Turn over time
Cell cycle Growth fraction
Turnover time of the germinative epithelium
Epidermal turnover time (
transit time= 14 days)
The time taken for a cell to pass from basal
layer to the surface of the skin
next 14 days
Transglutaminase, TGase 1, is localized at the cell membrane.
the proteins form an insoluble structure named “cornified envelope”
the cell surface .
Filaggrin (FLG), also encoded in the EDC, is the main component of
keratohyalin granules to which the granular layer (stratum granulosum,
SG) owes its name.
Upon dephosphorylation and proteolysis of the profilaggrin precursor,
filaggrin is dispersed and causes the aggregation of the keratin
Simultaneously the nucleus is degraded and cell organelles disappear
by an unknown mechanism.
Ultimately, keratins remain as the prevailing proteins inside the
cornified envelopes strongly contributing to the mechanical resistance
of the cornified layer (stratumcorneum, SC).
In addition, keratins can also regulate pathways involved in growth,
proliferation, migration and apoptosis of epithelial cells
The epidermis is the outermost layer of the skin and is separated
from the underlying dermis by the basement membrane.
Keratinocytes, which compose the epidermis, proliferate within
the basal cell layer
As differentiation proceeds, keratinocytes progress upwards
through the different epidermal layers (the spinous layer,
granular layer and cornified layer or stratum corneum),becoming
anucleated and increasingly compacted in size, before being
eventually lost from the skin surface by desquamation (shedding
of the outer layers of skin).
Each stage of epidermal differentiation is characterised by the
expression of specific proteins,
Transglutaminases cross-link plakins and involucrin.
Other desmosomal proteins are also cross-linked
,forming a scaffold along the entire inner surface of the
High calcium level increases differentiation.
8 % of the basal cells -(K-1/K10) undergo
Orchestrated expression of keratins and subunits of
Terminal differentiation (keratinization):
Change in keratin expression .
Formation of corneocyte.
DIFFERENTIATING EPIDERMAL KERATINOCYTES
Basal layer as proliferative cells express K-5 and K-14
The process of differentiation starts with the K –
10/K-1 expression (in TA cells)
K-2 is expressed at later stages of differentiation(
1.Formation of keratin
2.Keratin filaments aggregate into bundles with the help
4.Changes in expression of
Growth factor receptors
Blood group antigens
As keratinocytes are transformed from mitotically active cells in the basal layer to fully
differentiated, enucleated squames in the cornified layer. Keratohyalin (profilaggrin- and
loricrin-containing) and lamellar (lipid-containing) granules extrude their contents in the
granular layer, leading to bundling of keratin filaments and replacement of the plasma
membrane with the highly cross-linked, lipid-covered cornified cell envelope
At the beginning of the granular
layer1. Keratohyalin granules Formation (KHG) (contains
2. Cell envelope proteins cross-linking (Involucrin and
In the spinous layer: Formation of
1. Lamellar bodies 2. KIF
As the cells enter the spinous layer
Switch of keratin synthesis
from K5/K14 to K1/K10
In the transitional zone
1. Filaggrin (keratin
bundling protein) acts as a
glue matrix that facilitates
dense packing of KIF into k.
2. activity of
3. Loss of organelles
5. extracelluar Ca++,
6. activity of
deposition of loricrin, cross
linking of involucrin
On entering the transitional zone
between the granular cell layer and
the Cornified layer
Profilaggrin is transformed into filaggrin
Corneocytes are shed into the
In upper stratum corneum
1. Formation of corneocyte bound lipid envelope
2. Plasma membrane and desmosomes become discontinuous,
corneodesmosomes are residual intercellular desmosomal
In lower stratum corneum
1. Dead keratinocytes packed with keratin macrofibrils
2. corneocyte bound protein envelope just beneath the plasma membrane
Desquamation of surface keratinocytes from the
stratum corneum is regulated by proteolytic
degradation of the cells’ desmosomes.
In response to certain signals probably an increase in calcium concentration during the
transition from the granular layers to the SC the lamellar bodies move to the apex of the
upper-most granular cells, fuse with the plasma membrane, and secrete their content into the
intercellular spaces through exocytosis.
