Nagashima-Type Palmoplantar Keratosis: Clinical Characteristics, Genetic Characterization, and Clinical Management

Table 1

The major clinical differentiating characteristics for distinguishing NPPK from other diseases with similar characteristics.

	NPPK [19]	MDM PPK [2, 33]	Greither PPK [34]	PPK-GN [35]	Bothnian PPK [36, 37]	Acral keratoderma [2]	Symmetrical lividities of the soles [38]	PSEK [39]
Hyperkeratosis	Mild and nonprogressive	Severe	Thick	Thick	Mild to thick	Thick	NM	+
Hyperhidrosis	+	+	+	NM	+	NM	+	NM
Flexion contractures	–	+	–	–	–	–	NM	NM
Constricting bands surrounding the digits	–	+	+	+ (occasionally)	–	+	NM	NM
Spontaneous amputation	–	+ (occasionally) [40, 41]	+	NM	–	+	NM	NM
Genetic model	AR	AR	AD	AR	AD	AR	NM	AD
Pathogenic gene	SERPINB7	SLURP1 [42]	KRT1 [34]	Unknown	AQP5 [36, 37]	TCF4 [43, 44]	NM	NM
Prevalence rate	Japan: 1.2/10,000; China: 3.1/10,000 [1]	Island of Meleda: common; General populations: 1/100,000	Rare	Rare	Rare	Rare	Rare	Rare
Whitish spongy change in affected areas upon exposure to water	+	–	–	–	+	–	NM	NM
Transgrediens	+	+	+	+	+	+	NM	NM
Progressive clinical presentation	- (after puberty)	+ (throughout the lives)	NM	NM	NM	NM	NM	NM
Special clinical features in addition to mentioned above		Perioral erythema, brachydactyly, nail abnormalities, and lichenoid plaques [41]; consanguineous marriage [45, 46]		The dorsal aspects of the finger joints are covered by hyperkeratotic plaques [35]	More hyperhidrotic, mild nail changes, and more rapid and obvious whitish spongy change and swelling upon exposure to water	Striate hyperkeratinosis of the palms and soles, and linear hyperkeratotic lesions over the Achilles tendon areas, ankles, elbows, and knees [2]	Symmetrical, bluish-red plaques on the soles of the feet, and rare involvement of palm [38]	Non-migratory erythematous and hyperkeratotic plaques that are distributed symmetrically over the body

NPPK: Nagashima-type palmoplantar keratosis; MDM: mal de Meleda; PPK-GN: Gamborg-Nielsen type PPK; PSEK: progressive symmetrical erythrokeratoderma; NM: not mentioned; +: present; −: not present; AR: autosomal recessive inheritance; AD: Autosomal dominant inheritance.