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Paul Henri Philippe Lefèvre, son of Louis Marie Clair Henry Lefèvre and Marie Louise Henriette Lefèvre,
was born in Vailly-sur-Sauldre (Cher, Centre-Val de Loire, France) on July 23, 1891. His father was a
physician and his mother was a housewife. As a student, Lefèvre was stripped of his bronze medal and
nearly thrown out of his medical school because of a regrettable incident on hospital premises involving
drunkenness and young ladies during the night of August 6, 1912 [Figure 3]. He graduated in 1921 as a
dermatologist. He completed internships between 1921 and 1925 at Saint Louis and Trousseau hospitals.
His thesis for the doctorate in medicine was entitled « Biological reactivation of the Bordet-Wassermann
reaction (Milian’s method). His practice in the research of hereditary syphilis » was published in 1925. He was
assistant to Dr. Papillon between August 15, 1923 and February 14, 1924. He died on June 30, 1982 in
Beauvais (Oise, Hauts-de-France, France).

[4]
Dr. Papillon and Dr. Lefèvre published the first cases of PLS in 1924 . They examined a six-year-old boy
and his four-and-a-half-year-old sister from Ulm (Germany), who presented at the Armand Trousseau
Children Hospital in Paris on February 1, 1924. Their parents were first cousins. Dr. Papillon and his
assistant Dr. Lefèvre observed striking lesions on the palms and soles as well as severe dental decay, which
was described in detail by Dr. Ruppé (Enfants-Assistés Hospital, Paris). Dr. Darier (Saint-Louis Hospital,
Paris) [Figure 6], internationally known as the doyen of French dermatology, agreed with the diagnosis of
palmoplantar keratoderma analogous to that previously observed by E. Ehlers on the Island of Mljet
(Meleda), Croatia (Mal de Meleda). However, the association of palmoplantar keratoderma with dental
decay was novel and highlighted in the article translated into English below. Dr. Petges and Dr. Delguel
presented a similar case in Bordeaux in 1927, apparently unaware of the communication from Papillon and
Lefèvre.

In 1951 Dr. Bataille, Paris Hospital stomatologist, presented another child with palmo-plantar keratoderma,
in whom he followed progressive alveolar loss over several months. In 1954, E. Bélanger reported in his
doctoral thesis the recessive character of “Papillon-Lefèvre type syndrome” or “Papillon-Lefèvre form”
referring to Mal de Meleda keratoderma. Bélanger failed to locate the original patients described by Papillon
and Lefèvre. It is historically interesting that the names and ethnicity of the children were not mentioned in
the 1924 paper and they may have been deliberately concealed at that time. Only two years earlier, Dr.
Papillon presented papers at the Paris Pediatric Society which did include patient names.

Two cases of familial symmetrical palmar and plantar keratoderma (Meleda’s disease) in a brother and
sister. Coexistence in the two cases of serious dental abnormalities, by Messieurs PAPILLON and PAUL
LEFEVRE

On February 1 two children, a boy aged six and his sister aged four-and-a-half, were admitted to Trousseau
hospital with lice infestation, pyoderma of the scalp and poor general condition. We are struck by the
lesions on the palms and soles of both children, which can be described as follows:

In the sister: the soles of both feet are entirely covered by symmetrical keratotic lesions with a distinct
border. These lesions consist of a scaly thickening, maximal in the creases which look like whitish lines of
parchment in a grid-like pattern. Between the creases are polygonal horny plaques, raised in places, which
can be removed as small scales several millimeters across; in places, the horny layer is less prominent, but
the skin is red, tense, hard and atrophic.

The sole is generally yellowish in color and an unpleasant odor emerges from the skin of this region; there is
some perspiration in the less thickened areas; by contrast, the scaly regions are dry.