Chapter 48 - Inherited Palmoplantar Keratodermas Flashcards
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Epidermolytic PPK
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Unna-Thost PPK
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Greither syndrome
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Loricrin keratoderma
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
PPK Bothnia type
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Mal de Meleda
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Nagashima-type PPK
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Olmsted syndrome
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Vohwinkel syndrome
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Keratitis–ichthyosis–deafness
syndrome
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Bart-Pumphrey syndrome
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Palmoplantar Keratoderma with deafness
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
PPK–congenital alopecia syndrome
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Hidrotic ectodermal dysplasia (Clouston) syndrome
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Huriez syndrome
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Papillon-Lefevre syndrome
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Naxos disease
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
PPK with deafness
Diffuse PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Striate PPK
Focal PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Hereditary keratosis palmoplantaris variant of Wachters
Focal PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Pachyonychia congenital
Focal PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Howel-Evans syndrome
Focal PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Richner-Hanhart syndrome
Focal PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Carvajal syndrome
Focal PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Punctate PPPK type I
Punctate PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Punctate PPPK type 2
Punctate PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Punctate PPPK type 3 (acrokeratoelastoidosis of Costa)
Punctate PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
TYPE OF PPK
(Diffuse, Focal, or Punctate)
Cole disease
Punctate PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Epidermolytic PPK
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Unna-Thost PPK
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Greither syndrome
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Loricrin keratoderma
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
PPK Bothnia type
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Mal de Meleda
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Nagashima-type PPK
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Olmsted syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Vohwinkel syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Keratitis–ichthyosis–deafness syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Bart-Pumphrey syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Palmoplantar Keratoderma with deafness
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
PPK–congenital alopecia syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Hidrotic ectodermal dysplasia (Clouston) syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Huriez syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Papillon-Lefevre syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Naxos disease
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
PPK with deafness
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Striate PPK
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Hereditary keratosis palmoplantaris variant of Wachters
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Pachyonychia congenital
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Howel-Evans syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Richner-Hanhart syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Carvajal syndrome
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Punctate PPPK type I
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Punctate PPPK type 2
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Punctate PPPK type 3 (acrokeratoelastoidosis
of Costa)
Without
TABLE 48-4
Summary for Palmoplantar Keratodermas
EXTRACUTANEOUS FEATURES
(With or Without)
Cole disease
With
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Epidermolytic PPK
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Unna-Thost PPK
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Greither syndrome
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Loricrin keratoderma
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
PPK Bothnia type
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Mal de Meleda
AR
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Nagashima-type PPK
AR
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Olmsted syndrome
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Vohwinkel syndrome
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Keratitis–ichthyosis–deafness syndrome
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Bart-Pumphrey syndrome
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Palmoplantar Keratoderma with deafness
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
PPK–congenital alopecia syndrome
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Hidrotic ectodermal dysplasia (Clouston) syndrome
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Huriez syndrome
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Papillon-Lefevre syndrome
AR
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Naxos disease
AR
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome
AR
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
PPK with deafness
Mitochondrial
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Striate PPK
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Hereditary keratosis palmoplantaris variant of Wachters
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Pachyonychia congenital
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Howel-Evans syndrome
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Richner-Hanhart syndrome
AR
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Carvajal syndrome
AR
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Punctate PPPK type I
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Punctate PPPK type 2
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Punctate PPPK type 3 (acrokeratoelastoidosis
of Costa)
AD
TABLE 48-4
Summary for Palmoplantar Keratodermas
MODE OF INHERITANCE
Cole disease
AD
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
Epidermolytic PPK
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
Unna-Thost PPK
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Diffuse, thickened, scaly yellowish PPK, with transgrediens
Greither syndrome
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Honeycomb-like PPK, starfish-like hyperkeratosis, prominent knuckle pads and pseudoainhum
Loricrin keratoderma
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Diffuse, homogenous hyperkeratosis with a yellowish hue; aquagenic wrinkling of palms
PPK Bothnia type
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
Mal de Meleda
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Most common type in Asians; transgrediens, nonprogressive, nonmutilating; well-demarcated, erythematous palmoplantar hyperkeratosis
Nagashima-type PPK
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
Olmsted syndrome
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
PPK with hearing impairment; mutilating honeycomb-like PPK, with starfish shaped keratotic plaques, pseudoainhum
Vohwinkel syndrome
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
Keratitis–ichthyosis–deafness syndrome
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Honeycomb-like, diffuse, sharply demarcated PPK, knuckle pads, leukonychia
Bart-Pumphrey syndrome
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Diffuse, transgrediens PPK with underlying erythema, knuckle pads, hearing impairment
Palmoplantar Keratoderma with deafness
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Well-defined, focal, nonmutilating transgrediens PPK; noncicatricial alopecia; recessive forms may have pseudoainhum and sclerodactyly
PPK–congenital alopecia syndrome
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Diffuse, velvety or cobblestone-like PPK with fissures; nail dystrophy and hair loss
Hidrotic ectodermal dysplasia (Clouston) syndrome
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Diffuse, yellowish-grey, nonerythematous PPK, scleroatrophy, sclerodactyly, SCCs
Huriez syndrome
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
Papillon-Lefevre syndrome
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Diffuse, well-demarcated, nontransgrediens PPK; wooly hair; right ventriculopathy
Naxos disease
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
Palmoplantar hyperkeratosis with SCC of skin and sex reversal syndrome
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Orange-yellow, diffuse, well-demarcated PPK with minimal to no erythema over pressure points; hearing impairment
PPK with deafness
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Linear, thickened hyperkeratotic plaques over palms and volar aspects of digits; circumscribed thickening of soles
Striate PPK
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
Hereditary keratosis palmoplantaris variant of Wachters
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
Pachyonychia congenital
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
Howel-Evans syndrome
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Well-demarcated, focal, white-yellow painful PPK on pressure points, bilateral keratitis, mental retardation
Richner-Hanhart syndrome
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Striate keratoderma; wooly hair, left ventriculopathy; lichenoid keratotic papules in flexural areas, nail abnormalities, unilateral deafness
Carvajal syndrome
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Painful, hyperkeratotic papules with central indentation irregularly distributed on palms and soles
Punctate PPPK type I
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Asymptomatic, firmly attached, yellow keratotic spines on palms and soles
Punctate PPPK type 2
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Asymptomatic, round-oval, white-yellow translucent hyperkeratotic or umbilicated papules
Punctate PPPK type 3 (acrokeratoelastoidosis of Costa)
TABLE 48-4
Summary for Palmoplantar Keratodermas
DIAGNOSIS
Punctate PPK, irregularly shaped, hypopigmented macules over proximal trunk, with internal organ calcification
Cole disease