Papuloerythroderma of Ofuji associated with chronic lymphatic leukaemia

ARTICLE

Auteur(s) : Irene Salgüero¹, Carmen Moreno², Ingrid Aguayo-Leiva¹, Antonio Harto¹

¹Department of Dermatologyn, Hospital Ramón y Cajal, Carretera Colmenar Viejo Km 9100, Madrid, Spain
²Dept of Pathology Hospital Ramón y Cajal, Madrid, Spain

Papuloerythroderma (PE) is a rare dermatological disorder characterized by the coalescence of pruritic papules into an erythroderma. We describe clinical features of Ofuji’s papuloerythroderma complicated by chronic lymphatic leukaemia (CLL-B). To our knowledge only one case of PE associated with CLL-B has been previously reported.

A 74-year-old male presented with a 7 month history of a pruritic papular rash on the trunk. He was diagnosed with chronic eczema and treated with topical corticosteroids, with only minor improvement of the pruritus. He had no personal history of atopic dermatitis, psoriasis or potentially causative drug intake. Dermatological examination revealed a papular rash on the trunk and limbs, sparing the skin folds on the abdomen, resulting in the typical “deck chair” sign (figure 1A). Several studies for visceral malignancy were performed with no relevant findings. Laboratory tests just showed slight eosinophilia. A skin biopsy showed a superficial perivascular infiltrate composed predominantly of lymphocytes with a few eosinophils (figure 1B). Immunohistochemical studies demonstrated the reactive nature of the infiltrate, which was composed wholly of T cells (CD3+, CD4+). No leukaemic B cells were identified. The patient was treated with topical corticosteroids and an occlusive dressing with significant clinical improvement. Three months later, without skin lesions, the patient presented with a 3 week history of malaise and weight loss associated with peripheral edema. Clinical examination revealed enlarged lymph nodes on laterocervical and occipital regions. Full blood count showed only anemia (haemoglobin of 9.4 g/dL). Bone marrow biopsy revealed a diffuse infiltration by LB. The cytometric study revealed the following immunophenotype: CD 19+, CD 20+, CD 5+, CD 21–, CD 22+, CD 23+/–. We diagnosed: CLL-B associated with papuloerythroderma. An waiting attitude was maintained, and the patient has not received treatment for the CLL-B. To date, the skin lesions have disappeared.

Papuloerythroderma was first described by Ofuji et al. in 1984 [1]. It is known to affect predominantly elderly males (7:1) but as it is an unusual disease is difficult to obtain precise data of its incidence. Characteristic clinical features are a widespread pruritic eruption of red papules that spares the skin folds, developing into the “deck-chair” sign, probably as a result of vascular occlusion that blocks the efferent signal of the immune reaction [3]. Serum tests often show marked eosinophilia, lymphopenia and high levels of IgE. It is unclear whether PE is a distinctive clinical situation reflecting a specific pathological disease or whether it reflects the pattern of expression that many inflammatory dermatoses, like psoriasis or eczema, take [2]. There are several cases of PE associated with cutaneous T cell lymphoma [4], suggesting that this clinical situation could represent a paraneoplastic syndrome or a pre-lymphomatous condition. Some authors have also suggested that PE represents an unusual T-cell lymphoma that is different from mycosis fungoides and Sézary syndrome [2]. Associations with visceral carcinomas, acquired immunodeficiency syndrome, hepatitis C viral infection, choledocholithiasis, cutaneous fungal infection, hypereosinophilic syndrome [3] have been described.

The histopathological findings of PE are non specific. A dense perivascular infiltrate mainly composed of T- lymphocytes, histiocytes and eosinophils is observed in the dermis [2]. The diagnosis of PE requires taking into account all clinical, analytical and histological data. A full examination is highly recommended, checking for visceral carcinoma and haematological malignancies [5], mainly T-cell lymphoproliferative disorders like mycosis fungoides [6].

In sum, we report a case of PE associated with CLL-B. We think it is of interest because the cutaneous papular rash disappeared with non aggressive treatment and after a short period of time the patient developed a CLL-B. We consider that PE could represent a pre-lymphomatous condition, consequently a close long-term follow up is required in these patients.

Acknowledgements

References

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3 Hur J, Seong JY, Choi TS, Jang JG, Jang MS, Suh KS, Kim ST, et al. Mycosis fungoides presenting as Ofuji’s papuloerythroderma. J Eur Acad Dermatol Venereol 2002; 16: 393-6.

4 Wong CL, Houghton JB, Andrew S, Griffiths CE. Papuloerythroderma of Ofuji associated with acute myeloid leukaemia. Clin Exp Dermatol 2003; 28: 277-9.

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