A Rare Syndrome Resembling Scleroderma: Huriez Syndrome.

Huriez syndrome, also referred to as "sclerotylosis," is a rare autosomal dominant genodermatosis characterized by the triad of congenital scleroatrophy of the distal extremities, palmoplantar keratoderma, and hypoplastic nails. The development of aggressive squamous cell carcinoma (SCC) arising in the scleroatrophic area is also a distinctive feature of the syndrome. Early diagnosis is important due to the early onset, mostly in the third to fourth decades of life, and aggressive progress of SCC, which occurs in around 15% of affected individuals. Our patient had palmoplantar keratoderma, scleroatrophy of the hands, and hypoplastic nails. Her mother and father had a second-degree blood relation. Two of her siblings had similar complaints and findings. She showed no sign of actinic keratosis or SCC, and was called for regular follow-ups. With this case, we want to emphasize that Huriez syndrome is a rare genodermatosis, mimicking scleroderma-like acrosclerosis, and early diagnosis is critical for recognizing and preventing the development of SCC.

Subungual exostosis and subungual osteochondromas: a description of 25 cases.

BACKGROUND: Subungual exostosis (SE) and subungual osteochondroma (SO) are an uncommon, benign tumor of the distal phalanx. The purpose of this retrospective study was to evaluate clinical, demographical, and radiological features; treatment modalities; and follow-up results in SE and SO cases. METHODS: Twenty-five cases were confirmed histopathologically as SE or SO. At the time of admission, clinical data were obtained on the age and sex of the patient, duration of symptoms, presence of pain, previous diagnoses and treatments, concomitant systemic diseases, family history, lesional localization, clinical and radiological features of the lesion, surgical treatment methods, and duration of follow-up. RESULTS: There were 14 patients in the SE group and 11 patients in the SO group. There was no statistically significant difference in gender, age, duration of symptoms, or pain between the two groups. Regarding the locations of the lesions, two (14.28%) were on the hands, 12 (85.72%) lesions were on the toes, 11 (91.67%) of which were on the great toe, and one (8.33%) was on the small toe of the left foot in the SE group. All the osteochondroma lesions were located on the toes. In five (45%) cases, the lesion was on the great toe. Two patients had residual lesions in the SE group. Recurrence occurred in one case in the SO group. CONCLUSIONS: Clinically and histopathologically, SE and SO appear to be two different entities. When diagnosed correctly and treated appropriately, the lesions have good functional and cosmetic results, as well as a very low recurrence rate.

Early Cosmetic Results and Midterm Follow-up Findings of Rhinophyma Patients Treated With High-Frequency Electrosurgery and a Discussion on the Severity Assessment of the Disease.

BACKGROUND: Different methods can be used in the surgical treatment of patients with rhinophyma. There are limited numbers of reports on high-frequency electrosurgery treatment. In addition, an efficient scoring system is required to evaluate severity of the disease in clinical progress and recurrence after treatment. OBJECTIVES: We evaluated patients with rhinophyma who were treated with high-frequency electrosurgery and discussed the methods used to assess severity of the disease. METHODS: Data and photos of 13 patients were retrospectively evaluated and scored via 2 different forms of the rhinophyma severity index. RESULTS: Median rhinophyma severity index scores of patients at first visits were significantly higher than those at second and third visits ( P = .002 and P = .002, respectively). Likewise, median modified rhinophyma severity scores of patients at first visits were significantly higher than those at second and third visits ( P = .001 and P = .001, respectively). Also, there was a strong positive correlation between these 2 assessment methods ( r = 0.838, P < .001). CONCLUSIONS: The rhinophyma severity assessment methods used in this study are positively correlated. High-frequency electrosurgery seems to be a procedure that is safe, effective, and relatively cost-effective in the treatment of rhinophyma lesions.