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REVIEW ARTICLE

Parakeratosis pustulosa: A diagnostic conundrum

Vikram K Mahajan, Nitin Ranjan
Department of Dermatology, Venereology and Leprosy, Dr. R. P. Govt. Medical College, Kangra (Tanda), Himachal Pradesh,
India

ABSTRACT

Parakeratosis pustulosa (PP) is not so rare an entity and remains under reported in the literature perhaps for the simple reason
of mistaken identity. The literature on PP was reviewed for epidemiology, etiology, clinicopathologic features, differential
diagnosis, clinical course, and the therapeutic measures described. A Medline search (PubMed, Google, IndMed) carried
out on 30 December 2012 revealed only 10 reports under the heading parakeratosis pustulosa; three were in languages
other than English. This chronic dermatosis is characterized by erythemato-squamous lesions affecting the periungual skin/
nail unit in children aged 5 years, mostly females. Nails show pitting and cross-ridging of one or more digits especially
of the fingers. Its etiology remains obscure, and onychomycosis, psoriasis or pustular psoriasis, and atopic or contact
dermatitis affecting the nail are important differentials. Acrodermatitis continua of Hallopeau remains the most common
misdiagnosis in these patients. Topical emollients remain the recommended treatment, while topical corticosteroids or
tretinoin are needed for recalcitrant cases. Evaluation of this as a distinct entity having benign, self-limiting clinical course
and good prognosis will save the patient from expensive therapy and unnecessary distress associated with other more
mutilating pustular dermatoses affecting the nail unit.

Key words: Acrodermatitis continua of Hallopeau, acropustulosis, onychodystrophy, onycholysis, pustular psoriasis

INTRODUCTION not so uncommon disorder for accurate diagnosis and

uniform documentation. In this communication, we

P arakeratosis pustulosa (PP; Syn: HjorthSabouraud

syndrome) is a chronic dermatosis characterized
by erythemato-squamous lesions affecting periungual
skin with pitting and cross-ridging of the nails of one
or more digits, almost with exclusivity in females
aged 5 years. It was first described by Brocq as
parakeratosis psoriasis formes, but the first report
on PP appeared in 1931 under the nomenclature
Parakratose microbinne du bot des doigts.[1,2]
However, Hjorth and Thomsen[3] are credited to
bring forth this entity in English literature under
the heading parakeratosis pustulosa. However, it
remains under reported in the literature perhaps for
the simple reason of mistaken identity. Its treatment
as well as status as an independent entity also remains
unclear. In view of the foregoing, we feel that there
is a strong need to delineate this rarely reported but
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DOI:
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briefly review the literature for a simplified clinical
description of this clinically less-familiar disease to
compare the features of other closely mimicking
dermatoses and to propose the clinical criteria for
diagnosis.
REVIEW OF LITERATURE
A Medline search (PubMed, Google, IndMed) carried
out on 30 December 2012 revealed only 10 reports
under the heading parakeratosis pustulosa; three were
in the languages other than English.[2-11] These were
analyzed for epidemiology, etiology, clinicopathologic
features, differential diagnosis, clinical course, and
therapeutic measures described.
Epidemiology
Its prevalence remains unknown as it is inadequately
described, often overlooked or misdiagnosed as
ADDRESS FOR CORRESPONDENCE
Dr. Vikram K Mahajan
Department of Dermatology, Venereology and Leprosy,
Dr. R. P. Govt. Medical College, Kangra (Tanda) 176 001,
Himachal Pradesh, India.
E-mail: vkm1@rediffmail.com

