Symmetrical acrokeratoderma: A peculiar entity in China? Clinicopathologic and immunopathologic study of 34 new cases

• Published in: Journal of the American Academy of Dermatology 2014-03, Vol.70 (3), p.533-538
• Main Authors: Liu, Zhen, MM, Zhou, Ying, MM, Chen, Rong-Yi, MD, Shi, Ge, MD, Li, Wen, MB, Li, Shi-Jie, Fan, Yi-Ming, MD
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Age Distribution; Biopsy, Needle; Case-Control Studies; China; Cohort Studies; Dermatology; Female; Follow-Up Studies; friction melanosis; Hand Dermatoses - epidemiology; Hand Dermatoses - pathology; histopathology; Humans; Hyperpigmentation - epidemiology; Hyperpigmentation - pathology; ichthyosis vulgaris; Ichthyosis Vulgaris - epidemiology; Ichthyosis Vulgaris - pathology; Immunohistochemistry; Incidence; Keratoderma, Palmoplantar - epidemiology; Keratoderma, Palmoplantar - pathology; Male; Rare Diseases; Risk Assessment; Severity of Illness Index; Sex Distribution; symmetrical acrokeratoderma; terra firma-forme dermatosis; Young Adult
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/j.jaad.2013.10.061

Background Symmetrical acrokeratoderma seems to be a new disorder in China, and 138 cases have been reported in the Chinese literature. Objective We sought to summarize the clinicopathologic features and immunophenotyping of inflammatory cells in 34 new cases. Methods Clinical data of 34 patients were prospectively collected over 4 years. Histopathology and immunostaining of infiltrated cells were performed in 27 and 9 patients, respectively. Results Brown to black hyperkeratotic patches were symmetrically distributed over the acral regions in 33 cases and on the scalp in 1 case, with a whitish change after water contact or sweating. The condition was aggravated in summer and alleviated in winter in 33 patients. History of ichthyosis vulgaris was seen in 23 cases. The typical histopathology included epidermal hyperkeratosis, acanthosis, and papillary dermal perivascular infiltrate of lymphohistiocytes. Number of CD3+ , CD4+ , and CD8+ cells increased in lesional and perilesional skin compared with normal-appearing skin. The skin lesions developed slowly but were confined to the acral predilection sites after the mean follow-up of 25.4 ± 13.8 months. Limitations The follow-up time was short. Conclusion This disorder may represent a peculiar dermatosis that is frequently associated with ichthyosis vulgaris. No specific therapy is available for the disorder.