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Fitzpatrick Tables > Chapter 48 - Inherited Palmoplantar Keratodermas > Flashcards

Chapter 48 - Inherited Palmoplantar Keratodermas Flashcards

1
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Epidermolytic PPK

A

Diffuse PPK

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2
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Unna-Thost PPK

A

Diffuse PPK

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3
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Greither syndrome

A

Diffuse PPK

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4
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Loricrin keratoderma

A

Diffuse PPK

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5
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

PPK Bothnia type

A

Diffuse PPK

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6
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Mal de Meleda

A

Diffuse PPK

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7
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Nagashima-type PPK

A

Diffuse PPK

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8
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Olmsted syndrome

A

Diffuse PPK

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9
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Vohwinkel syndrome

A

Diffuse PPK

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10
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Keratitis–ichthyosis–deafness
syndrome

A

Diffuse PPK

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11
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Bart-Pumphrey syndrome

A

Diffuse PPK

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12
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Palmoplantar Keratoderma with deafness

A

Diffuse PPK

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13
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

PPK–congenital alopecia syndrome

A

Diffuse PPK

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14
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Hidrotic ectodermal dysplasia (Clouston) syndrome

A

Diffuse PPK

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15
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Huriez syndrome

A

Diffuse PPK

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16
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Papillon-Lefevre syndrome

A

Diffuse PPK

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17
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Naxos disease

A

Diffuse PPK

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18
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome

A

Diffuse PPK

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19
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

PPK with deafness

A

Diffuse PPK

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20
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Striate PPK

A

Focal PPK

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21
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Hereditary keratosis palmoplantaris variant of Wachters

A

Focal PPK

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22
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Pachyonychia congenital

A

Focal PPK

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23
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Howel-Evans syndrome

A

Focal PPK

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24
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Richner-Hanhart syndrome

A

Focal PPK

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25
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Carvajal syndrome

A

Focal PPK

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26
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Punctate PPPK type I

A

Punctate PPK

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27
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Punctate PPPK type 2

A

Punctate PPK

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28
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Punctate PPPK type 3 (acrokeratoelastoidosis of Costa)

A

Punctate PPK

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29
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

TYPE OF PPK
(Diffuse, Focal, or Punctate)

Cole disease

A

Punctate PPK

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30
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Epidermolytic PPK

A

Without

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31
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Unna-Thost PPK

A

Without

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32
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Greither syndrome

A

Without

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33
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Loricrin keratoderma

A

Without

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34
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

PPK Bothnia type

A

Without

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35
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Mal de Meleda

A

Without

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36
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Nagashima-type PPK

A

Without

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37
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Olmsted syndrome

A

With

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38
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Vohwinkel syndrome

A

With

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39
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Keratitis–ichthyosis–deafness syndrome

A

With

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40
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Bart-Pumphrey syndrome

A

With

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41
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Palmoplantar Keratoderma with deafness

A

With

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42
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

PPK–congenital alopecia syndrome

A

With

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43
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Hidrotic ectodermal dysplasia (Clouston) syndrome

A

With

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44
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Huriez syndrome

A

With

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45
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Papillon-Lefevre syndrome

A

With

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46
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Naxos disease

A

With

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47
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome

48
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

PPK with deafness

49
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Striate PPK

50
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Hereditary keratosis palmoplantaris variant of Wachters

51
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Pachyonychia congenital

52
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Howel-Evans syndrome

53
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Richner-Hanhart syndrome

54
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Carvajal syndrome

55
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Punctate PPPK type I

56
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Punctate PPPK type 2

57
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Punctate PPPK type 3 (acrokeratoelastoidosis
of Costa)

58
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

EXTRACUTANEOUS FEATURES
(With or Without)

Cole disease

59
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Epidermolytic PPK

60
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Unna-Thost PPK

61
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Greither syndrome

62
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Loricrin keratoderma

63
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

PPK Bothnia type

64
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Mal de Meleda

65
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Nagashima-type PPK

66
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Olmsted syndrome

67
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Vohwinkel syndrome

68
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Keratitis–ichthyosis–deafness syndrome

69
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Bart-Pumphrey syndrome

70
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Palmoplantar Keratoderma with deafness

71
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

PPK–congenital alopecia syndrome

72
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Hidrotic ectodermal dysplasia (Clouston) syndrome

