Chapter 48 - Inherited Palmoplantar Keratodermas

Question 1

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Epidermolytic PPK

Answer 1

Diffuse PPK

Question 2

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Unna-Thost PPK

Answer 2

Diffuse PPK

Question 3

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Greither syndrome

Answer 3

Diffuse PPK

Question 4

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Loricrin keratoderma

Answer 4

Diffuse PPK

Question 5

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

PPK Bothnia type

Answer 5

Diffuse PPK

Question 6

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Mal de Meleda

Answer 6

Diffuse PPK

Question 7

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Nagashima-type PPK

Answer 7

Diffuse PPK

Question 8

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Olmsted syndrome

Answer 8

Diffuse PPK

Question 9

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Vohwinkel syndrome

Answer 9

Diffuse PPK

Question 10

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Keratitis–ichthyosis–deafness
syndrome

Answer 10

Diffuse PPK

Question 11

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Bart-Pumphrey syndrome

Answer 11

Diffuse PPK

Question 12

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Palmoplantar Keratoderma with deafness

Answer 12

Diffuse PPK

Question 13

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

PPK–congenital alopecia syndrome

Answer 13

Diffuse PPK

Question 14

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Hidrotic ectodermal dysplasia (Clouston) syndrome

Answer 14

Diffuse PPK

Question 15

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Huriez syndrome

Answer 15

Diffuse PPK

Question 16

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Papillon-Lefevre syndrome

Answer 16

Diffuse PPK

Question 17

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Naxos disease

Answer 17

Diffuse PPK

Question 18

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome

Answer 18

Diffuse PPK

Question 19

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

PPK with deafness

Answer 19

Diffuse PPK

Question 20

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Striate PPK

Answer 20

Focal PPK

Question 21

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Hereditary keratosis palmoplantaris variant of Wachters

Answer 21

Focal PPK

Question 22

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Pachyonychia congenital

Answer 22

Focal PPK

Question 23

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Howel-Evans syndrome

Answer 23

Focal PPK

Question 24

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Richner-Hanhart syndrome

Answer 24

Focal PPK

Question 25

TABLE 48-4 Summary for Palmoplantar Keratodermas TYPE OF PPK (Diffuse, Focal, or Punctate) Carvajal syndrome

Answer 25

Focal PPK

Question 26

TABLE 48-4 Summary for Palmoplantar Keratodermas TYPE OF PPK (Diffuse, Focal, or Punctate) Punctate PPPK type I

Answer 26

Punctate PPK

Question 27

TABLE 48-4 Summary for Palmoplantar Keratodermas TYPE OF PPK (Diffuse, Focal, or Punctate) Punctate PPPK type 2

Answer 27

Punctate PPK

Question 28

TABLE 48-4 Summary for Palmoplantar Keratodermas TYPE OF PPK (Diffuse, Focal, or Punctate) Punctate PPPK type 3 (acrokeratoelastoidosis of Costa)

Answer 28

Punctate PPK

Question 29

TABLE 48-4 Summary for Palmoplantar Keratodermas TYPE OF PPK (Diffuse, Focal, or Punctate) Cole disease

Answer 29

Punctate PPK

Question 30

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Epidermolytic PPK

Answer 30

Without

Question 31

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Unna-Thost PPK

Answer 31

Without

Question 32

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Greither syndrome

Answer 32

Without

Question 33

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Loricrin keratoderma

Answer 33

Without

Question 34

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) PPK Bothnia type

Answer 34

Without

Question 35

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Mal de Meleda

Answer 35

Without

Question 36

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Nagashima-type PPK

Answer 36

Without

Question 37

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Olmsted syndrome

Answer 37

With

Question 38

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Vohwinkel syndrome

Answer 38

With

Question 39

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Keratitis–ichthyosis–deafness syndrome

Answer 39

With

Question 40

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Bart-Pumphrey syndrome

Answer 40

With

Question 41

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Palmoplantar Keratoderma with deafness

Answer 41

With

Question 42

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) PPK–congenital alopecia syndrome

Answer 42

With

Question 43

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Hidrotic ectodermal dysplasia (Clouston) syndrome