Components of the stratum corneum
Highly insoluble cell envelope.
Present in stratum corneum.
It’s development is triggered by intracellular calcium.
Involucrin is main envelope precursor.
1. Loricin 6. Envoplakin
2. Cornifine 7. Periplakin
3. Pancornulin 8. 61KDa protein
Keratins are defined as intermediate filament forming proteins
with specific physicochemical properties produced in any
They are multigene family of proteins constituting 85% of the
total cellular protein in the cornified cells of the epidermis and
encoded by a family of approximately 30 proteins
Each keratin is characterized by a chain of amino acids as the
primary structure, which varies in the number and sequence of
amino acid as well as in polarity, charge and size.
Keratin filaments have a tripartite secondary structure
consisting of an N-terminal head domain, a central α-helical rod
domain and C-terminal tail domain and all the proteins are able
to self assemble into filaments.
Functions of keratin in the epidermis:
1. Crucial role in keratinization
2. Integral part of the structural network that make
hemidesmosomes, desmosomes, BM (Structural
3. Maintaining spatial relation between the nucleus
and cytoplasmic organelles
4. Transfer of information between the nucleus and cell
surface and vice versa i.e. cell signaling.
Any defect along this pathway
DIORDERS OF KERATINIZATION
TISSUE EXPRESSION DISEASE ASSOCITION
1 10 Suprabasal keratinocytes Bullous congenital icthyosiformis
Diffuse non epidermolytic PPK
1 9 Suprabasal keratinocytes
2 10 Upper spinous , granular Icthyosiform bullosa of siemens
3 12 cornea Meesmann’s corneal dystrophy
4 13 Mucosal epithelium White sponge nevus
5 14 Basal keratinocytes Epidermolytic bullosa complex
6a 16 Outer root
Paronychia congenita type 1 ;
Focal non-epiderdermolytic PPK
6b 17 Nail bed ,epidermal
Paronychia congenita type II,
8 18 Simple epithelium Cryptogenic cirrhosis
DISTURBANCE IN EPIDERMAL KINETICS
Enhanced cell proliferation
Enlargement of the germinative cell
Increased mitotic rates
Broadening of epidermis
DISTURBANCE IN EPIDERMAL DIFFERENTIATION
Incomplete differentiation in post mitotic phase
Faulty and accelerated cornification
Retension of of pyknotic nuclei of epidermal cells
Leads to gap between cells
Loss of barrier function of the epidermis
Morphologic presentaion of apoptosis of keratinocytes
Eosinophilic cytoplasm ,pyknotic nucleus
Cells are packed with keratin filaments
Cell will tent to round up
Loose it’s attachment with surrounding cells
Ichthyotic skin disorders
Ichthyotic skin disorders are classified into the following
• Noncongenital ichthyoses develop 4 weeks after birth and
palms and soles.
• Congenital ichthyoses present with collodion membrane
erythroderma at birth or manifest within 4 weeks.
Variants in which the skin lesions are but one facet of a
systemic illness (syndromic ichthyosis).
Ichthyosis vulgaris is characterized by deficiency of profilaggrin, a major constituent
of the keratohyalin granules.
Ultrastructurally, the keratohyalin granules are reduced, spongy or crumbly and
associated with decreased amounts of filaggrin.
Reflecting a defective epidermal synthesis of filaggrin
Filaggrin aggregates keratin intermediate filaments in the lower stratum corneum and is
to form free amino acids including urocanic and pyrrolidone carboxylic acids critical as
water-binding compounds in the stratum corneum.
the epidermal differentiation complex on chromosome 1q21 has identified mutations in
the gene encoding filaggrin
Since the filaggrin gene is a major susceptibility gene for atopic dermatitis, mutations
have also been shown in atopic dermatitis
Ichthyosis vulgaris is characterized by mild to moderate orthohyperkeratosis associated
with a hyperplastic, atrophic or normal epidermis. The key feature is a thin or absent
granular cell layer
Commonest form and also the mildest.
