjustified.
The parents were reassured of the benign nature of
this condition and the child was discharged from
hospital. The rash gradually settled over a 6-month
period and her general practitioner reports that she
remains well at 3 years of age.
EPF is a rare self-limiting disorder of unknown
aetiology, characterized by recurrent pruritic vesico-
pustular lesions on an erythematous base. It was
first described by Ofuji et al. in 1970 as a condition
affecting the face, trunk and limbs of Japanese
adults.1 The first case in an infant was described by
Lucky et al. in 19842 and to date there have been
38 cases reported in infants aged , 1 year; in nine
of these EPF was present in the first 24 h of life
(including this one).2±14 In addition, one HIV-related
case has been reported in an infant with onset at
3 months of age.15 It has been suggested that the

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 Neonatal eosinophilic pustular folliculitis X D. A. Buckley et al.
disease in infants is distinct from that in adults as
Neutrophilic intracorneal
and subcorneal pustules

Eosinophilic spongiosis;
subcorneal eosinophilic

containing neutrophils;
Eosinophilic folliculitis;

the distribution and configuration of lesions is

occasional eosinophils

eosinophilic folliculitis
electron microscopy
Birbeck granules on

subcorneal pustules;
Subcorneal pustules

different.7 In adults, lesions often have an annular

Langerhans'cells;

configuration, occur mainly on the face, trunk and

proximal limbs and may involve the palms and
Infiltrate of
Histology

pustules

digits. In infants the lesions exhibit secondary

crusting and occur predominantly in a perifollicular
distribution on the scalp with some involvement of
the trunk and occasionally the extremities.
Neutrophils,

Neutrophils,

Eosinophils
Eosinophils

eosinophils

eosinophils

Histiocytes
occasional
occasional

The histological findings may also differ between

Tzanck

adults and infants. Common to both is a striking

smear

infiltrate of eosinophils in the epidermis with

spongiosis, subcorneal pustules and a dense dermal
some patients

perifollicular infiltrate of eosinophils, histiocytes and

outbreaks in
eosinophilia

Up to 15%

May occur

May occur

lymphocytes. However in adults the infiltrate invades

Peripheral

During

sebaceous glands and the outer root sheath, often

leading to spongiosis of the outer root sheath.7 A
No

Tzanck smear shows numerous eosinophils and

recur for 2 months

Gram staining is negative for bacteria. In some

Cyclical outbreaks
7±10 days, crops

Varies depending

for 3 months to

cases a peripheral eosinophilia is found during

Resolves within

Resolves within

involvement

outbreaks. EPF of infancy is more common in

on systemic
Lesions last

to years
Duration

males and follows a cyclical course with a total

1 week

5 years
weeks

duration of 3 months to 5 years. Treatment is

largely unhelpful but topical steroids may reduce
pruritus.
Birth272 h

Birth224 h

The importance of EPF in neonates is in

period or
Neonatal

Birth or

Birth or

distinguishing it from other disorders causing a

Onset

later
later

later

pustular eruption in infancy (Table 1). The com-

monest of these is erythema toxicum neonatorum
Vesicles, pustules,

Papules, pustules,

which affects about one-third of infants and is

vesicles, pustules
Pruritic papules,

pustules, crusts
vesicles, crusts

generally recognized by parents, midwives and

pigmented

paediatricians. Poorly defined erythematous macules,

Table 1 Clinical features of noninfective pustular eruptions in neonates.

pustules
Macules,

Vesicles,
macules
vesicles,
Lesions

progressing in some cases to vesicles or pustules,

develop after 24±72 h of life (occasionally from
birth) on the forehead, face, chest, trunk and
extremities. Lesions disappear in a matter of hours
Scalp, flexures
thighs, palms,
Neck, trunk,

Scalp, trunk
Face, trunk,

face, trunk)
Hands, feet

and the eruption resolves within a week. Biopsy is

(^ hands,
(^ scalp,

rarely necessary, as it is generally easily distin-

limbs

soles

feet)
guished from EPF by the lack of crusting and scalp
Site

involvement; when it is carried out the histological

findings are similar to those of EPF and erythema
infants; 0.1±0.3%
of white infants

toxicum neonatorum is distinguished by its self-

in black males
One-third of

4±5% black

Rare; mainly
limiting course.
Incidence

neonates

Transient neonatal pustular melanosis is char-

acterized by the evolution of vesicopustules with-

Rare

Rare
out surrounding erythema into postinflammatory
pigmented macules with a surrounding collarette
pustular melanosis
Transient neonatal
Erythema toxicum
of scale. The macules fade spontaneously within

Langerhans cell
acropustulosis
3±4 weeks. The scalp is rarely involved and both
neonatorum

histiocytosis

Eosinophilic
Tzanck smear and histology show a predominance
Diagnosis

folliculitis
Infantile

pustular
of neutrophils. The dermis is generally uninvolved
histologically although late lesions may show
252 q 2001 Blackwell Science Ltd X Clinical and Experimental Dermatology, 26, 251±255