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Mahajan and Ranjan: Parakeratosis pustulosa
psoriasis, eczema, acrodermatitis continua, atopic
or contact dermatitis, and perhaps remains under
reported in the literature for the simple reason of this
mistaken identity.
Etiology
Its etiology remains obscure. Absence of PP in
siblings/family members suggests that it is not
genetically transmitted. It has often been related
with psoriasis, chronic dry fissured eczematoid
dermatitis, or onychomycosis. Hjorth and Thomsen[3]
reviewed 91 cases and noted personal or family
history of psoriasis in 12 and atopic dermatitis in
16 patients, while the search for fungus was futile
in 20 out of 36 patients examined. In a long-term
follow-up (1-9 years) of 20 patients, Tosti et al.[7]
observed psoriasis lesions over scalp, left dorsal foot,
and inguinal folds, either at first presentation (three
patients) or as subsequent development of psoriasis/
pitting of nails (five patients). They could also confirm
presence of atopic or contact dermatitis in six other
patients. Pandhi et al.[9] implicated thumb sucking as
a causative factor. However, a benign clinical course,
spontaneous remissions, and improvement with
emollients alone in most patients suggest that PP is a
distinct entity whose etiology remains obscure.
Clinical Features
Much of the clinical profile of PP described by various
authors is based on an elucidating review of 91 cases
by Hjorth and Thomsen.[3] The disorder occurs almost
exclusively in children aged 5 years and mostly
affects young girls. Thumb or middle finger is affected
more often than the toes. The lesions start close to the
free end of the nail as few pustules or vesicles in about
25% cases, evolving into more eczematoid changes
over the adjacent skin.[4] Pink or skin-colored and
sharply delineated plaques are densely studded with
scales [Figure 1]. In long-standing cases, peripheral
collarets of scales may be seen. In most cases, only the
tips of digits are affected and the skin changes may
extend on to the nail folds or dorsal side of the digits.
The distal nail plate shows onycholysis usually at the
corner. Though uncommon, subungual hyperkeratosis,
when occurs, rarely extends beyond 1-2 mm into
the nail bed.[4] Thickening and/or pitting of nail are
uncommon, but ridging is usual. The distal pulp may
be normal or may show mild erythema and scaling.
However, pain and itching are conspicuously absent.[3]
Briefly, erythema and scaling of periungual skin are
the predominant features while pustule formation may
occur only in the beginning. Subungual hyperkeratosis
results in lifting up of the nail plate from the nail bed
causing deformity resembling onycholysis.
Indian Journal of Paediatric Dermatology | Vol 15| Issue 1 | January-April 2014
Diagnostic Methods
The diagnosis remains clinical and is made best by
exclusion. The proposed clinical criteria [Table 1] will
assist in making early clinical diagnosis and uniform
documentation until its status as an independent entity
or otherwise becomes clear. Histopathologic studies
are sparse. Only three reports describe the histologic
features similar to chronic eczema or psoriasis.[5,6,9] As
early as 1974, de Dulanto et al.[5] reported the histology
of one case and observed hyperkeratosis, parakeratosis,
pustules, acanthosis, mild exocytosis, papillomatosis,
and polymorpholymphocytic infiltrates. Later,
Avci et al.[6] additionally noted many dyskeratotic cells
in stratum spinosum. Pandhi et al.[9] also described
hyperkeratosis, parakeratosis, mild acanthosis, and
papillomatosis with infiltrate around the dilated blood
vessels. These histologic features of hyperkeratosis,
parakeratosis, mild acanthosis, papillomatosis, and
polymorpholymphocytic infiltrate described in a
few cases are nonspecific or similar to those seen in
Figure 1: Parakeratosis pustulosa in a 6-month-old child. Erythematous,
sharply delineated plaque densely studded with thin scales is present
predominantly over pulp of the digit and nail fold of the right thumb. Also
note minimal onycholysis and no nail dystrophy/pitting
Table 1: Proposed diagnostic criteria for parakeratosis
pustulosa
Major criteria
Patients are children, particularly young girls aged5 years
Benign course and spontaneous remissions
Other dermatoses are excluded by adequate means, especially if
there is no evidence of fungal elements by KOH examination/culture
Minor criteria
Absence of similar disease in family members
Rare and transient pustulation, not extending beyond the initial
phase of the disease
Response to topical emollients
Nonspecic histologic features
In a patient having sharply demarcated asymptomatic, erythematous, scaly
lesions affecting the periungual skin of one or more digits associated with lateral
onycholysis and ridging of nails, all of the following three major and any three
minor criteria are sufficient for a diagnosis of parakeratosis pustulosa
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Mahajan and Ranjan: Parakeratosis pustulosa

psoriasis or chronic eczema, and may not be helpful
in diagnosis. Routine laboratory investigations also
have no significant abnormality. Most investigative
workup recommended is largely for exclusion of other
dermatoses.
Differential Diagnosis
PP needs to be differentiated from other
dermatoses affecting the nail unit, such as psoriasis,
acropustulosis (pustular psoriasis and acrodermatitis
continua of Hallopeau), atopic or contact dermatitis,
onychomycosis (dermatophytic/non-dermatophytic),
and chronic (mostly candidal) paronychia.
Differentiation of various pustular dermatoses
affecting the nail unit is perhaps more important for
better therapeutic outcome. Notably, psoriasis/pustular
psoriasis and acrodermatitis continua of Hallopeau,
which usually are more chronic and incapacitating
than PP, may cause diagnostic confusion. Important
differentiating features of these are listed in Table 2.
Acrodermatitis continua of Hallopeau sometimes can
be very aggressive resulting in resorptive osteolysis
of digits, but is mostly less mutilating than pustular
psoriasis of the nail unit which is a more destructive
form of acropustulosis that may cause nail loss in
the long term.[12] In contrast, pustules are rare and
transient in PP and seen in the initial stages only.
Development of sheets of coarse scales is seen more
with psoriasis-associated PP-like lesions.[3] Presence of
psoriasis lesions elsewhere on the body in such cases
will help in the diagnosis and must be looked for. Fungal
infections affecting the nail units, however, need not
be confused with PP and should be excluded by KOH
preparation, fungal culture, and/or biopsy with special
stains for fungus. Chronic paronychia mostly affects
adults who are diabetic or whose hands are exposed
to wet work (housewives, dish washers, bar tenders,
laundry or canteen workers). The involved nails show
loss of cuticle, erythematous, swollen, and mildly
tender nail folds, and pus formation underneath.
Nail discoloration and dystrophy is frequent in
long-standing cases. Atopic or contact dermatitis can
be diagnosed clinically (itchy, oozing lesions) or by
patch testing with suspected allergens, respectively.
Moreover, pustulation is not a regular feature here,
not even a transient one.
Management and Prognosis
No specific therapy has been recommended. Though
topical corticosteroids have been used in most reports,
topical emollients remain the recommended mode
of treatment. Few patients may need treatment with
topical tretinoin. It has a benign and non-mutilating
clinical course and prognosis is good. The onset is
insidious and it usually regresses as the child grows
up. A prolonged clinical course is often marked with
spontaneous but unpredictable remissions, while
recurrences are the rule. Children who develop
nail psoriasis during clinical course perhaps had
acrodermatitis continua/psoriasis in the beginning.