Inherited disorder of keratinization associated with
decreased conversion of profilaggrin to filaggrin that is
characterized by fine scaling predominantly affecting the
extensor surfaces of the extremities with sparing of the
flexures and tendency towards improvement in the
Filaggrin is an epidermal protein which is needed for
aggregation of keratin intermediate filament and retention
of moisture in the stratum corneum.
Onset : early childhood (in between 3-12 months of age)
Ichthyosis vulgaris (association
Ichthyosis vulgaris is frequently associated with
keratosis pilaris and atopic dermatitis so their C/F are
found with it, accounting keratotic lesions on on
palmer creases (keratosis punctata), Follicular
hyperkeratoses on shoulders, buttocks, thighs and
upper arms as in case of KP and hay fever, asthma,
eczema or urticaria may be presented as a
manifestation of AD.
Ichthyosis seen only in men as a result of steroid
X-Linked recessive inheritance.
Males are affected and females are asymptomatic
Onset : usually before 3 months of age.
The children are commonly born via C/S, with failure
of progression of labor owing to a placental sulfatase
deficiency and low maternal urinary estrogen level.
Large dark polygonal scales divided by wide splits
prominently on trunk and extensor extremities. The
palms and soles are nearly always spared.
The sides of the neck usually are involved giving rise to
a unwashed look (dirty neck)
Ocular involvement : Corneal opacity.
Cryptorchidism, testicular carcinoma.
The disease is associated with a deficiency of the microsomal enzyme, steroid
sulfatase/STS (sterol sulfate sulfohydrolase/arylsulphatase C).
This is a membrane-bound enzyme, which hydrolyses the 3-â-sulfate esters of
cholesterol and the sulfated steroid hormones
It is characterized by a raised serum cholesterol sulfate.
The corneocytes contain excess cholesterol 3-sulfate and
diminished free sterol.
Steroid sulfatase deficiency possibly results therefore in persistence of the lipid
contents of the membrane-coating granules and hence increased or persistent
adhesion between adjacent keratin plates in the stratum corneum.
Increased amounts of cholesterol sulfate may inhibit the epidermal serine protease
activity, which results in retention of corneodesmosomes leading to less shedding of
scales and retention hyperkeratosis.
The gene locus for recessive X-linked ichthyosis is within the Xp22.3 region of the X
Lesions show non-specific features of compact hyperkeratosis and slight acanthosis
associated with a granular cell layer, which may be normal or increased in thickness
Present at birth or appears soon after.
Usually involves the entire cutaneous area.
It is a severe form of Ichthyosis and also is very
Decreased or absent transglutaminase-1 activity.
Onset : Birth- collodion baby.
Lamellar Ichthyosis (C/F)
H/O a collodion-like (a colourless or yellow syrupy liquid)
membrane encasing the baby at birth which desquamates
over the first 2/3 weeks.
Scales : Thick dark (grayish-brown), strikingly quadrangular,
free at edges and adherent at centre; tend to be largest at
extremities where these large plate-like scales are separated
by superficial fissuring (similar to a dry river bed).
Involvement of palm and soles : Ranges from minimal hyper-
linearity to severe keratoderma.
Ectropion and eclabium : Ectropion is the turning out of the
eyelid so that the inner surface is exposed.
Eclabium is eversion of a lip.
Tautness of facial skin is responsible for these.
A number of forms of ichthyosis present at birth with
infant encased in a tight membrane of adherent
keratinocytes, which has been compared to parchment
Kollodes is the Greek word for glutinous or glue-like.
The membrane is then shed, leaving either normal
skin (lamellar exfoliation of newborn) or, more often,
one of the forms of nonbullous congenital
ichthyosiform erythroderma or lamellar ichthyosis.
Rare severe ichthyosis presenting at birth.
All three enzymes have autosomal recessive inheritance have
Tranglutaminase-1 (TGM1) at 14q11.2; also involved in lamellar ichthyosis.
Two lipoxygenases at 17p13.1 (ALOX12B and ALOXE3).
