6 The finding of PDGF-α and c-kit in tissues from KD

patients suggests that imatinib or another tyrosine

kinase inhibitor may be effective in the disease.210

EOSINOPHILIC PUSTULAR
FOLLICULITIS

AT-A-GLANCE
■ Three clinical types that are characterized by
Part 6

follicular papules and pustules, and may involve

the head, trunk, and extremities.
■ Classic eosinophilic pustular folliculitis (Ofuji
::

disease). A
■
Neutrophilic, Eosinophilic, and Mast Cell Disorders

Typically occurs in Japanese patients, who

have chronic, recurrent follicular pustules,
with a tendency to form circinate plaques, in a
seborrheic distribution.
■ Eosinophilic pustular folliculitis associated with
immunosuppression.
■ Most often occurs in patients with human
immunodeficiency virus infection, who have
severely pruritic papules of the face and upper
trunk.
■ Eosinophilic pustular folliculitis of infancy/
neonatal period.
43x05a
■ Follicular pustules of the scalp.
■ Tendency for recurrences and chronicity (except
eosinophilic pustular folliculitis of infancy).
■ Characterized by follicular and perifollicular
eosinophil infiltration.
■ Associated with peripheral blood eosinophilia.

CLINICAL FEATURES B

Figure 40-11 Eosinophilic pustular folliculitis. A, Itchy

At present, eosinophilic pustular folliculitis (EPF) is
reddish papules and pustules on the cheek. B, An HIV-
divided into 3 variants: classical EPF, immunosup- associated case with itchy red papules on the upper arm,
pression-associated EPF, and infantile EPF.211 Immu- shoulder, and back.
nosuppression-associated EPF may be subdivided into
an HIV-associated type and a malignancy-associated
type.212-214 Classical EPF presents as recurrent crops or HIV-associated EPF tends to manifest as extremely
clusters of follicular papules and pustules, which may pruritic discrete follicular papules, typically involving
form an annular pattern and usually resolve in 7 to the head and neck and often the proximal extremities
10 days (Fig. 40-11A). Lesions predominantly involve (Fig. 40-11B). Rosenthal et al emphasized the urticarial
the face and trunk but also may affect the extremities, quality of such lesions.217
with involvement of the palms and soles in approxi-
mately 20% of patients.215 In infantile type of EPF,
lesions typically are located on the scalp, but also may
ETIOLOGY AND PATHOGENESIS
be found on the face and extremities. In some neonates The etiology of EPF remains unknown. The occasional
who have pustular eruptions that clinically resemble association with HIV infection suggests the possible
EPF and typically have peripheral blood eosinophilia, contribution of immunocompromised status to the
the disorder may be classified more appropriately development of EPF. Interestingly, some cases of HIV-
670 under the term eosinophilic pustulosis because the cuta- associated EPF develop lesions after starting highly
neous infiltrates are not folliculocentric.216 In contrast, active anti-retroviral therapy (HAART), indicating that

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 EPF may occur as a result of immune reconstruction
rather than immunodeficiency in these HAART-treated
there is head and neck involvement, and palmar–
plantar pustular psoriasis may also be included in
6
cases.218-220 Drugs,221,222 parasitic or viral infections,223,224 the differential diagnosis when there is hand and
and pregnancy225,226 are also reported as possible fac- foot involvement. Acneiform eruption, rosacea, and
tors related to EPF. The higher expression of ICAM-1 lupus miliaris disseminatus faciei may resemble EPF.
and leukocyte function-associated antigen-1 on the Erythema toxicum neonatorum, acropustulosis, and
follicular epithelium, and VCAM-1 around hair fol- acne neonatorum also should be considered in infants.
licles in EPF lesions, suggest that these molecules are Follicular mucinosis usually is clinically and histo-
relevant to the selective migration of eosinophils and logically distinguishable from EPF. One of the most
lymphocytes to the hair follicles in EPF.227 One study important differential diagnoses is cutaneous T-cell
demonstrated that an interaction between PGD2 and lymphoma, which can resemble EPF both clinically
a chemoattractant receptor-homologous molecule and histopathologically.212
expressed on T-helper type 2 (CRTH2) cells may be
involved in the pathogenesis of classical EPF and
CLINICAL COURSE, PROGNOSIS,

Chapter 40 :: Eosinophilic Diseases

that indomethacin may exert its therapeutic effect by
reducing CRTH2 expression, as well as by inhibiting AND MANAGEMENT
PGD2 synthesis.228
The infantile type of EPF has a good prognosis,
whereas classical and HIV-associated EPF are charac-
DIAGNOSIS terized by recurrences. Postinflammatory pigmenta-
tion may be seen as lesions resolve, but scarring does
Patients suspected of having EPF should be evaluated not occur.
for underlying immune deficiency, particularly HIV Topical glucocorticoids and topical calcineurin
infection. Peripheral blood eosinophilia is a com- inhibitors generally are the first approach to the
ponent of all 3 types of EPF. TARC is reported to be treatment of all types of EPF. Topical tacrolimus is
elevated in serum and to correlate with peripheral helpful for facial lesions.234 Nonsteroidal antiinflam-
blood eosinophilia.229 Although patients with classi- matory drugs, particularly indomethacin, also are
cal EPF usually have eosinophilia with leukocytosis, recommended as first-line therapy; clinical improve-
HIV-positive patients often exhibit eosinophilia with ment may be observed within 2 weeks and is associ-
lymphopenia. Low CD4+ T-cell counts and high IgE ated with a decrease in peripheral blood eosinophil
levels are typical of HIV-associated EPF.217 Elevated counts.235-237 Ultraviolet light therapy (ultraviolet B or
serum IgE is uncommon in infantile EPF.230 Histo- psoralen and ultraviolet A) may be beneficial. Topical
logically, the most striking feature is the infiltration of permethrin, systemic retinoids, systemic glucocor-
eosinophils into hair follicles and perifollicular areas, ticoids, cyclosporine, itraconazole, metronidazole,
sometimes with follicular damage. The infiltrates also cetirizine, minocycline, dapsone, and IFNs have been
may contain lymphocytes and neutrophils, and may tried with success.235,236 Antiretroviral treatment that
be perivascular as well as follicular.231 Flame figures results in increased CD4 cell counts often is associ-
can also be observed in infantile EPF.230 Follicular ated with improvement in HIV-associated EPF. The
mucinosis has been noted in association with EPF232; first-line treatment of infantile EPF is observation or
however, T-cell clonality is not observed in EPF-asso- topical steroid therapy. Infantile EPF tends to resolve
ciated follicular mucinosis.233 spontaneously within several years and usually
shows good response to topical steroids.230
DIFFERENTIAL DIAGNOSIS
Table 40-11 outlines the differential diagnosis of EPF.
Folliculitis secondary to bacterial or fungal infection
PAPULOERYTHRODERMA
must be kept in mind, particularly in immunosup- OF OFUJI
pressed patients. Based on the distribution of lesions,
seborrheic dermatitis should be considered, when
AT-A-GLANCE
TABLE 40-11 ■ Papuloerythroderma tends to occur in elderly
Differential Diagnosis of Eosinophilic males.
Pustular Folliculitis ■ The lesions are usually pruritic.
■
■ Folliculitis, bacterial or fungal Erythroderma-like eruptions formed by
■ Seborrheic dermatitis confluent flat-topped, red-to-brown papules are
■ Palmar–plantar pustular psoriasis characteristic.
■ Acne, including acne neonatorum ■ The unique sparing of the abdominal skin folds is
■ Erythema toxicum neonatorum termed deck-chair sign.
■ Acropustulosis
■ Follicular mucinosis (Continued) 671

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