follicular type begins on the face in infancy.
follicular plugs, which may be filiform, are present from infancy or
develop in early childhood on the nose, cheeks and later on the
limbs and neck. The keratosis is succeeded by atrophy, which gives
rise to atrophoderma on the cheeks.
alopecia of scalp and eyebrows starting during childhood or up to
the early teenage years is characteristic of the disorder. Some
cases may show an associated photophobia, corneal opacity, deafness,
nail deformities and mental retardation. There may be numerous
This is a
disease of abnormal keratinization that appears at birth or in early
begin usually on the face mainly on the eyebrows as erythematous
lesions with small horny plugs that extend to the cheeks and
peripherally. Hair follicles may be destroyed leaving areas of
alopecia of the eyebrows.
agent, using low concentration of salicylic acid; 2 per cent in 20
per cent urea topically.
icthyosis has the same skin manifestations as the other types but it
is caused by different diseases.
may be associated with other manifestations such as anhidrosis,
malabsorption and liver diseases.
diagnosis of an itchy icthysiform skin lesion in infancy or early
childhood should exclude the possibility of Hodgkin‘s disease.
different diseases that have icthysiform manifestations besides the
other manifestations are:
reaction as a reaction to medications used for lowering cholesterol.
rubra piliaris (PRP) is a chronic inflammatory disease characterized
by fine acuminate, horny, follicular papules that affects both sexes
equally and occurs at any age.
The cause of
the disease is unknown. The essential abnormality appears to be
epidermal over activity. Genetic factors have been considered since
the disease is transmitted as an autosomal dominant trait.
PITYRIASIS RUBRA PILIARIS
pityriasis rubra piliaris of infants and young children may have a
different clinical picture than that in older children and adults.
The onset of
the disease begins usually between the ages of 5 and 10 years.
usually begins in early childhood as an erythematous slightly scaly
macules and papules associated with follicular and perifollicular
central keratotic plugging.
Fig. 366. Pityriasis Rubra Piliaris
Fig. 367. Pityriasis Rubra Piliaris
condition is exceedingly chronic followed sometimes by infection.
Spontaneous clearing is usually within two years or may progress to
generalized exfoliate dermatitis leaving dull, red glazed, atrophic
skin that is very sensitive for minor stimuli.
is usually the first site involved that presents with erythema and
scaling, leaving cicatricial alopecia after healing of the lesions.
sites involved are the sides of the neck, trunk, extensor surfaces
of the limbs, the back of the hands, palms and soles, which show
may be thickened, dull rough, brittle and become striated in long
appearance of the
skin due to involvement of extensive area, presenting with sharply
with keratotic follicular plugs. Parakeratosis, irregular
acanthosis, liquefaction degeneration of the basal layer and
infiltrate with mast and plasma cells around the dilated blood
lines of treatment are used in the treatment of pityriasis rubra
piliaris but most have disappointing results.
steroids: alone or
in combination with salicylic acid may give some improvement in the
dry scaly lesions, especially those involving palms and soles.
steroids: can be
used especially in generalized cases with manifestations of
drugs: these drugs
should be used under certain strict conditions. These are indicated
in older age groups, in reluctant cases and in lesions, which are
not responding to other lines of treatments.
oral retinoids may give good results.
may improve some
In high doses is believed to give encouraging results, but care of
hypervitaminosis. A which may elicit a skin eruption.
these may carry hazardous side effects, especially in infants. The
results may be unencouraging.
that this line of treatment should not be used and not recommended
in young age group.
This is a
genetic disorder of abnormal keratinization which is characterized
by persistent eruption of hyperkeratotic papules mainly on the
appear mainly on the seborrheic areas of the face, scalp margins,
forehead, ears, nasio-labial folds, upper chest and back.
disease presents with several clinical types:
skin lesions are
firm greasy, crusted papules, skin colored, yellowish or brown that
may be misdiagnosed as acne or seborrheic dermatitis. If the
adherent crust is removed, a central funnel-shaped orifice may
sometimes be exposed.
lesions: show heavy
crusted papules like seborrhea, but has a characteristic spiny feel
mainly on the anogenital region, the groins and the natal cleft.