Frequently born as collodion baby.
Fine white scales and erythroderma. Also ectropion and scarring
Nail dystrophy, short stature, cardiac malformations.
Evolve of the word ‘Harlequin’ : Harlequin or Arlecchino in Italian or
Arlequin in French is the most popularly known of the comic servant characters from
the Italian Commedia dell'arte and its descendant, the Harlequinade. In French passion plays
Hellequin, a black-faced emissary of the devil, is said to have roamed the countryside with a group
of demons chasing the damned souls of evil people to Hell.
Synonym: Ichthyosis congenita gravis.
A severe disorder that affects the skin in utero, causing thick, horny, armor-
like plates covering the entire surface.
Ears are rudimentary or absent, eclabium and ectopion are severe.
Abnormalities of profilaggrin, K6 and K16 expression have been reported.
Recessive inheritance has been favoured and is supported by reports of
Usually the child is stillborn or dies soon after delivery; although there are
reports of a few survivors, with lifelong systemic retinoids.
Bullous Ichthyosiform Erythroderma (Borcq)
Synonym: Epidermolytic hyperkeratosis.
Uncommon generalized disorder with blisters and
Mutations in keratin1 and 10 genes.
There is altered assembly process of cornified cell envelopes.
It has been described as an incidental finding in normal skin,
skin adjacent to epidermal tumor (both benign and
malignant) and normal oral mucosa.
Epidermolytic hyperkeratosis (C/F)
At birth, widespread blisters and erosions; child looks as if
Then development of distinctive dirty, spiny, hyperkeratotic
lesions, often scattered on an erythematosus background;
most often in flexures.
Palmoplantar keratoderma common.
Epidermolytic hyperkeratosis skin is usually has a
characteristic pungent odor, thought to be related to super-
infection by mixed flora.
1) Ichthyosis vulgaris : Histology reveals mild hyperkeratosis with an
reduced/absent granular layer; normal thickness of spongy layer,
normal dermis. Electron microscopy : keratohayalin granules.
2) X-linked Ichthyosis : Elevated plasma cholesterol sulfate level or
lipoprotein electrophoresis showing increasing motility of low-
density lipoproteins (LDLs).
3) Lamellar Ichthyosis : The transglutaminase-1 can be stained in
frozen sections of skin; histology shows orthokeratotic
hyperkeratosis and mild to moderate acanthosis.
4) Epidermolytic hyperkeratosis : H/E shows compact hyperkeratosis.
Granular layer is markedly thickened and contains coarse
keratohyaline granules. Electron microscopy : Perinuclear haloes.
Features of different types of
Features IV X-linked
Inheritance AD X-linked AR AD
Severity Mild Moderate Severe Becomes less
severe with age
Defect Flaggrin protein Steroid
Distribution All over body All over body
All over body,
palms and sole
All over body,
Features of different types of
Features IV X-linked
Onset 3-12 months of
months of age
Spared areas Flexures and
Other features Fine scales,
Treatment Emollients Emollients Retinoids-
oral retinois +
Prognosis Good Good Causes serious
Ichthyosis Linearis Circumflexa
Inherited autosomal-recessive disorder.
Migratory annular and polycyclic patches occur.
May first appear as generalized exfoliative
erythroderma; later lesions predominate on trunk and
extrimities, appear as polycyclic patches characterized
by constantly changing patterns.
Congenital reticular ichthyosiform
erythroderma (ichthyosis ‘en confettis’ /ichthyosis variegata)
The patients are born with congenital ichthyosiform
During childhood the integument clears gradually so
that enlarging patches of normal skin appear to be
enclosed by erythrokeratotic and hyperpigmented
areas in a reticular arrangement.
Associated features : hypertrichosis, and palmoplantar
hyperkeratosis, hypogonadism, growth retardation,
hepatomegaly, keratoacanthoma or squamous cell
Congenital reticular ichthyosiform
erythroderma (ichthyosis ‘en confettis’ /ichthyosis variegata)
Histologically: there is psoriasiform hyperplasia.
