Acta Dermato-Venereologica 98-5CompleteContent | Page 15

526 SHORT COMMUNICATION Generalized Chronic Itch as a First Sign of Malignancy Resembling Paraneoplastic Sensomotoric Neuropathy Minaya BEIGI 1 , Michael HÄBERLE 2 , Andreas GSCHWENDTNER 3 , Ulrich BAUM 4 and Elke WEISSHAAR 1 * Department of Clinical Social Medicine, Environmental and Occupational Dermatology, Ruprecht Karls University Heidelberg, Voßstr. 2, DE-69115 Heidelberg, 2 Private Practice for Dermatology, Künzelsau, 3 Institute of Pathology, and 4 Institute for Diagnostic and Interventional Radiology, Caritas Hospital, Bad Mergentheim, Germany. *E-mail: [email protected] 1 Accepted Feb 13, 2018; Epub ahead of print Feb 13, 2018 Malignancies can be accompanied by paraneoplastic symptoms or syndromes, which may be caused by an indirect effect of the malignancy and may be the first sign of a cancer (1–4). The symptom “paraneoplastic itch” (PI) describes itch as a systemic reaction to the presence of a malignancy, “neither induced by the local presence of cancer cells nor by tumour therapy” (3). PI is often associated with haematological malignan- cies, but has also been reported in patients with solid tumours. Paraneoplastic neurological syndromes (PNS) are a group of rare neurological disorders that usually precede the detection of the underlying cancer (4). PNS can affect any part of the nervous system and many are associated with onconeural antibodies (4–6). We report here a patient with generalized pruritus that preceded a paraneoplastic neurological syndrome associated with onconeural anti-Hu antibodies due to large cell neuroendocrine carcinoma (LCNEC). The aim of this report is to highlight the consequence of misdiagnosed or underdiagnosed chronic itch (CI). CASE REPORT A 68-year-old man developed CI on normal-looking skin (head, arms, shoulder, thighs) in December 2015. Seve- ral treatments, e.g. topical corticosteroids and systemic antihistamines had no effect. When he presented to the Itch Clinic at University Hospital Heidelberg for the first time in March 2016 the CI had a burning, stinging and sharp character and was suspected to be of neuropathic origin. The severity of itch, assessed by numerical rating scale (NRS), was 10 (scale 0–10 [0: no itch, 10: maxi- mum imaginable itch]). His quality of life (assessed by question) was severely reduced and the patient reported severe sleeplessness due to CI. His medical history revealed a good response of CI only to a therapy with systemic corticosteroids, prednisolone 20 mg per day for 2 weeks. His medical history included myocardial infarction, pneumonia, heartburn, iron deficiency and active smoking. The patient was advised to get a full body check-up (internal medicine, neurology), but did not do so because he attributed the itch to psychological distress. Four months later, in July 2016, the patient had lost 10 kg of weight and developed nausea and vomiting. He reported loss of hearing, tinnitus and vertigo, as well as walking impairment, dysaesthesia and numbness of doi: 10.2340/00015555-2910 Acta Derm Venereol 2018; 98: 526–527 the legs. Neurological examination revealed cerebellar ataxia and a bilateral vestibular pathology. Electroneuro- graphy revealed a severe sensomotoric polyneuropathy. To detect a potential underlying malignancy, imaging examinations (e.g. chest X-ray, abdominal ultrasound, pelvis computed tomography (CT) scan) and lumbar puncture were performed. Imaging showed no evidence of a tumour, but lumbar puncture revealed the presence of onconeural anti-Hu antibodies. In the further course of the disease, the patient developed neurological gast- rointestinal paresis, as well as micturition disturbance as a part of PNS. Therefore, a close follow-up scheme was set up to detect the underlying malignancy. During the second half of 2016 itching was still present, but had decreased. Due to the dominance of the other symptoms and their im- pairment, as well as the patient’s reduced general health status, he did not present to the dermatologist and only incomplete information about itch is available. In No- vember 2016, CT scan showed a suspicious mediastinal lymph node enlargement (Fig. 1) so that a lymph-node- biopsy was taken. The biopsy proved lymph node metas- tases by a large cell neuroendocrine carcinoma (LCNEC), a rare subgroup of high-grade neuroendocrine tumour, typically of pulmonary origin. This diagnosis requires the presence of a neuroendocrine pattern and positive staining with neuroendocrine markers (chromogranin A, synaptophysin, CD 56) in immunohistochemistry, which was demonstrated in the patient (Fig. 2). However, the primary carcinoma location could not be detected. The Fig. 1. Computed tomography (CT) scan (November 2016) showing axial slices. The arrow indicates subcarinal lymph node enlargement. This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2018 Acta Dermato-Venereologica.