A Comment on Frontal Fibrosing Alopecia (Axel Munthe's Syndrome)

Abstract

Frontal fibrosing alopecia (FFA) represents a peculiar condition with a quasi-symmetrical, marginal alopecia along the frontal and temporal hairline with scarring. Steven Kossard is credited with the original description of the condition in 1994. Since its first description, FFA has become increasingly common while its etiology has remained obscure. While FFA has been related to lichen planopilaris, in fact, the pattern of clinical disease presentation might be more specific for the condition than the underlying inflammatory autoimmune reaction. It has been speculated as to whether FFA existed before Kossard's original report since artistic depictions dating from the 15^th to 16^th century often show a high frontotemporal hairline. Rather, these represent fashionable forms of frontal pseudo-alopecia than FFA. Nevertheless, there is compelling evidence that FFA existed well before 1994 from the Swedish Physician Axel Munthe's (1857-1948) account of the distinctive features of the condition in his book of memoirs "The Story of San Michele." Therefore, Axel Munthe is to be acknowledged the first description of FFA in 1929.

Keywords: Axel Munthe; Mamsell Agata; fashionable forms of frontal pseudo-alopecia; frontal fibrosing alopecia.

Figure 1

Fashionable forms of frontal pseudo-alopecia: (a) King Henry V of England and (b) Elizabeth Woodville, Royal Collection, Windsor

Figure 2

In the atrium of the Villa San Michele at Anacapri: The author (far right) together with hair and nail experts Jerry Shapiro, Eckart Haneke, and Bertrand Richert-Baran (second from left to right)