The horny layer is thickened and parakeratotic.
The parakeratotic corneocytes have enlarged nuclei.
keratinocytes of the upper layers : prominent
perinuclear vacuolation and contain few keratohyalin
Their cell borders are well defined an intracytoplasmic
eosinophilic granules are absent. Some of the
vacuolated keratinocytes are binucleated.
keratin 2e is missing, the other epidermal keratins are
There is hyperkeratosis and well-developed psoriasiform
There is parakeratosis with prominent nuclei. Note the cytoplasmic
vacuolation. Eosinophilic intracytoplasmic inclusions are absent
Sjögren–Larsson syndrome (SLS) is an autosomal recessive
disorder characterized by congenital ichthyosis, mental
retardation, and spastic paresis
long-chain fatty alcohol is deposited in cultured
fibroblasts, whin blood cells, and serum in SLS
mutations in the fatty aldehyde dehydrogenase (FALDH)
gene (ALDH3A2) were responsible for the development of
SLS. However, the exact pathomechanisms of this
ichthyosis in SLS is not fully understood.
(A) Histopathology of ichthyotic skin lesion of S L syndrome. Orthohyperkeratosis with mild hypergranulosis was
(B) Ultrastructurally, at the stratum granulosum/stratum corneum interface, abnormal apparently empty lamellar
granules (white arrowheads) were seen in the granular cells and lipid vacuoles (white arrows) were observed in
the cornified cells.
(C–E) Vacuoles, presumably lipid droplets (white arrows) and irregularly shaped abnormal intercellular materials
(black arrows) were apparent in the stratum corneum layers. Scale bars=50 (A) and 0.3 m (B–E).
Other name : Senter syndrome.
Vascularization of cornea, deafness, hyperkeratotic
palms and soles, hypotrichosis, partial anhydrosis, nail
dystrophy and tight heel cords are the characteristic
Treatment with acitretin (isotretinoin exacerbate
corneal vascularization), cyclosporin A eye drop.
Congenital hemidysplasia with ichthyosiform
erythroderma and limd defects.
Unilateral inflammatory nevi and ipsilateral limb
X-linked dominant and lethal in hemizygous male.
H/E : presence of foamy macrophages in dermal
Vitamin deficiency: Vitamin A, vitamin B6, and nicotinic acid
Infections: Leprosy, tuberculosis, syphilis.
Medications: nicotinic acid (most common), triparanol,
Systemic diseases : Sarcoidosis, hypothyroidism, lupus
Malignancy : lymphoma specially Hodgkin’s lymphoma; also
occurs in NHL, mycosis fungoids, multiple myeloma.
Caution: Whenever ichthyosis appears in adult life for the
first time, exclude an underlying malignancy.
Severe xerosis in the elderly.
Pityriasis rotunda (pityriasis circinata)
Patients present with persistent, very sharply defined,
circular or oval areas of hyper- or hypopigmentation
associated with a fine scale.
The sex incidence : equal
Lesions: multiple and frequently numerous,
,characteristically noninflammatory and asymptomatic
,often, confluent, measuring 0.5–28 cm in diameter ,
located on the trunk and limbs&sometimes associated with
gradual remission during the summer months and relapse
Acutaneous marker of severe internal disease:tuberculosis,
cancer (particularly hepatoma), leukemia,cirrhosis, ovarian
and uterine disease.
The histological features :hyperkeratosis with a diminished
or absent granular cell layer and loss of the epidermal ridge
Peeling skin syndrome
characterized by a spontaneous, lifelong peeling of the
stratum corneum without bleeding or pain. The mode
of inheritance is autosomal recessive.
Three types can be distinguished:
1. type A a generalized continued shedding or peeling
of the entire skin without signs of inflammation or
other symptoms is present from birth or develops
2. Type B appears, resembles and is characterized by
isolated erythematous lesions which then peel,
leaving burning superficially denuded red patches
with a peripheral collarette. A mutation of
corneodesmosin has been identified.
