Ofuji’s papuloerythroderma. An association with early gastric cancer

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.

4. Grob JJ, Collet-Villette AM, Horchowski N, et al. Ofuji papuloerythroderma. Report of a case with T cell skin lymphoma and discussion of the nature of this disease. J Am Acad Dermatol 1989; 20: 927-31.

5. Plantin P, Milochau P, Leroy JP, et al. Papuloérythrodermie d'Ofuji: efficacité de l'interféron alpha. Ann Dermatol Vénéréol 1992; 119: 643-5.

6. Dwyer CM, Chapman RS, Smith GD. Papuloerythroderma and cutaneous T cell lymphoma. Dermatology 1994; 188: 326-8.

7. Akioka J, Moriwaki SI, Yagi H, et al. A case of papuloerythroderma: an association with early cancer. Acta Dermatol 1988; 83: 181-4.

8. Ofuji S. Papuloerythroderma. J Am Acad Dermatol 1990; 22: 697.

9. Schepers C, Malvehy J, Azón-Masoliver A, et al. Papuloerythroderma of Ofuji: a report of 2 cases including the first European case associated with visceral carcinoma. Dermatology 1996; 193: 131-5.

10. Garcìa-Patos V, Repiso T, Rodrìguez-Cano L, Castells A. Ofuji papuloerythroderma in a patient with the Acquired Immunodeficiency Syndrome. Dermatology 1996; 192: 164-6.

11. Lonnee ER, Toonstra J, Van Der Putte SCJ, et al. Papuloerythroderma of Ofuji in a HIV-infected patient. Br J Dermatol 1996; 135: 500-1.

12. Bachmeyer C, Petit A, Nahmias M, Sigal-Nahum M. Papuloérythrodermie d'Ofuji. Aspect clinique d'un syndrome hyperéosinofilique? Ann Dermatol Vénéréol 1991; 118: 895-7.

13. Harris DWS, Spencer MJ, Tidman MJ. Papuloerythroderma-clinical and ultrastructural features. Clin Exp Dermatol 1990; 15: 105-6.

14. Riadh WA, Keefe M, Chapman RS. Papuloerythroderma-another case of a new disease. Arch Dermatol 1991; 127: 96-8.

15. Nazzari G, Crovato F, Nigro A. Papuloerythroderma (Ofuji): two additional cases and review of the literature. J Am Acad Dermatol 1992; 26: 499-501.

16. Saurat JH. Papuloerythroderma: disease or pattern? Dermatology 1993; 186: 163.

17. Bettioli V, Mantovani L, Altieri E, Strumia R. Ofuji papuloerythroderma: report of a European case. Dermatology 1993; 186: 187-9.