Jayakar Thomas et al. / International Journal of Dermatopathology And Surgery, 2016; 2(1): 8-10.

e - ISSN - 2455- 247X

International Journal of Dermatopathology &
Surgery

Journal homepage: www.dermatopathologyjournal.com

ACUTE GENERALISED PUSTULAR PSORIASIS OF VON

ZUMBUSCH – A RARE CASE REPORT
Jayakar Thomas1, ShriSindhuja T2, Chinthaamani KPR2, Manoharan D3
1
Professor & Head, 2Junior Residents, 3Professor, Department of Dermatology, Venereology & Leprosy,
Sree Balaji Medical College & Bharath University, Chennai 600044, Tamil Nadu, India.

Corresponding Author: - Jayakar Thomas

E-mail: jayakarthomas@gmail.com

Article Info ABSTRACT

Received 15/12/2015 Von Zumbusch is the most severe form of generalized pustular psoriasis (GPP) that usually starts as
Revised 27/12/2015 multiple erythematous, tender plaques later becoming studded with tiny sterile pustules that coalesce
Accepted 02/01/2016 to form lakes of pus. It can precede the onset of psoriasis or can be a continuum of the disease. We
report a case of acute generalized pustular psoriasis of Von Zumbusch in a 30year old Asian male
Key words: Von who presented with acute onset of reddish raised lesions associated with pustules all over the body.
Zumbusch, sterile
pustules, acute
generalized pustular
psoriasis

INTRODUCTION
Generalized pustular psoriasis is an extreme form
of psoriasis that presents with fiery erythematous plaques
studded with pustules all over the body. It is classified as:
Acute generalized pustular psoriasis of Von Zumbusch,
Generalized pustular psoriasis of pregnancy, Infantile and
juvenile type, Circinate and Localized (not hands and feet)
(Griffiths & Barker, 2008). Von Zumbusch, a type of
generalized pustular psoriasis is named after a
dermatologist from Germany, Leo Ritter von Zumbusch,
who first described the earliest recorded case of
generalized pustular psoriasis in 1910 (Sandra Philipp,
2014). It is more commonly seen in adults and the disease
runs a more benign course in children. The etiology is
unknown but genetic correlation with HLA-B27 has been
well established in the past. Clinically it is characterized by
fever that lasts for several days associated with sudden
generalized eruption of sterile pustules of size 2-3 mm in
diameter distributed all over the body including the nail
bed, palms and soles.
Case Report: A 30 year old male came to our OPD with
complaints of reddish raised lesions associated with
pustules all over the body for the past 2 weeks. There was
history of fever following which he developed pus filled
lesions over the right leg which later progressed to involve
all over the body. He had a similar episode 2 years back,
for which he had been hospitalized. He had been diagnosed
with psoriasis vulgaris 10 years ago and has been on T.
Methotrexate for the past 2 years. History of seasonal
variation was present (lesions aggravated in the winter
season). There was no history of drug intake, topical
application prior to the onset of the lesions. No history of
itching, oliguria, jaundice or joint pain.
Dermatological examination revealed multiple
erythematous scaly plaques with pustules studded over the
periphery of the plaque distributed all over the body. Few
plaques coalesce to form larger plaques. Sparing of palms,
soles and face noted. Scalp, nails and oral mucosa were
normal.
Systemic examination revealed normal study.
Complete blood analysis done was normal. Punch biopsy
taken from the site of the lesion revealed hyperkeratosis,
extensive neutrophilic infiltrates in the subcorneal layer
with spongiotic pustules.

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 Jayakar Thomas et al. / International Journal of Dermatopathology And Surgery, 2016; 2(1): 8-10.
Figure 1 & 2. show clinical photographs of pin point pustules lining erythematous pigmented plaques over the trunk
and upper limb
Figure 3. shows neutrophilic infiltrates forming
Spongiform pustule of Kogoj in the spinous layer
DISCUSSION
Von Zumbusch, a type of acute generalized
pustular psoriasis is of two types. In the first, typical
psoriasis of early onset progresses to pustular psoriasis
after some years often provoked by withdrawal of steroids
or other factors and the second type where the onset of
psoriasis occurs later in life and is usually atypical, acral or
flexural in distribution (Harvey Baker & Terence J. Ryan,
1968). Attacks are characterized by fever that last several
days followed by a sudden generalized eruption of sterile
pustules which occurs all over the body including the nail
bed, palms and soles. The pustules usually arise on the
highly erythematous skin lesions and then become
confluent as the disease becomes severe leading to
erythroderma. Triggering factors are irritant topical therapy
such as coal tar, anthralin (Raghavendra Rao et al., 2007)
and drugs such as sudden withdrawal of steroids, iodide,
lithium, progesterone and terbinafine. Involvement of oral
mucosa is not uncommon, with the tongue appearing
clinically and histologically synonymous with geographic
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Figure 4. shows hyperkeratosis with extensive
neutrophilic infiltrates in the stratum corneum forming
Munro’s microabscess, and regular elongation of rete
ridges
tongue. Nails may become hyperkeratotic or become
separated by subungual lakes of pus and shedding of the
nail soon follows. In the absence of effective treatment,
death can occur in the acute stage. Complications linked
with acute generalized pustular psoriasis are
hypoalbuminaemia due to sudden loss of plasma protein
into the tissues, followed by hypocalcaemia. Oligemia may
cause acute and fatal renal tubular necrosis. Staphylococcal
infection may complicate the course of the disease.
Inflammatory polyarthritis is common. When the disease
lasts for a long period, generalized hair loss may occur.
Histopathology study reveals typical features of psoriasis
accompanied by spongiosis and pooling of numerous
neutrophils in the intraepidermal zone. Laboratory findings
in acute generalized pustular psoriasis of Von Zumbusch
include absolute lymphopenia at the onset of the disease,
followed by polymorphonuclear leukocytosis. ESR is
usually raised. Plasma albumin, zinc and calcium may be
low partially due to malabsorption. GPP has to be
 Jayakar Thomas et al. / International Journal of Dermatopathology And Surgery, 2016; 2(1): 8-10.

differentiated from subcorneal pustular dermatosis of
Sneddon and Wilkinson (a variant of IgA pemphigus)
wherein, sterile pustules have predilection for flexures,
axillary and inguinal folds and commonly seen in women.
Although subcorneal pustules are seen in both conditions,
spongiform pustules are seen only in pustular psoriasis
(Amanda Robinson et al., 2012).Successful treatment
modalities include withdrawal of provocative factors,
acitretin, dapsone, methotrexate, cyclosporine, colchicine,
biologic agents such as basiliximab (Salim et al., 2000)
and phototherapy (PUVA & NB-UVB) (Herbert
Honigsmann et al., 2000). Systemic steroids are used only
as a lifesaving measure in case of crisis.
CONCLUSION
Acute generalized pustular psoriasis is a rare but
severe type of pustular psoriasis distinctly featured by
sterile spongiform pustules, generalized distribution, and
systemic symptoms. Timely monitoring and sufficient
management is warranted. When untreated or inadequately
treated, can be fatal.
ACKNOWLEDGEMENTS: None.
CONFLICT OF INTEREST:
The authors declare that they have no conflict of interest.
STATEMENT OF HUMAN AND ANIMAL RIGHTS
All procedures performed in human participants
were in accordance with the ethical standards of the
institutional research committee and with the 1964
Helsinki declaration and its later amendments or
comparable ethical standards. This article does not contain
any studies with animals performed by any of the authors.

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