Answer 43

With

Question 44

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Huriez syndrome

Answer 44

With

Question 45

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Papillon-Lefevre syndrome

Answer 45

With

Question 46

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Naxos disease

Answer 46

With

Question 47

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome

Answer 47

With

Question 48

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) PPK with deafness

Answer 48

With

Question 49

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Striate PPK

Answer 49

Without

Question 50

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Hereditary keratosis palmoplantaris variant of Wachters

Answer 50

Without

Question 51

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Pachyonychia congenital

Answer 51

With

Question 52

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Howel-Evans syndrome

Answer 52

With

Question 53

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Richner-Hanhart syndrome

Answer 53

With

Question 54

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Carvajal syndrome

Answer 54

With

Question 55

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Punctate PPPK type I

Answer 55

Without

Question 56

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Punctate PPPK type 2

Answer 56

Without

Question 57

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Punctate PPPK type 3 (acrokeratoelastoidosis of Costa)

Answer 57

Without

Question 58

TABLE 48-4 Summary for Palmoplantar Keratodermas EXTRACUTANEOUS FEATURES (With or Without) Cole disease

Answer 58

With

Question 59

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Epidermolytic PPK

Answer 59

AD

Question 60

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Unna-Thost PPK

Answer 60

AD

Question 61

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Greither syndrome

Answer 61

AD

Question 62

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Loricrin keratoderma

Answer 62

AD

Question 63

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE PPK Bothnia type

Answer 63

AD

Question 64

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Mal de Meleda

Answer 64

AR

Question 65

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Nagashima-type PPK

Answer 65

AR

Question 66

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Olmsted syndrome

Answer 66

AD

Question 67

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Vohwinkel syndrome

Answer 67

AD

Question 68

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Keratitis–ichthyosis–deafness syndrome

Answer 68

AD

Question 69

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Bart-Pumphrey syndrome

Answer 69

AD

Question 70

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Palmoplantar Keratoderma with deafness

Answer 70

AD

Question 71

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE PPK–congenital alopecia syndrome

Answer 71

AD

Question 72

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Hidrotic ectodermal dysplasia (Clouston) syndrome

Answer 72

AD

Question 73

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Huriez syndrome

Answer 73

AD

Question 74

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Papillon-Lefevre syndrome

Answer 74

AR

Question 75

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Naxos disease

Answer 75

AR

Question 76

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome

Answer 76

AR

Question 77

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE PPK with deafness

Answer 77

Mitochondrial

Question 78

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Striate PPK

Answer 78

AD

Question 79

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Hereditary keratosis palmoplantaris variant of Wachters

Answer 79

AD

Question 80

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Pachyonychia congenital

Answer 80

AD

Question 81

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Howel-Evans syndrome

Answer 81

AD

Question 82

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Richner-Hanhart syndrome

Answer 82

AR

Question 83

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Carvajal syndrome

Answer 83

AR

Question 84

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Punctate PPPK type I

Answer 84

AD

Question 85

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Punctate PPPK type 2

Answer 85

AD

Question 86

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Punctate PPPK type 3 (acrokeratoelastoidosis of Costa)

Answer 86

AD

Question 87

TABLE 48-4 Summary for Palmoplantar Keratodermas MODE OF INHERITANCE Cole disease

Answer 87

AD

Question 88

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Most common form of diffuse keratoderma, with erythematous sharp margins at the edge of the palms and soles, with thickening and fissuring

Answer 88

Epidermolytic PPK

Question 89

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Diffuse, well-demarcated, yellowish, thick hyperkeratosis with an erythematous rim overlying the palms and soles; smooth and waxy texture

Answer 89

Unna-Thost PPK

Question 90

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Diffuse, thickened, scaly yellowish PPK, with transgrediens

Answer 90

Greither syndrome

Question 91

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Honeycomb-like PPK, starfish-like hyperkeratosis, prominent knuckle pads and pseudoainhum

Answer 91

Loricrin keratoderma

Question 92

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Diffuse, homogenous hyperkeratosis with a yellowish hue; aquagenic wrinkling of palms

Answer 92

PPK Bothnia type

Question 93

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Progressive, mutilating PPK with transgrediens; yellowish waxy hyperkeratotic plaques with a red, scaly border over palms and soles; risk for malignancies

Answer 93

Mal de Meleda

Question 94

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Most common type in Asians; transgrediens, nonprogressive, nonmutilating; well-demarcated, erythematous palmoplantar hyperkeratosis