Figure 1 (a) Crusted papules scattered throughout the scalp (day 7). (b) Crusted papules and pigmentation on the abdomen
(day 7).
dermal melanophages. Some cases bear clinical
and histological similarities to erythema toxicum
neonatorum.
Infantile acropustulosis is a relatively rare disorder
which may have its onset in the neonatal period and
persist throughout infancy and early childhood.
Intensely pruritic crops of small red papules on the
hands and feet evolve within 24 h into large vesicles
and pustules, several mm in diameter. Occasionally
the scalp, face and trunk may be affected and some
authors consider this to be a variant of EPF.14 A
Tzanck smear shows predominantly neutrophils,
histopathology shows subcorneal pustules containing
neutrophils and occasional eosinophils and there may
be dermal oedema and a perivascular lymphocytic
infiltrate.
LCH may present at birth and is characterized
by papules and vesicles with crusting. The scalp
and flexures are sites of predilection but any site
may be affected. In the benign congenital
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Neonatal eosinophilic pustular folliculitis X D. A. Buckley et al.
self-healing type of LCH (Hashimoto-Pritzker var-
iant) there is no organ involvement, the infant is
otherwise well and lesions involute spontaneously.
Histology shows an infiltrate of Langerhans' cells
positive for markers such as CD1a and S100;
electron microscopy shows the characteristic Bir-
beck granules.
This case of EPF is unusual in its early age of
onset and occurrence in a female infant. Lack of an
accurate diagnosis led to unnecessary antibiotic
therapy. Most cases of pustular eruptions in neo-
nates are due to benign and self-limiting conditions.
Accurate distinction of more significant disorders is
generally possible on clinical grounds with the aid of
a skin smear for Gram staining and a Tzanck smear
for cytology. Occasionally a skin biopsy is necessary.
Prompt referral of neonates with pustular eruptions
to a dermatologist will generally allow parental
reassurance and prevent inappropriate and futile
treatment.
253
Neonatal eosinophilic pustular folliculitis X D. A. Buckley et al.
References
1 Ofuji S, Ogino A, Horio T et al. Eosinophilic pustular
folliculitis. Acta Derm Venereol (Stockh) 1970; 50: 195±
203.
2 Lucky AW, Esterly NB, Heskel N et al. Eosinophilic pustular
folliculitis in infancy. Pediatr Dermatol 1984; 1: 202±6.
3 Picone Z, Madile BM. Eosinophilic pustular folliculitis
(Ofuji's disease) in a newborn. Pediatr Dermatol 1992; 9:
178.
4 Onorato J, Laude T. Infantile eosinophilic pustular follicu-
litis. Pediatr Dermatol 1993; 10: 292±4.
5 Taieb A, Bassan-Andrieu L, Maleville J. Eosinophilic
pustulosis of the scalp in childhood. J Am Acad Dermatol
1991; 27: 55±60.
6 Khorshid SM, Glover M, Cerio R. Infantile eosinophilic
254
Figure 2 (a) Perifollicular infiltrate with
predominance of eosinophils, and
subcorneal eosinophilic pustules
(haematoxylin & eosin  40). (b)
Eosinophilic infiltration of the epidermis
with associated spongiosis
(haematoxylin & eosin  100).
pustular folliculitis: report of the first British case. Eur J
Dermatol 1997; 7: 385±7.
7 Darmstadt GL, Tunnessen WW, Sweren RJ. Eosinophilic
pustular folliculitis. Pediatrics 1992; 89: 1095±8.
8 Boone M, Dangoisse C, Andre J et al. Eosinophilic pustular
folliculitis in three atopic children with hypersensitivity to
Dermatophagoides pteronyssimus. Dermatology 1995; 190:
164±8.
9 Garcia-Patos V, Pujol RM, de Moragas JM. Infantile
eosinophilic pustular folliculitis. Dermatology 1994; 189:
133±8.
10 Giard F, Marcoux D, McCuaig C et al. Eosinophilic pustular
folliculitis (Ofuji's disease) in childhood: a review of four
cases. Pediatr Dermatol 1991; 8: 189±93.
11 Duarte AM, Kramer J, Yusk JW et al. Eosinophilic pustular
folliculitis in infancy and childhood. Am J Dis Child 1993;
147: 197±200.
q 2001 Blackwell Science Ltd X Clinical and Experimental Dermatology, 26, 251±255
 Neonatal eosinophilic pustular folliculitis X D. A. Buckley et al.

12 Dupond AS, Aubin F, Bourezane Y et al. Eosinophilic
pustular folliculitis in infancy: report of two affected
brothers. Br J Dermatol 1995; 132: 296±9.
13 Larralde M, Morales S, Munoz AS et al. Eosinophilic
pustular folliculitis in infancy: report of two new cases.
Pediatr Dermatol 1999; 16: 118±20.
14 Vicente A, Espana E, Idoate M et al. Are eosinophilic
pustular folliculitis of infancy and infantile acropustulosis
the same entity? Br J Dermatol 1996; 135: 807±9.
15 Ramdial PK, Morar N, Dlova NC, Aboobaker J.
HIV-associated eosinophilic folliculitis in an infant. Am J
Derm Pathol 1999; 21: 241±6.

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