Successful treatment of recalcitrant lymphomatoid papulosis in a child with PUVA-bath photochemotherapy

ARTICLE

Auteur(s) : Wolfram Hoetzenecker¹, Emmanuella Guenova¹, Konrad Hoetzenecker², Amir Yazdi¹, Martin Röcken², Mark Berneburg¹

¹Department of Dermatology, University Hospital of Tuebingen, Liebermeisterstrasse 25 D-72076 Tuebingen, Germany
²Department of Cardiac Surgery, Medical University of Vienna, Austria

Lymphomatoid papulosis (LyP) is a rare recurrent lymphoproliferative disorder of the skin usually affecting elderly adults (median age of onset: 45 years). However, in rare cases, LyP may also occur in children [1]. These young patients pose a difficult therapeutic challenge, as guideline-therapies, such as high doses of systemic corticosteroids, methotrexate, INF-α and oral PUVA are given to children very reluctantly or are contraindicated [2, 3].

A 7-year-old Caucasian boy presented in 2007 with a 2-week history of generalized cutaneous eruption associated with fever up to 38.5 °C. Previous antibiotic treatment with cephalosporine had no effect. Medical history revealed recurrent headache, otherwise, the patient felt healthy. On physical examination, multiple erythematous papules, plaques, and nodules on the trunk, face, arms and legs were noted (figure 1). Some nodules displayed a haemorrhagic and ulceronecrotic center. Furthermore, painless swelling of inguinal lymph nodes was palpable. Histology of skin specimen showed a scattered dermal infiltrate of large lymphocytes, of which some were CD30 positive. Based on the clinical and immunopathological features, LyP was diagnosed. As serological blood tests indicated a recent infection with Mycoplasma pneumoniae, we initiated systemic antibiotic treatment. However, eruptions continued to increase and therefore therapy was changed to prednisolone 20 mg daily for two weeks without any effect. As the patient suffered from significant peer-group pressure in school, we initiated PUVA-bath photochemotherapy. Treatment sessions were performed 4 times per week at the beginning, followed by two times weekly, for a total period of 9 weeks. Doses were gradually increased at each third therapy, up to a maximum single dose of 1.9 J/cm². The cumulative UVA dose was 18.7 J/cm². After 8 treatment sessions, the lesions began to flatten and subside. After 20 treatments all skin lesions had completely cleared. Therapy was well tolerated and no local or systemic side effects were observed.

Therapy guidelines of LyP include systemic treatment with corticosteroids, methotrexate, INF-α and PUVA photochemotherapy [2, 3]. While usually well tolerated in adults, most of these treatment options may be critical in children due to side effects (methotrexate, oral PUVA photochemotherapy) and lack of application security (INF-α). Here we describe a case of LyP in a 7-year-old patient treated with PUVA-bath photochemotherapy. Although the follow-up period (1.5 years) in our patient is still short and later recurrence or progression into malignant lymphomas cannot be excluded, our case demonstrates the efficacy of PUVA-bath photochemotherapy. Successful treatment of LyP in a child with PUVA-bath photochemotherapy was first described in 1995 [4]. Compared with conventional oral PUVA therapy, PUVA-bath photochemotherapy offers numerous advantages: There are no systemic side effects such as nausea, vomiting and cataractogenesis due to negligible serum levels of methoxsalen. Furthermore, PUVA-bath photochemotherapy does not induce photosensitivity in the face and hands, which is of special value in children who cannot be kept indoors all day. Thirdly, studies by Hannuksela-Svahn et al. have shown that PUVA-bath photochemotherapy does not seem to increase the risk of skin cancer incidence compared to conventional PUVA therapy, which is significantly associated with cutaneous carcinogenesis [5, 6].

However, as long-term risks of PUVA-bath photochemotherapy are not completely known as yet and approximately 10% of patients with LyP develop malignant lymphomas, regular follow-up visits are needed for our young patient.

Acknowledgements

References

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5 Hannuksela-Svahn A, Sigurgeirsson B, Pukkala E, et al. Trioxsalen bath PUVA did not increase the risk of squamous cell skin carcinoma and cutaneous malignant melanoma in a joint analysis of 944 Swedish and Finnish patients with psoriasis. Br J Dermatol 1999; 141: 497-501.

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