73
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Huriez syndrome

74
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Papillon-Lefevre syndrome

75
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Naxos disease

76
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome

77
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

PPK with deafness

A

Mitochondrial

78
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Striate PPK

79
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Hereditary keratosis palmoplantaris variant of Wachters

80
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Pachyonychia congenital

81
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Howel-Evans syndrome

82
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Richner-Hanhart syndrome

83
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Carvajal syndrome

84
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Punctate PPPK type I

85
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Punctate PPPK type 2

86
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Punctate PPPK type 3 (acrokeratoelastoidosis
of Costa)

87
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

MODE OF INHERITANCE

Cole disease

88
Q

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

A

Epidermolytic PPK

89
Q

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

A

Unna-Thost PPK

90
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Diffuse, thickened, scaly yellowish PPK, with transgrediens

A

Greither syndrome

91
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Honeycomb-like PPK, starfish-like hyperkeratosis, prominent knuckle pads and pseudoainhum

A

Loricrin keratoderma

92
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Diffuse, homogenous hyperkeratosis with a yellowish hue; aquagenic wrinkling of palms

A

PPK Bothnia type

93
Q

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

A

Mal de Meleda

94
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Most common type in Asians; transgrediens, nonprogressive, nonmutilating; well-demarcated, erythematous palmoplantar hyperkeratosis

A

Nagashima-type PPK

95
Q

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

A

Olmsted syndrome

96
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

PPK with hearing impairment; mutilating honeycomb-like PPK, with starfish shaped keratotic plaques, pseudoainhum

A

Vohwinkel syndrome

97
Q

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

A

Keratitis–ichthyosis–deafness syndrome

98
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Honeycomb-like, diffuse, sharply demarcated PPK, knuckle pads, leukonychia

A

Bart-Pumphrey syndrome

99
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Diffuse, transgrediens PPK with underlying erythema, knuckle pads, hearing impairment

A

Palmoplantar Keratoderma with deafness

100
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Well-defined, focal, nonmutilating transgrediens PPK; noncicatricial alopecia; recessive forms may have pseudoainhum and sclerodactyly

A

PPK–congenital alopecia syndrome

101
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Diffuse, velvety or cobblestone-like PPK with fissures; nail dystrophy and hair loss

A

Hidrotic ectodermal dysplasia (Clouston) syndrome

102
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Diffuse, yellowish-grey, nonerythematous PPK, scleroatrophy, sclerodactyly, SCCs

A

Huriez syndrome

103
Q

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

A

Papillon-Lefevre syndrome

104
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Diffuse, well-demarcated, nontransgrediens PPK; wooly hair; right ventriculopathy

A

Naxos disease

105
Q

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

A

Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome

106
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Orange-yellow, diffuse, well-demarcated PPK with minimal to no erythema over pressure points; hearing impairment

A

PPK with deafness

107
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Linear, thickened hyperkeratotic plaques over palms and volar aspects of digits; circumscribed thickening of soles

A

Striate PPK

108
Q

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

A

Hereditary keratosis palmoplantaris variant of Wachters

109
Q

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

A

Pachyonychia congenital

110
Q

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

A

Howel-Evans syndrome

111
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Well-demarcated, focal, white-yellow painful PPK on pressure points, bilateral keratitis, mental retardation

A

Richner-Hanhart syndrome

112
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Striate keratoderma; wooly hair, left ventriculopathy; lichenoid keratotic papules in flexural areas, nail abnormalities, unilateral deafness

A

Carvajal syndrome

113
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Painful, hyperkeratotic papules with central indentation irregularly distributed on palms and soles

A

Punctate PPPK type I

114
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Asymptomatic, firmly attached, yellow keratotic spines on palms and soles

A

Punctate PPPK type 2

115
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Asymptomatic, round-oval, white-yellow translucent hyperkeratotic or umbilicated papules

A

Punctate PPPK type 3 (acrokeratoelastoidosis of Costa)

116
Q

TABLE 48-4
Summary for Palmoplantar Keratodermas

DIAGNOSIS

Punctate PPK, irregularly shaped, hypopigmented macules over proximal trunk, with internal organ calcification

A

Cole disease

Fitzpatrick Tables (77 decks)

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