ARTICLE
Ofuji's papuloerythroderma (OP) [1] is a distinctive clinical entity
of as yet unknown etiology that usually affects the elderly, with a chronic
relapsing course over months or years. Its diagnosis is usually made by
the clinician in virtue of a peculiarly widespread lichenoid, pruritic
skin eruption with characteristic sparing of the body folds: "Deck Chair
Sign". Histological examination is unhelpful. The recognition of blood
eosinophilia and sometimes of increased serum IgE levels, makes the diagnosis
easier. Oral corticotherapy seems to be the most effective treatment.
Papuloerythroderma may occasionally be associated with B or T lymphomas
[2-6] or visceral malignancy [7-9] and Acquired Immunodeficiency Syndrome
[10, 11].
Case report
We report the case of an 88-old man who presented with a several months'
history of a pruritic generalized eruption consisting of flat-topped,
red-brown papules with lichenified appearance and linear pattern, coalesced
into large sheets producing an erythroderma-like aspect.
This dermatosis involved the trunk, mostly the chest and abdomen (Fig.
1), and the proximal regions of the limbs, with a striking sparing
of body folds, especially the popliteal fossae and transverse positional
thoracic and abdominal folds. There was also fissured palmoplantar keratoderma
with an acral cyanosis (Fig. 2).
Results of routine laboratory tests showed only
a high blood eosinophil count (2,510 cells/mm3), and a mild
increase of serum IgE levels (375 U/ml). The peripheral lymphocyte count
and lymphocyte subset proportions were normal. Sézary cells in
peripheral blood were not detectable. Neither physical examination nor
echography showed lymphadenopathy or liver and spleen enlargement.
A skin biopsy specimen revealed only a dense perivascular lymphohistiocytic
infiltrate with eosinophils in the papillary dermis.
On account of the peculiar systemic diseases
which have been reported to be related to this dermatosis, investigations
appropriate for visceral malignancy detection were performed, and a histological
examination of a gastric wall biopsy specimen drawn by endoscopy showed
early intramucosal gastric cancer. The patient refused to undergo any
surgical treatment.
Therapy with oral corticosteroids achieved a complete recovery of pruritus
and resolution of erythroderma after 16 days treatment.
A mild relapse, with spontaneous recovery, occurred after 8 months.
Discussion
Papuloerythroderma of Ofuji is a condition first described in 1984;
so far about 56 cases have been reported in the literature, most of which
concerned Japanese patients. This entity has been correlated to various
specific diseases such as lymphoma [2-6], hypereosinophylic syndrome [12],
AIDS [10, 11] and visceral malignancy [7-9]; but its rarity, especially
in countries other than Japan, does not allow us to clarify its relationship
with paraneoplastic syndromes or prelymphoma conditions. On the other
hand it might represent a peculiar pattern of the expression of several
inflammatory dermatoses in elderly people [8, 13-17]. To our knowledge,
papuloerythroderma has not been previously reported in association to
early gastric cancer in the European and North-American literature. Nine
reported cases [7, 8] of gastric cancer have been associated with papuloerythroderma.
In our opinion, the reporting and long term follow-up of further cases
are a necessary condition for a definitive nosological placement and prognostic
evaluation of this rare dermatosis.
REFERENCES
1. Ofuji S, Furukawa F, Miyachi Y, et al. Papuloerythroderma.
Dermatologica 1984; 169: 125-30.
2. Takiguchi Y, Shimada K, Suzuki M, et al. A case of B-cell
lymphoma preceded by papuloerythroderma (Ofuji). Jpn J Clin Dermatol
1987; 41: 985-9.
3. Nakajima M, Hashikawa Y, Komatsu Y, et al. Leukemic T cell
lymphoma with lichen rubber-like erythroderma. Rinsho Derm (Tokio)
1988; 30: 1523-7.
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