Disorders of epidermal maturation and keratinization

Published on 08/03/2015 by admin

Filed under Pathology

Last modified 22/04/2025

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Disorders of Epidermal Maturation and Keratinization

Major keratins and specific locations 194
Ichthyoses 194

Ichthyosis vulgaris 194
X-linked ichthyosis 195
Collodion baby differential 195

Ichthyosis congenita 196

Congenital ichthyosiform erythroderma 196
Lamellar ichthyosis 196
Bullous ichthyosis 197
Ichthyosis bullosa of Siemens 197
Netherton’s syndrome 198
Erythrokeratoderma variabilis 198
Progressive symmetric erythrokeratoderma 199
Harlequin fetus 199
Follicular ichthyosis 199
Acquired ichthyosis 199
Pityriasis rotunda 199
Refsum’s syndrome 200

Other ichthyosis-related syndromes 200

Sjögren-Larsson syndrome 200
“KID” syndrome 200
Conradi-Hünermann-Happle syndrome 201
“CHILD” syndrome 201
Tay’s syndrome 201
IBIDS 201
Multiple sulfatase deficiency 201
“MAUIE” syndrome 201
Neutral lipid storage disease 201
Shwachman syndrome 201

Palmoplantar keratoderma and related conditions 202

Palmoplantar keratoderma 202
Unna-Thost syndrome 202
Greither’s syndrome 202
Olmsted’s syndrome 202
Vohwinkel’s syndrome 202
Vorner’s syndrome 203
Howel-Evans syndrome 203
Papillon-Lefèvre syndrome 203
Mal de Meleda 203
PPK + woolly hair conditions 204
Punctate palmoplantar keratoderma 204
Striate palmoplantar keratoderma 204
Circumscribed keratoderma 204
Acquired keratoderma 204
Arsenic keratosis 205
Aquagenic acrokeratoderma 206
Oculocutaneous tyrosinosis 207
Acrokeratoelastoidosis 207
Pachyonychia congenita 208

Cornoid lamellation 208

Porokeratosis of Mibelli 208
Disseminated superficial actinic porokeratosis 209
Linear porokeratosis 209
Punctate porokeratotic keratoderma 210

Epidermolytic hyperkeratosis 210

Epidermolytic hyperkeratosis 210
Epidermolytic acanthoma 211

Acantholytic dyskeratosis 211

Focal acantholytic dyskeratosis 211
Acantholytic acanthoma 212
Darier’s disease 212
Galli-Galli disease 213
Grover’s disease 213
Persistent acantholytic dermatosis 214
Hailey-Hailey disease 214
Warty dyskeratoma 215

Discrete keratotic lesions 215

Flegel’s disease 215
Kyrle’s disease 216
Multiple minute digitate keratoses 216
Waxy keratoses 216

Miscellaneous epidermal genodermatoses 216

Acrokeratosis verruciformis of Hopf 216
Xeroderma pigmentosum 217
Ectodermal dysplasias 217
Nevoid hyperkeratosis of the nipple 218
Peeling skin syndrome 218

Miscellaneous disorders 219

Granular parakeratosis 219
White sponge nevus 219

Ichthyosis vulgaris

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Major keratins and specific locations

• K1-8 (type II) = basic and high molecular weight (chromosome 12)
• K9-20 (type I) = acidic and low molecular weight (chromosome 17)
• Upper spinous and granular layer = K2e and K10
• Stratum spinosum = K1 and K10
• Basal layer = K5 and K14
• Cornea = K3 and K12
• Mucosa = K4 and K13
• Palms/soles = K6 and K16 (also in psoriasis, warts, AK, SCC)

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Disorders of Epidermal Maturation and Keratinization

Major keratins and specific locations 194
Ichthyoses 194

Ichthyosis vulgaris 194
X-linked ichthyosis 195
Collodion baby differential 195

Ichthyosis congenita 196

Congenital ichthyosiform erythroderma 196
Lamellar ichthyosis 196
Bullous ichthyosis 197
Ichthyosis bullosa of Siemens 197
Netherton’s syndrome 198
Erythrokeratoderma variabilis 198
Progressive symmetric erythrokeratoderma 199
Harlequin fetus 199
Follicular ichthyosis 199
Acquired ichthyosis 199
Pityriasis rotunda 199
Refsum’s syndrome 200

Other ichthyosis-related syndromes 200

Sjögren-Larsson syndrome 200
“KID” syndrome 200
Conradi-Hünermann-Happle syndrome 201
“CHILD” syndrome 201
Tay’s syndrome 201
IBIDS 201
Multiple sulfatase deficiency 201
“MAUIE” syndrome 201
Neutral lipid storage disease 201
Shwachman syndrome 201

Palmoplantar keratoderma and related conditions 202

Palmoplantar keratoderma 202
Unna-Thost syndrome 202
Greither’s syndrome 202
Olmsted’s syndrome 202
Vohwinkel’s syndrome 202
Vorner’s syndrome 203
Howel-Evans syndrome 203
Papillon-Lefèvre syndrome 203
Mal de Meleda 203
PPK + woolly hair conditions 204
Punctate palmoplantar keratoderma 204
Striate palmoplantar keratoderma 204
Circumscribed keratoderma 204
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