Peeling skin syndrome
3-In type C (acral peeling skin syndrome), involvement
is confined to the backs of the hand and feet
A homozygous missense mutation in the gene of
transglutaminase-5 has been identified.
Histological features of peeling skin syndrome
Type A: a plane of separation either within the lower
part of an otherwise normal horny layer or above the
granular cell layer. an intracellular splitting is within
Type B: epidermis is psoriasiform with an absent or
reduced granular cell layer and marked parakeratosis.
The split is at the level of the granular cell layer.
Type C peeling skin syndrome: the horny layer is
detached from the stratum granulosum
Peeling skin syndrome: the biopsy is taken from the edge of the lesion. the stratum
corneum is clearly separated from the underlying epidermis.
Lesions usually present soon after birth or during the first year of life and are of
two types, typically present simultaneously:
• Type 1 lesions : ymmetrically distributed, discrete figurate, and often bizarre
patches of erythema, which vary in size, shape, number, and location over
periods of hours and days . These are sometimes temperature or stress related.
Type 2 lesions : well-defined, fixed geographical, reddish-yellowbrown greasy,
hyperkeratotic plaques arising either within the erythematous lesions or, more
often, independently.,asymptomatic, mild pruritus or burning sensations
The condition particularly affects the face, buttocks, and extensor surfaces of
Occasionally associated with high estrogen levels ,Hypertrichosis (of vellus
hairs) and mildkeratoderma of the palms and soles
Erythrokeratoderma variabilis harbor s connexin(Cx) 31 or Cx30.3 mutations
The histopathological features : not specific, orthohyperkeratosis, variable
parakeratosis, irregular acanthosis, and papillomatosis with an undulating skin
surface. Dyskeratotic cells with pyknotic nuclei reminiscent of the grains of
Darier .The granular cell layer appears normal. A perivascular
lymphohistiocytic inflammatory cell infiltrate may be present in the superficial
variabilis: in these lesions
there is more pronounced
Progressive symmetric erythrokeratodermia(Gottron's
Inherited as an autosomal dominant with incomplete
penetrance.Both sexes are equally affected
Presents in the first year of life with fixed symmetrical,
and sometimes pruritic, erythematous scaly plaques
lacking transient migratory erythema .
On the extensor surfaces including the elbows, knees,
buttocks, dorsal surfaces of the feet and hands, and
The face, chest, and abdomen are typically unaffected.
Additional features : palmoplantar keratoderma and
pseudoainhum(constriction bands on the fingers and
Pathogenesis and histological features
A mutation in the loricrin gene on chromosome 1q21
A connexin gene disorder(?).
Histologically:marked basket-weave hyperkeratosis with
focal parakeratosis hypergranulosis, and psoriasiform
1. Paranuclear vacuolation :in the granular cell layer.
2. A perivascular lymphocytic infiltrate is present in the
3. loricrin-rich intranuclear granules in the granular cell
4. Lamellar granules are increased in number
5. lipid droplets may be evident in the cornified cells
Pachyonychia congenita type I
Focal (nonepidermolytic) palmoplantar keratoderma
with oral hyperkeratosis (Jadassohn-Lewandowsky
syndrome, focal palmoplantar keratoderma with oral
hyperkeratosis, palmoplantar ectodermal dysplasia
type I) :an autosomal dominant mode of inheritance
The features : massive hyperkeratosis of the distal nail
beds of the fingers and toes, resulting in elevation and
apparent thickening of the nail plate.
Associations :Palmoplantar keratoderma,
hyperhidrosis follicular keratosis, xerosis, and
verrucous lesions on the elbows, knees, and lower legs.
Mutations :in keratin K16 and K6a genes.
Pachyonychia congenita type 1: there is gross nail deformity with transverse arching of the distal portion.
of the ostium
Pachyonychia congenita type II
Pachyonychia congenita type II (palmoplantar
ectodermal dysplasia type II, Jackson-Lawler
syndrome, Jackson-Sertoli syndrome) :an autosomal
Mild focal palmoplantar keratoderma over pressure
areas, subungual hyperkeratosis, epidermal cysts,
steatocystoma multiplex, abnormal eyebrows and
body hair (pili torti), natal teeth, angular cheilosis, and
Mutations in kerat in 17 and keratin 6b genes.
mutations in keratin 17 may also result in
steatocystoma multiplex in isolation
Acrokeratosis verruciformis of Hopf
An autosomal dominant mode of inheritance.
