LETTER TO EDITOR Repeated hyperhidrosis and chilblain-like swelling with ulceration of the fingers and toes in hereditary sensory and autonomic neuropathy type II Dear Editor We report a case of hereditary sensory and autonomic neuropathy type II (HSAN-II), involving repeated hyperhidrosis and chilblain-like swelling with ulceration of the fingers and toes of unknown cause. These symptoms were similar to scle- rodactyly and a certain type of systemic sclerosis but did not satisfy any criteria. Dermatologists should recognize such rare hereditary sensory and autonomic neuropathy associated with various skin manifestation. The patient was a 43-year-old woman with no particular past medical history, and her grandmother had osteoarthritis. The patient had suffered repeated hyperhidrosis and chilblain- like swelling with ulceration of the fingers and toes of unknown cause since approximately 17 years of age. At 38 years of age, she visited the Dermatological Department of Wakayama Medical University Hospital for the chief complaints of hyperhidrosis and shortening of the fingers and toes (Fig. 1a, b). During subsequent follow up, development of cracks in the fingers without obvious infection was observed. Excessive sweating was marked in the palms and soles. Routine labora- tory findings showed no particular results. Collagen diseases were not suggested. No abnormality was noted in the cranial nerve system, and muscle atrophy and weakness were absent in the motor system. On sensory nerve conduction studies, the median and ulnar sensory nerve action potentials were markedly reduced on bilateral sides, and no action potential was evoked in the peroneal nerve on either side. Radiograms of the bilateral second to fifth fingers showed marked shortening, in which the distal phalanges were lost and only the proximal ends of the middle phalanges remained. Shortening of the bilateral first phalanges was also noted, mainly on the right side (Fig. 1c). The radiograms of the toes showed erosion and destruction of bones and deviation of bone fragments. A skin biopsy had no characteristic findings. The sequence study detected a homozygous 1-base inser- tion generating a premature stop codon (c.3237_3238insT, p.Asp1080X) in the WNK1 (WNK1/HSN2) gene, which is known to be the cause of HSAN-II. Under careful dermatological treatments including topical tretinoin tocopherol ointment, no infection occurred in the fingers but all fingers gradually shortened (Fig. 1d). Hereditary sensory and autonomic neuropathy is a rare heredi- tary peripheral nerve disorder, which mainly manifests sensory Correspondence: Fukumi Furukawa, M.D., Ph.D., Takatsuki Red Cross Hospital, 1-1-1 Abuno, Takatsuki, Osaka 569-1096, Japan. Email: h7gygyff@gmail.com (a) (b) (c) (d) Figure 1. (a) Shortening of the fingers. (b) Shortening of the toes. (c) X-ray findings of the fingers. (d) Shortening of the fingers after 5 years. 1 © 2018 Japanese Dermatological Association doi: 10.1111/1346-8138.14336 Journal of Dermatology 2018; : 1–2