Acta Dermato-Venereologica 99-3CompleteContent | Page 21

SHORT COMMUNICATION 333 Acrokeratosis Paraneoplastica-like Findings as a Manifestation of Systemic Lupus Erythematosus Laura HUILAJA 1,2 , Minna SORONEN 1,2 , Anna KARJALAINEN 3 and Kaisa TASANEN 1,2 PEDEGO Research Unit, University of Oulu, 2 Department of Dermatology and Medical Research Center Oulu, and 3 Department of Medicine, Oulu University Hospital, Aapistie 5A, FIN-90020 Oulu, Finland. E-mail: [email protected] ¹ Accepted Nov 14, 2018; E-published Nov 14, 2018 Paraneoplastic acrokeratosis (Bazex syndrome) is a dermatosis associated with underlying malignancy, most often with squamous cell carcinoma of the upper aerodigestive tract (1). It typically occurs in middle-aged or older men with papulosquamous plaques over acral areas. During the course of the dermatosis skin symptoms spread over the trunk, extremities and scalp (1). Histolo- gical findings of paraneoplastic acrokeratosis are variable and the diagnosis is based on clinical findings (1, 2). In most cases cutaneous findings precede the malignant process for months or even a year (3, 4). CASE REPORT After obtaining informed consent, we report here the case of a 40-year-old healthy woman who was referred to the dermatologist with skin symptoms that had started 2 months earlier. At the time of consultation, she had a brownish hyperkeratosis on the helixes of the ears, tip of the nose and chin (Fig. 1a, b). The skin on the neck had spots of hyperpigmentation, but these were unpalpable. The hypothenar areas of the palms, fingertips (Fig. 1c, e) and soles had peeling psoriasis-like patches and she had a few urticarial lesions on the shins. There were follicularly highlighted reddish patches with some hy- perkeratotic plaques on her chest and upper arms (Fig. 1f, g). Her hair was coarse and diffusely thinned. She had no other symptoms, but she reported having experienced pain in her fingers during recent months. Paraneoplastic acrokeratosis was considered and she was screened for the possible underlying malignancy by chest and breast radiography, abdomen ultrasound examination, gastros- copy, as well as examination by otorhinolaryngologist and gynaecologist with normal findings except mild unilateral pleuritis. Her blood cell count was normal except for slight thrombocytopaenia. After 2 months, the patient was febrile and had pain in her fingers and soles. Skin findings were even more prominent (Fig. 1d). Skin biopsy was ambiguous, with features of lichen sclerosus as well as scleroderma. Enlarged spleen and adenopathy in several areas was found in computer tomography (CT), followed by po- sitron emission-CT (PET-CT) with similar findings and bilaterial pleural effusion. Lymphoma was suspected, but only reactive findings were seen in repeated lymph node biopsies. At this time, she had anaemia, thrombo- cytopaenia and leuco- and lymphopaenia. In addition, she had markedly elevated serum anti-nuclear antibody Fig. 1. Clinical presentation. Paraneoplastic acrokeratosis-like lesions as a skin finding of systemic lupus erythematosus (SLE). Brownish hyperkeratosis in (a) the nose tip and (b) the auricle. (c, d) Peeling, psoriasiformic patches in palms and soles. (e) Hyperkeratosis of the fingertips and under the nails. (f, g) Follicularly highlighted reddish patches on the chest. This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica. doi: 10.2340/00015555-3085 Acta Derm Venereol 2019; 99: 333–334