Table 2: Comparative features of parakeratosis pustulosa and other pustular dermatoses affecting the nail unit
Features Parakeratosis pustulosa Acrodermatitis continua of Pustular psoriasis of nails Psoriasis of nails
Hallopeau
Age of onset/sex Young children usually5 years May occur in children and is rare Any age, and rare in Any age, less common in
of age, mainly females in young adults. More common in children. Occurs in either children. Occurs in either
females sex as a part of more sex mostly as a part of
generalized disease more generalized disease
Clinical course Benign, painless, clinical course Aggressive. Sometimes mutilating, Aggressive and often Benign, painless
is usually chronic, recurrences painful, and progresses to mutilating
are often generalized pustular psoriasis
Spontaneous Usual Uncommon Not as a rule Not as a rule
remissions
Digital osteolysis Not seen Occurs in aggressive form Not seen Not seen
Nail Deformity Lateral onycholysis and ridging Distal onycholysis and subungual Onycholysis may be Subungual hyperkeratosis,
are common. Pitting and hyperkeratosis are common. Nail proximal. Commonly characteristic nail pitting,
subungual hyperkeratosis are loss and dystrophy occur often due associated with nail loss. onycholysis, and nail
uncommon to pustules in nail bed Nail thickening is usual dystrophy are frequent.
Oil-drop sign is more
specic
Pustular lesions Rare or transient at onset Common during clinical course Common in nail bed or Not a feature
matrix during clinical course
Scaling Scaling occurs in some cases Scaling occurs mostly over tips of Not seen Seen over periungual skin
digits/free end of nail at any stage when involved
Skin lesion of Not seen Not seen Skin lesions of pustular Skin lesions of plaque
psoriasis psoriasis psoriasis
Histopathology Nonspecic and variable. Mostly Features are of pustular psoriasis Features are of pustular Features are of psoriasis
mimics eczema psoriasis

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Mahajan and Ranjan: Parakeratosis pustulosa
Unresolved Questions
Its treatment as well as status as an independent entity
remains unclear. According to Richert and Andre,[13] PP
evolves toward complete healing within a few months/
years without any sequelae or frank psoriasis in some
patients. However, it may be appropriate to agree to
the opinion of Tosti et al.[7] that the definite diagnosis
of PP is based on the location and features of the
nail abnormalities that make the nail symptoms very
distinctive, and finally should rest upon dermatological
evaluation, patch testing, and long-term follow-up,
i.e. exclusion of other dermatoses affecting the nail
unit (vide supra). Acrodermatitis continua of Hallopeau
is the most common disorder mistaken for PP as is
evident from the images accompanying some published
reports. For instance, the patient described by Park
et al.[11] who had spontaneous nail loss, erythematous
scaly patch with pustule formation, and clearance with
systemic etretinate appears to be of acrodermatitis
continua of Hallopeau. Similarly, PP described in
patients as old as 18 years of age appears ambiguous.[7]
CONCLUSION
The disorder appears rare largely due to unfamiliarity
and infrequent reporting in the literature. We suggest
that PP should be considered as a distinct entity
affecting the nail unit in children 5 years and should be
evaluated as such. Also, all those cases with identifiable
etiologic factors need not be labeled as PP. Histologic
features of psoriasis/pustular psoriasis (periungual skin
biopsy may not always be possible in child patients) or
concurrent presence of psoriasis lesions will exclude a
diagnosis of PP. This will save the patient/parents from
unnecessary distress and expensive therapy associated
with more mutilating dermatoses of the nail unit.
Indian Journal of Paediatric Dermatology | Vol 15| Issue 1 | January-April 2014
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How to cite this article: Mahajan VK, Ranjan N. Parakeratosis pustulosa:
A diagnostic conundrum. Indian J Paediatr Dermatol 2014;15:12-5.
Source of Support: Nil, Conflict of Interest: Nil
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