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 15th to 16th 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.

Case report of connubial frontal fibrosing alopecia

“…Steven Kossard is credited with the original description of the condition,[ 1 ] and eventually its nosological classification as a frontal variant of lichen planopilaris,[ 2 ] though there is circumstantial evidence of its existence well before 1994,[ 17 ] and the observation of cutaneous lupus erythematosus presenting as FFA[ 18 ] suggests that the pattern of clinical disease presentation may be more specific for the condition than the underlying inflammatory autoimmune reaction, whether lichen planus or lupus erythematosus. Ultimately, it has been discussed to what extent a background of androgenetic alopecia may contribute to the particular clinical presentation of a pattern-type of cicatricial hair loss.…”

Familial cicatricial alopecia: Report of familial frontal fibrosing alopecia and fibrosing alopecia in a pattern distribution

“…Moreover, there is evidence that FFA was described well before Kossard's original report and the use of modern sunscreens. [ 26 ]…”

A case of frontal fibrosing alopecia in a 19th century portrait?

“…2 Even though it has been identified only 25 years ago, there are likely previous cases of FFA that went unnoticed. 3 Trüeb identified the possible first description of FFA in The Story of San Michele (1929), a book of memoirs by Swedish medical doctor Axel Munthe.…”