Bullous lesions in Bazex syndrome and successful treatment with oral psoralen phototherapy

Abstract

A 59-year-old man presented with a psoriasiform dermatitis with associated bullae and destructive nail dystrophy of the hands and feet. He had lost 10 kg weight over 6 months and a mass in the neck was noted. He was provisionally diagnosed with Bazex syndrome (acrokeratosis paraneoplastica) and subsequent investigations revealed a squamous cell carcinoma in the right piriform fossa. His skin lesions were treated with oral psoralen and ultraviolet A phototherapy and this cleared the cutaneous changes, but the nail changes have persisted at 18 months follow up.

Bazex Syndrome (Acrokeratosis Paraneoplastica) Diagnosed in a Patient with Oral Persistent Ulcerations

“…The presence of mucosal lesions associated with skin changes in the current case may be supported by previously published cases that reported the co-existence of two paraneoplastic disorders, e.g., Bazex syndrome associated with paraneoplastic pemphigoid or paraneoplastic pemphigus [1,2]. In a similar way, Gill et al [2] provided various explanations for the vesicles and bullae that may appear simultaneously to Bazex syndrome, including coexisting porphyria cutanea tarda, epidermolysis bullosa acquisita, bullous pemphigoid, bullous lichen planus or paraneoplastic pemphigus associated with the typical skin changes.…”

“…The importance of any paraneoplasia is the obvious association with an underlying malignancy. In Bazex syndrome's this association is even more important, inasmuch as cutaneous eruptions can predate clinical evidence of cancer by several months or even years in approximately 75% of the patients [2]. Any clinical sign that could suggest Bazex syndrome should automatically guide a cutaneous biopsy followed by a complete screening of the upper aerodigestive tract.…”

“…The main features of this paraneoplasia is the presence of symmetrical papulosquamous eruptions (psoriasiform cutaneous eruptions), nail dystrophy and skin scaling usually localized in the body extremities (acral distribution) which may affect ears, nose, cheeks, hands, feet, fingers, toes, elbows and knees [1]. Less frequently, skin bullae and vesicles as well as mucosa alterations can occur concomitantly to the more typical cutaneous lesions [1,2].…”

“…Likewise, regional or distant metastasis of head and neck SCC are often present. Although rare, association with other malignant tumours has been reported, including cutaneous squamous cell carcinoma, transitional cell carcinoma of the bladder, metastatic thymic carcinoma, ductal carcinoma of the breast, adenocarcinomas of the colon and prostate, liposarcomas, Hodgkin's disease, peripheral T-cell lymphoma, metastatic neuroendocrine tumour, bronchial carcinoid tumour and tumours of the liver, stomach, thymus, uterus, vulva, genitourinary tract and bone marrow [1][2][3][4][5][6][7][8][9].…”

Acrokeratosis Paraneoplastica with Adenocarcinoma of the Colon Treated with Topical Tretinoin

“…Treatment with antibiotics, topical steroids, keratolytics, vitamin D, and PUVA achieve only transient remission 2,9,13 . Despite some cases in which the cutaneous manifestations are completely healed by acitretin or etretinate without removal of the primary cancer 14,15 , the treatment of skin lesions is directly related to eradication of the underlying neoplasm.…”