Skin Diseases First Described by Japanese Dermatologists. Part 1: Papuloerythroderma of Ofuji
Wednesday, January 12, 2011
Papuloerythroderma of Ofuji (PE) was first described as a novel
clinical entity by Professor Shigeo Ofuji, Kyoto University, Japan,
in 1984 as a peculiar form of erythroderma quite different from
ordinary exfoliative dermatitis.1 Initial skin lesions
of PE comprise solid lichenoid papules that demonstrate a
cobblestone-like appearance and develop into erythroderma. Another
important characteristic feature of PE is the so-called "deck-chair
sign", sparing skin folds and creases. As one of the co-authors of
the first report of PE, I witnessed how and why Ofuji considered
these skin manifestations, which did not correspond to any of the
known dermatoses, to be specific. As PE is still underdiagnosed and
poorly recognized in non-Asian countries, this article reviews the
clinical features and possible pathoetiology of PE in order to
improve the world-wide recognition of PE.
Why Is It Named PE?
Ofuji noticed several atypical erythroderma cases that were
considered different from ordinary exfoliative erythroderma because
they began with solid papules that then developed into
erythroderma-like lesions. He documented these fairly
characteristic cases in Japanese journals pre-1984 and confirmed
that nobody had observed or described similar cases before. He then
reported four cases as a possible new clinical entity under the
name of PE in 1984.1
PE starts with the appearance of flat-topped papules that spread
over the body, resulting in a cobblestone-like assembly and
confluence which is not observed in idiopathic exfoliative
dermatitis (Figure 1). The body is finally and extensively
involved, except for the face, forming erythroderma-like lesions.
At this point, several other skin diseases, such as sarcoidosis,
lichen planus and lymphoma, should be excluded. However,
differential diagnosis is not difficult because histopathology
always reveals non-specific perivascular infiltration of
lymphocytes and histiocytes into the upper dermis, with occasional
tissue eosinophilia (Figure 2).
Figure 1. Cobblestone-like assembly of solid
papules as an initial lesion of PE.
Figure 2. Histopathology of PE. Non-specific
perivascular infiltration of lymphocytes and histiocytes is seen in
the upper dermis with tissue eosinophilia (hematoxylin and eosin
In addition, the axillae, inguinal regions, cubital fossae and
the big furrows on the abdomen are clearly spared from the lesions
- later termed the "deck-chair sign" (Figure 3).2 The
mechanism by which the "deck-chair sign" appears still remains
unclear. Possible involvement of ultraviolet light or contact
dermatitis seems unlikely. My simple speculation is that some
"occlusive dressing effect" of topical corticosteroids may have
something to do with this phenomenon because, in skin folds,
topical corticosteroids stay longer, penetrate deeper and work
better, leading to a stronger clinical effect in interrupting new
formation of the rash.
Figure 3. The body is extensively involved with
an erythroderma-like appearance. The characteristic sparing of the
skin folds is called the "deck-chair sign".
Systematic Review of The Worldwide Literature
Torchia et al. published an extensive systematic review
of the worldwide literature on PE in 2010.3 They
reviewed 170 cases of PE and concluded that PE represents a rather
monomorphous entity, both clinically and histologically, with the
remarkable exception of cutaneous T-cell lymphoma (CTCL).
They suggested the following etiological classification of PE as
a result of their analysis:
- Primary (idiopathic) PE.
- Symptomatic PE secondary to atopic dermatitis, drugs,
infections and malignancies.
- PE-like CTCL.
- Pseudo-PE with the "deck chair sign" in the absence of
Finally, they proposed diagnostic criteria for PE, as shown in
Table 1. Using these criteria, we can reach an exact diagnosis of
PE, or make a differential diagnosis of secondary PE, PE-like CTCL
Table 1. Redrawn from Torchia D et
- Erythroderma-like eruption formed by the coalescence of
flat-topped, red-to-brown papules with a cobblestone-like
- Sparing of skin folds and creases (deck-chair sign)
- Histopathological exclusion of CTCL (and other skin
- Workup and follow-up to exclude a causative link with
malignancies, infections, drugs and atopy
Additional Minor Criteria
- Age greater than 55 years
- Male gender
- Peripheral and/or tissue eosinophilia
- Increased serum IgE
- Peripheral lymphopenia
Criteria 1-5: primary (idiopathic) papuloerythroderma
Criteria 1-4: secondary (symptomatic) papuloerythroderma.
Criteria 1,2,3,5: papuloerythroderma-like CTCL.
What To Do When You Encounter A Patient With PE
In such circumstances, one has to exclude the possibility of
CTCL, as well as any complicated internal malignancy. Since the
synchronous or diachronical coexistence of PE and malignancy was
reported in 21.76% of patients, which was much higher than
expected, even in such elderly patients, patients should be checked
for both solid and hematological malignancies; however, in this
study, the majority of PE patients were generally healthy except
for skin eruptions.3
Recent reports have indicated that drugs might be causative
agents for PE, suggesting that drug-reactive Th2 cells play an
important role in its pathogenesis.4 A review of the
literature revealed that various drugs can cause drug-induced PE
following a mean administration period of 2 years and 3
months.3,4 Despite the chronic type of eruptions,
provocation tests but not patch tests were positive in all
patients. The authors demonstrated that Th2 cells were a
propagating population in drug-induced PE, which is consistent with
both the peripheral and tissue eosinophilia seen in most cases.
As for the treatment for PE, there is no evidence-based standard
therapy. After excluding underlying causes of PE, mild symptomatic
therapies, such as antihistamines for pruritus and topical
corticosteroids with or without phototherapy, appear to be
reasonable, as most patients are usually otherwise healthy and PE
lesions improve slowly after several years.
Although PE is a rare condition and not well-recognized by
non-Asian dermatologists, it is very easy to make a diagnosis once
you notice certain clinical features. More studies, however, are
required to answer the still unresolved questions of why PE is
found mainly in men, and rarely in women, and why the 'deck chair
The following points serve as a take-home message for
- PE is different from exfoliative erythroderma because it starts
as solid lichenoid papules that develop into an erythroderma-like
- The "deck-chair sign", eosinophilia and increased IgE are of
great help for correct diagnosis.
- If you find a patient with PE, check carefully for complicated
internal malignancies and drugs as possible causative agents.
- Ofuji S, Furukawa F, Miyachi Y, et al.
Papuloerythroderma. Dermatologica. 1984;169:125-130.
- Farthing CF, Staughton RC, Harper JI, et al.
Papuloerythroderma-a further case with the 'deck chair sign'.
- Torchia D, Miteva M, Hu S, et al. Papuloerythroderma
2009: two new cases and systematic review of the worldwide
literature 25 years after its identification by Ofuji et al.
- Sugita K, Kabashima K, Nakamura M, et al. Drug-induced
papuloerythroderma: analysis of T cell populations and a literature
review. Acta Derm Venereol. 2009;89:618-622.