Photodermatol Photoimmunol Photomed 2007; 23: 37–38 Blackwell Munksgaard
r 2007 The Authors. Journal compilation r 2007 Blackwell Munksgaard
Brief communication
Acrodermatitis paraneoplastica responding to topical PUVA treatment Ayad E. Abrou, Jamie Hope, Diane Jackson-Richards, Henry W. Lim, David M. Ozog Department of Dermatology, Henry Ford Hospital, Detroit, MI, USA
Acrodermatitis paraneoplastica is a rare paraneoplastic entity that has been associated with multiple neoplasms, most commonly squamous cell carcinoma of the aerodigestive tract. Therapies have focused on treatment of the underlying carcinoma. We report a
case of acrodermatitis paraneoplastica successfully treated with PUVA therapy.
A
auricular and supraclavicular node chains. The nails were thickened and yellow, with marked subungual debris surrounded by erythema and scaling in the paronychial skin. There was erythema with thick scales on the dorsum of the hands and fingers (Fig. 1). The soles of the feet revealed thick erythematous plaques. The nose and ears were clear. Fungal cultures of the nails and skin were repeatedly negative. X-ray of the hands was negative for osteomyelitis or psoriatic changes. Histopathological examination of the affected skin showed epidermal acanthosis with hyperkeratosis and parakeratosis. Direct immunofluorescence staining of the skin was negative. A biopsy of one of the involved lymph nodes demonstrated a large B-cell follicular lymphoma, stage 3-A, grade 3, with no bone marrow involvement. The diagnosis and treatments were discussed with the patient, and a regimen of CHOP (cyclophosphamide, doxorubicin hydrochloride, vincristine, and prednisone) with Rituximab chemotherapy was begun. The patient underwent three rounds of chemotherapy treatment. During the treatments and shortly thereafter, his cutaneous lesions did not improve. The patient chose not to complete another course of the chemotherapy, and topical therapy with 0.1% 8-meths oxypsoralen in Lubriderm (Pfizer, Murris Plains, NJ, USA) followed by 20 min with UVA radiation was started for control of his cutaneous manifestations within 4 weeks of his last chemotherapy. The patient showed dramatic improvement of his skin after topical PUVA treatment, during which time there was no treatment for his lymphoma. A total of 33 treatments were administered to the hands, and 47 treatments to the feet. Before the initiation of the treatments, his hands and feet were hyperkeratotic,
crodermatitis paraneoplastica, also known as Bazex syndrome, is the cutaneous manifestation of an underlying neoplastic disease, typically of the aerodigestive tract. This is a rare syndrome first described in 1965 by Bazex et al. in a patient with a squamous cell carcinoma of the tongue (1). Since that time, virtually all patients reported in the literature with Bazex syndrome have been found to have an underlying neoplasm. We present a case of a patient with Bazex syndrome who declined to continue chemotherapeutic treatment for B-cell lymphoma. Following cessation of the chemotherapy, the patient’s hands and feet were treated with topical psoralen plus ultraviolet A (PUVA) as a palliative measure. This treatment led to dramatic improvement of his skin condition, while his lymphoma was untreated and unchanged.
Case report A 63-year-old Caucasian male was referred to the Department of Dermatology for a refractory eruption involving his fingertips and nails, and toenails. He complained of difficultly working with his hands as well as pain with ambulation secondary to fissuring, hypertrophy, and swelling. The condition began 9 months before his presentation. He had been treated with topical and oral corticosteroids as well as topical and oral antifungals without improvement. He denied any constitutional symptoms including weight loss or night sweats. He was a cigarette smoker (45 packyear). He had been drinking a six pack of canned beer daily for more than 10 years. Physical examination revealed significant left cervical lymphadenopathy including the posterior
Key words: acrodermatitis paraneoplastica (Bazex syndrome); psoralen plus ultraviolet A (PUVA).
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Abrou et al.
Fig. 1. Pre-treatment: Erythema, thick scales, and edema on the dorsum of hands and fingers. Note the dystrophic nails.
Fig. 2. Clearance of most lesions after the completion of 33 topical psoralen plus ultraviolet treatments. Note the persistence of periungal erythema and scaliness, and the nail dystrophy. swollen, and fissured, with markedly deformed nails. After completion of topical PUVA therapy, all skin lesions resolved, except for erythema and scaliness on the periungual area. However, the nails continued to be dystrophic (Fig. 2).
neoplasm (2). Furthermore, there are cases of the skin lesions reappearing when the cancer recurs. Numerous sources have reported that the cutaneous symptoms are unlikely to clear until the underlying neoplasm has been successfully eliminated. Several treatments of the skin condition have been attempted; yet, the cutaneous manifestations of Bazex syndrome have previously been shown to be almost refractory in the setting of an untreated neoplasm. Case reports show rare and inconsistent improvement with systemic etretinate, corticosteroids, and antimicrobials, and similar inconsistent results with topical treatments of tar, salicylic acid, vitamin D, antimicrobials, corticosteroids, keratolytics, and UVB irradiation (2). Nail changes will typically persist even after the skin manifestations have resolved following successful treatment of the neoplasm (3). Cases of patients with Bazex syndrome and persistent skin manifestations after successful treatment of the underlying malignancies have been reported (4, 5). Our patient had skin involvement that was not responsive to chemotherapy. However, his skin significantly improved after topical PUVA therapy. Such a response has been reported with both topical and systemic PUVA treatment (4, 5). The patient had continued involvement of the nails, with only a modest improvement after PUVA therapy. This is in keeping with many of the reported cases where nail changes persisted even after treatment of the underlying malignancy and skin improvement.
References 1. Mutasim DF, Meiri G. Bazex syndrome mimicking a primary autoimmune bullous disorder. J Am Acad Dermatol 1999; 40: 822–825. 2. Bolognia JL. Bazex syndrome: acrokeratosis paraneoplastica. Semin Dermatol 1995; 14: 84–89. 3. Jacobson FK, Abildtrub N, Laurensen SO, Brandrub F, Jensen NK. Acrokeratosis paraneoplastica (Bazex’ syndrome). Arch Dermatol 1984; 120: 502–504. 4. O’Brien TJ. Bazex syndrome (acrodermatitis paraneoplastica). Australas J Dermatol 1995; 36: 91–93. 5. Gill D, Fergin P, Kelly J. Bullous lesions in Bazex syndrome and successful treatment with oral psoralen phototherapy. Australas J Dermatol 2001; 42: 278–280.
Discussion Acrodermatitis paraneoplastica is a paraneoplastic eruption involving mainly acral skin and nails. Characteristic psoriasiform lesions occur before the diagnosis of the neoplasm in 67% of cases, concurrent with the diagnosis in 18% of cases, and after the diagnosis in 15% of cases (2). In a review of published cases with adequate clinical information and treatment, it was found that greater than 90% of patients improved following appropriate treatment of the
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Accepted for publication 11 May 2005 Corresponding author: David M. Ozog, M.D. Department of Dermatology K-16 3031 W. Grand Blvd, Suite 800 Detroit, MI 48322 USA Tel: 11 313 916 4060 Fax: 11 313 916 2093 e-mail:
[email protected]
