ARTICLE
More than a decade ago Lucky et al. [1] reported five infants
with a recurrent cutaneous eruption consisting of pruritic papulopustules
mainly on the scalp but also involving the trunk and extremities. They
proposed that the disease be called infantile eosinophilic pustular folliculitis
(EPF), i.e. a variant of Ofuji's eosinophilic pustular folliculitis
[2] occurring in infancy. More recently, further cases have been described
in the dermatological and paediatric literature having the same clinicopathological
findings [3-9].
We report an infant with a clinicopathological picture similar to those
cited above, which to our knowledge is the first reported case from the
United Kingdom.
Case report
A six month old Caucasian male infant presented at the dermatology clinic
of the Royal London Hospital with a 12 week history of a recurrent, itchy,
pustular eruption. His skin was normal at birth and there was no past
or family history of atopy. At the age of three months, he developed itchy,
yellowish, crusted pustules and follicular papules on the scalp (Fig.
1) with subsequent spread to the trunk. On his back were minute
vesicles and pustules on an erythematous and urticarial base (Fig.
2). The eruption was clearly intermittent with 2-3 week periods
of spontaneous remission and exacerbation. He had been unsuccessfully
treated by his family physician with topical mupirocin, fucidin and systemic
erythromycin. Bacterial and fungal smears and cultures were repeatedly
negative as were KOH preparations for the scabies mite. A full blood count
at the time of exacerbation showed a white cell count of 9,000 with 15%
eosinophils. A skin biopsy from his scalp showed dermal oedema and a superficial
and deep perivascular and periadnexal inflammatory infiltrate, containing
a significant number of eosinophils (Fig.
3A) and concentrating mainly in and around the hair follicles
(Fig. 3B). Specific stains
for bacteria and fungi were negative. These features were consistent with
a diagnosis of an infantile form of eosinophilic pustular folliculitis.
The child was treated with topical 1% hydrocortisone ointment with a very
good response. He continued to have recurrences of the scalp lesions which
were responsive to low-moderate potency topical steroids. The eruption
cleared up completely at the age of 32 months without any residual scarring.
He has not had a recurrence after another six months.
Discussion
In 1970 Ofuji et al. [2] reported three, adult male Japanese
patients with a recurrent, pruritic, follicular, pustular eruption on
the face, trunk and extremities. Histologically, these lesions demonstrated
intense infiltration of the hair follicles with eosinophils. Clinically,
lesions arise on an erythematous base and become confluent to form indurated
and polycyclic plaques with a tendency to central clearing and peripheral
extension. Involvement of the trunk, proximal extremities and face is
common, although the scalp, with or without alopecia, legs, palms and
soles can also be involved. The duration, frequency and extent of the
exacerbations vary widely. Peripheral eosinophilia is a regular feature.
Overall, the disease is uncommon and has been reported mainly in the
Japanese literature. The infantile form of the disease, which was first
reported in 1984 [1], is even rarer. In contrast to the adult form, the
scalp is universally involved in infantile cases. Lesions are inflammatory,
crusted papules with pustules. Involvement of the trunk is not uncommon
and occurs as discrete vesiculopustules on an erythematous base and not
in the form of annular and polycyclic lesions seen in adult form of the
disease [2]. Microscopically, the lesions show an eosinophilic infiltrate
in and around the hair follicles associated with spongiosis and disruption
of the follicular epithelium. Typically, there is a recurrent waxing and
waning course with healing, without scar formation. Peripheral blood leukocytosis
and eosinophilia, especially in association with exacerbation of skin
lesions, have been reported in around 85% of the infants. The disease
is self-limiting in the majority of children and because it may be unrecognised,
it may be commoner than the literature suggests.
The association of adult Ofuji disease and HIV
infection is now well established [10, 11] and probably is an indicator
of a low CD4 count and poor prognosis [12]. Interestingly, HIV-associated
eosinophilic folliculitis is also different from classical Ofuji's disease
both clinically and histologically (Table
I).
Although the origin of the disease is unknown, EPF shares the histological
appearance of erythema toxicum neonatorum [13] and some cases of infantile
acropustulosis [14] and may well represent the persistent form of the
former disorder which is very common during the neonatal period. An interesting
point is the reported association between some cases of EPF, atopic diathesis
[3, 6] and evidence of hypersensitivity to Dermatophagoides pteronyssimus
[8]. Two patients in the Taieb series [4] had relatively high levels
of IgE and another two, low serum IgA. In the series by Lucky et al.
[1] one patient had low levels of IgE and IgA with high IgM and another
patient had a defect of white cell motility. Giard et al. [3] also
reported one patient with reduced numbers of CD8 suppressor lymphocytes
with hyper-reactivity of B-cells and a poor response to mitogens.
Although at the present time the significance
of these immunological findings is not known, the fact that EPF has been
reported in association with HIV infection and especially low CD4 counts
[10-12] and also in patients with systemic lymphoma [15], should alert
us to the possibility of immunological dysfunction in these patients.
The differential diagnoses for infants are many and include scalp impetigo
and bacterial or fungal folliculitis which can be excluded by negative
microbiological investigations. Tinea infection may reproduce the
histological appearance of EPF [16] and can be excluded with specific
stains. Scabies may involve the scalp in infancy and must be seriously
considered. Erythema toxicum neonatorum mimics the changes of EPF clinically
and histologically except that the involvement of the scalp is less common
and the disease is limited to the neonatal period. Infantile acropustulosis
is characterised by vesicle and pustule formation, mainly on the extremities,
with a predominant neutrophilic infiltrate in skin biopsy [17]. Recurrent
pruritic pustules are common features of both infantile acropustulosis
and EPF [18]. These disorders may well represent different clinical expressions,
of the same basic pathophysiology. Transient neonatal pustular melanosis
is usually present at birth with very superficial, predominantly neutrophilic
pustules, peripheral scale and pigmented macules which generally last
for less than 48 h and is limited to the newborn period.
Incontinentia pigmenti, infantile forms of immunobullous dermatoses
and, in particular, Langerhans cell histiocytosis, should also be considered
in the differential diagnosis, as well as congenital syphilis, congenital
and neonatal candidiasis, viral infections especially Herpes simplex
and Varicella zoster and different forms of milliaria which all
have specific diagnostic characteristics.
The response of EPF to therapy is variable. Generally, response to topical
antibiotics has been poor and the reported response to systemic antibiotics,
especially erythromycin, might have been due to the anti-chemotactic activity
of the drug [6]. Response to medium to high potency topical steroids is
usually good [6] but our patient and several others [5] responded completely
to topical 1% hydrocortisone ointment which should, therefore, be considered
as first line treatment. There are several reports of clinical response
to Dapsone in adults. In view of the pruritic nature of this disorder,
systemic anithistamines may be used and the anti-eosinophil migration
drug, cetirizine hydrochloride, may be more successful in the primary
treatment of this disease [12-21]. In adults, systemic steroids [20],
UVB [12] and non-steroidal anti-inflammatory drugs such as indomethacin
[22] have all been tried with variable degrees of improvement.
CONCLUSION
Acknowledgements
The authors wish to thank Ms. Geraldine Garnett-Frizelle for assistance
with the preparation of the manuscript.
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