Answer 94

Nagashima-type PPK

Question 95

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Mutilating PPK with periorificial keratotic plaques; symmetric, sharply demarcated diffuse PPK with painful fissures and erythematous borders; hair abnormalities are common

Answer 95

Olmsted syndrome

Question 96

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS PPK with hearing impairment; mutilating honeycomb-like PPK, with starfish shaped keratotic plaques, pseudoainhum

Answer 96

Vohwinkel syndrome

Question 97

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Erythrokeratoderma, grainy PPK with a rough, stippled appearance, chronic cheilitis and perleche, abnormal ectodermal features, progressive keratitis

Answer 97

Keratitis–ichthyosis–deafness syndrome

Question 98

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Honeycomb-like, diffuse, sharply demarcated PPK, knuckle pads, leukonychia

Answer 98

Bart-Pumphrey syndrome

Question 99

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Diffuse, transgrediens PPK with underlying erythema, knuckle pads, hearing impairment

Answer 99

Palmoplantar Keratoderma with deafness

Question 100

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Well-defined, focal, nonmutilating transgrediens PPK; noncicatricial alopecia; recessive forms may have pseudoainhum and sclerodactyly

Answer 100

PPK–congenital alopecia syndrome

Question 101

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Diffuse, velvety or cobblestone-like PPK with fissures; nail dystrophy and hair loss

Answer 101

Hidrotic ectodermal dysplasia (Clouston) syndrome

Question 102

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Diffuse, yellowish-grey, nonerythematous PPK, scleroatrophy, sclerodactyly, SCCs

Answer 102

Huriez syndrome

Question 103

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Diffuse, erythematous, sharply demarcated hyperkeratotic PPK with transgrediens; severe progressive periodontitis and loss of primary and permanent teeth

Answer 103

Papillon-Lefevre syndrome

Question 104

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Diffuse, well-demarcated, nontransgrediens PPK; wooly hair; right ventriculopathy

Answer 104

Naxos disease

Question 105

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS PPK with multiple SCCs and sclerodactyly; chronic periodontal disease leading to loss of teeth; hypogenitalism, altered plasma sex hormone levels, and sex reversal

Answer 105

Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome

Question 106

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Orange-yellow, diffuse, well-demarcated PPK with minimal to no erythema over pressure points; hearing impairment

Answer 106

PPK with deafness

Question 107

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Linear, thickened hyperkeratotic plaques over palms and volar aspects of digits; circumscribed thickening of soles

Answer 107

Striate PPK

Question 108

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Yellowish, nontransgrediens, symmetric, nummular hyperkeratotic plaques on pressure points of the soles; painful transverse fissures may be present

Answer 108

Hereditary keratosis palmoplantaris variant of Wachters

Question 109

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Thickened toenails, plantar keratoderma and plantar pain; oral mucosa may be involved, with cyst formation and natal teeth

Answer 109

Pachyonychia congenital

Question 110

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Focal yellowish thick plaques over pressure points on the palms and soles, with painful fissures; esophageal SCCs, follicular hyperkeratosis, oral leukokeratosis

Answer 110

Howel-Evans syndrome

Question 111

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Well-demarcated, focal, white-yellow painful PPK on pressure points, bilateral keratitis, mental retardation

Answer 111

Richner-Hanhart syndrome

Question 112

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Striate keratoderma; wooly hair, left ventriculopathy; lichenoid keratotic papules in flexural areas, nail abnormalities, unilateral deafness

Answer 112

Carvajal syndrome

Question 113

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Painful, hyperkeratotic papules with central indentation irregularly distributed on palms and soles

Answer 113

Punctate PPPK type I

Question 114

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Asymptomatic, firmly attached, yellow keratotic spines on palms and soles

Answer 114

Punctate PPPK type 2

Question 115

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Asymptomatic, round-oval, white-yellow translucent hyperkeratotic or umbilicated papules

Answer 115

Punctate PPPK type 3 (acrokeratoelastoidosis of Costa)

Question 116

TABLE 48-4 Summary for Palmoplantar Keratodermas DIAGNOSIS Punctate PPK, irregularly shaped, hypopigmented macules over proximal trunk, with internal organ calcification

Answer 116

Cole disease