In infancy or early childhood as dry, rough, brownish
or skin-colored verrucoid, keratotic papules.
located particularly on the backs of the hands and feet,
and on the knees and elbows.
Loss of function of the sarco- (endo-) plasmic
reticulum Ca2+ ATP ase2 mutant in acrokeratosis
verruciformis provides evidence that acrokeratosis
verruciformis and Darier's disease are allelic disorders.
The lesions are acanthotic with a prominent granular
cell layer, typically showing a ‘church spire’ appearance
Hyperkeratosis lenticularis perstans((Flegel's disease)
Equal sex incidence in fourth or fifth decade.
large numbers of 1–5-mm discrete, gray, graybrown or red-
brown, circular scaly papules.
Initial lesions :on the dorsum of the foot, the lower legs,
upper arms, and pinnae,buttocks, trunk, and dorsal aspects
of the hands with punctate keratoses on the palms and
Asymptomatic or mildly pruritic.
Early lesions :lamellar hyperkeratosis, focal parakeratosis,
and an essentially normal epidermis.
An established lesion: hyperkeratosis ¶keratosis,
inconspicuous or absent granular cell cell, intercellular
edema , foci of basal cell degeneration and a chronic
inflammatory cell infiltrate a perivascular or lichenoid
Lesions are small, multiple
and covered by a well-developed
(A) scanning view of an
established lesion showing
hyperkeratosis, parakeratosis, and
a superficial bandlike chronic
hyperkeratosis, focal epidermal
atrophy and basal cell
liquefactive degeneration. Note the
high-power view showing
spongiosis with microvesiculation,
cytoid bodies, and a
The lymphocytes are an admixture of CD4+ T-helper cells
and, less frequently CD8+ T-suppressor cells.. Sézary-like
forms have been described. Langerhans cells are highly
In the atrophic areas:
1. cytokeratin 1 &10 , filaggrin, and loricrin are absent.
2. Rudimentary keratohyalin granules, absence,
3. vacuolation or abnormally lamellated membrane coating
4. failure to form a compact keratin, and cornified envelope
in the corneocytes
It affects the axillae intertriginous areas including
submammary and intermammary skin, groins, vulva,
perianal region and, lower back, buttocks, and flanks.
In women than males. the middle aged to elderly; children
are rarely involved.
It presents as pruritic or burning erythematous,
hyperpigmented, and hyperkeratotic patches, papules, or
As a result of a contact reaction to an antiperspirant or
creams, shampoos, and soaps.
A failure to transform profilaggrin to filaggrin with the
resultant failure in degradation of keratohyalin granules.
A massive hyperkeratosis with parakeratosis and
retention of keratohyalin granules in the stratum
The underlying epidermis :acanthosis or even some
degree of thinning. Hair infundibula are occasionally
Necrotic areas with invasion of neutrophils or
perforation of the epidermis are rarely found.
The superficial dermis contains a sparse perivascular
(A) there is marked thickening of the
horny layer with parakeratosis
(B) high-power view showing retention
of the keratohyalin granules.
Hereditary disorder of keratinization
characterized by expanding atrophic anular
patch(es) surrounded by prominent
keratotic ridge called the cornoid
NOT inherited as a primary genetic condition.
They may occur as part of a generalised
skincondition(some of which may
be inherited) or as a result of another
More likely to present in adulthood
CAUSES OF ACQUIRED KERATODERMA
I. INFLAMMATORY SKIN CONDITIONS
III. CIRCULATORY PROBLEMS
IV. 2ry TO INHERITED CONDITIONS THAT MAY
NOT USUALLY RESULT IN PPK
V. DRUGS AND TOXINS
VI. INTERNAL DISORDERS
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