Depigmentation developed in a large number of people in Japan who had used cosmetics containing 2% of 4-(4-hydroxyphenyl)-2 butanol (Rhododenol). The total number of patients is estimated to be at least 18,909 in June 2014. To clarify the clinical and epidemiological features of Rhododenol-induced leukoderma in Japan, a nation wide survey was performed from July 17, 2013 to September 7, 2013. The clinical data of 1,338 cases were analyzed. Hypopigmented spots were observed on the sites of application of Rhododenol in 96% of the cases. The face (92.9%) and the neck (58.8%) were frequently involved sites. The most common pattern was of incompletely depigmented spots (42%). The completely depigmented spot type and the mixed type comprised 17% and 28%, respectively. Inflammation was observed in 43.8% of the cases. Most of the cases (85%) were clinically indistinguishable from idiopathic vitiligo. A second investigation in the future would further deepen our understanding about the course and the prognosis of this disease.
Pemphigus vulgaris (PV) is an autoimmune mucocutaneous blistering disease caused by anti-desmoglein (Dsg) autoantibodies. Alopecia has been reported as a manifestation of PV; however, its incidence is quite low. In this study, we attempted to delineate the characteristics and clinical significance of this rare symptom, based on the clinicopathological observations of a 31-year-old Japanese male alopecic PV case we treated. Notably, the hair loss in this PV case was not characterized by alopecic patches, but rather by the finding that hair shafts with visibly intact outer root sheath (ORS) were easily collected using a gentle hair pull test. Direct immunofluorescence detected intracellular IgG deposition on ORS keratinocytes. In addition, acantholysis was observed within ORS where Dsg1 and 3 were weakly expressed. These findings supported the thesis that hair loss in PV might well be coined as "hair Nikolsky" phenomenon. This pathological pattern disappeared as the patient recovered from PV, suggesting the usefulness of assessing the hair Nikolsky phenomenon in the management of PV.