A rare case of Vohwinkel’s syndrome

Keratoderma hereditarium mutilans or Vohwinkel’s syndrome is a rare autosomal dominant type of palmoplantar keratoderma characterized by honeycomb appearance, pseudoainhum leading to auto-amputation and stellate keratosis on knuckles. It’s mode of inheritance is autosomal dominant with mutation in loricrin and connexin 26 genes. We report the case of a 35-year-old female with alopecia universalis since birth and transgradient palmoplantar keratoderma, impaired hearing and pseudoainhum formation since childhood. This case is being reported to highlight the association of Vohwinkel’s syndrome with sensorineural hearing loss and alopecia universalis.

Prevalence and diagnostic value of the ultrasonographic honeycomb appearance of the spleen in cats

Objectives The aim of this study was to report the prevalence of a honeycomb appearance of the spleen in a population of referral cats presented for ultrasound examination, and to determine the diagnostic value of this finding vs the definitive diagnosis, the splenic cytological and haematological results. Methods Data were obtained from the medical records (2016–2018) of cats that had an ultrasonographic honeycomb appearance of the spleen, a splenic cytological diagnosis and a complete blood count. Results Twenty-five cats were included. Prevalence of the honeycomb pattern was 6.8%. None of the spleen was considered normal on cytology and four types of lesions were found: lymphoid hyperplasia (64%), neoplasia (16%), extramedullary haematopoiesis (12%) and splenitis (8%). A honeycomb pattern was successfully identified with a linear high-frequency probe in all cats, but only in 36% of cases with the micro-convex probe. Follow-up information was available for four cats, in which the honeycomb appearance persisted up to 105 days after the first examination; there was persistence of the honeycomb pattern in all cases. Cats with a splenic cytological diagnosis of extramedullary haematopoiesis had the lowest haemoglobin plasma concentration ( P = 0.011). Conclusions and relevance Honeycomb appearance of the spleen is uncommon in cats and, in our study, was systematically associated with cytological alterations; most of the time it was benign (84%). The use of a high-frequency linear probe improves its detection rate. No epidemiological, ultrasonographic or clinical criteria allow differentiation between the different types of infiltration and fine-needle aspiration is therefore recommended.