Coalescence of the papules produces irregular warty plaques or
papillomatous masses, which in the flexures become vegetative and
lesions may simulate seborrheic dermatitis, fungal infections or
psoriasis of the flexural areas.
and soles: may show
hemorrhagic macules and punctate keratoses or minute pits.
are characteristics either showing white or red longitudinal bands
or V shaped red and white bands at the free margin of the nail.
are white umbulicated or “cobblestone“ papules on the palate,
tongue, buccal mucosa, epiglottis, and pharyngeal wall simulating
leucoplakia vulva, esophagus or rectum.
Patients with Darrier‘s disease appear to have an increased
susceptibility to herpes simplex infections, Pox virus infection
Kaposi‘s varicelliform eruption and increased incidence of chronic
histology shows a distinctive form of dyskeratosis, with corps ronds
and supra basal acantholysis.
patients with mild disease require no treatment other than a simple
emollient and instructed to expose affected areas to sun.
with more severe disease, the use of etretinate or isotretinoin
usually results in significant improvement.
may give good results. The dose is 0.5-1.0 mg/kg/d.
1-2 mg/kg/d is another medication that can help some cases.
by CO2 Laser and Dermabrasion can be used for severe warty cases
especially in intertriginous hypertrophic types.
verruciformis is a disease of abnormal keratinization inherited as
an autosomal dominant, but sporadic cases also occur.
affects both sexes and is usually present at birth or appears in
warty papules, which are flat or convex, present on the back of the
hands, feet, knees, elbows, and on the forearms. Small groups or
isolated papules may develop in other sites.
which may be diffusely thickened, show small keratoses and
punctiform breaks in the dermatoglyphic pattern.
the lesions may cause blister formation.
may be thickened and white.
lesions may appear in the first few months of life and is usually
obvious by the age of four.
are even, very thick, yellow hyperkeratotic patches that involve the
whole foot, starting on the heel and anterior arch, spreading later
to the palms causing thickening of nails.
hyperhidrosis is common and usually predispose to fungal infections.
This is a
rare syndrome that runs in some families having marriages from their
keratoderma is characterized by extension onto the dorsal surfaces
of the hands and feet and over the knees and elbows. The condition
is associated with eczema that is often secondarily infected. In
early infancy, scaling and thickening soon follow redness of palms
and soles. This is usually diffuse but sometimes appear in islands,
extending to the dorsal surfaces in a glove-like distribution.
thickening or koilonychia,
alone or in combination with steroids topically under occlusion may
give good results.
Fig. 368. Mal de Meleda
Fig. 369. Mal de Meleda
Fig. 370. Mal de Meleda
ointment may give temporary improvement.
may be of some value.
This is an
inherited disease of abnormal keratinization affecting infants and
thickening of the palms and soles simulating psoriasis.
that may cause an unpleasant odor.
usually normal but may be sparse.
resulting in severe gingivitis that may predispose to loss of the
teeth . The permanent teeth may be lost in the same fashion.
calcification, especially in the attachment of the tentorium and
choroid, has been noted in some cases.
represent variants of abnormal keratinization, which are more common
condition may be congenital or acquired.
may appear early in young age as a familial type or may be acquired
in response to repeated trauma or friction as by tight shoes over
the bony prominence of the palms or soles. The most common site is
over the head of the third metatarsals where the lesion may be
mistaken as skin wart.
show a central degenerative core in the middle of dense
hyperkeratosis, which should be distinguished from the black
thrombotic vessels of the verruca.
callosities respond to local application of salicylic acid (20%) and
lactic acid (20%) in collodion base for few days. Before each
application the dead tissues can be removed by shaving.
cases can be resurfaced by CO2 laser.
nigricans is an inherited genodermatosis that is characterized by
hyperkeratosis and skin pigmentation. The affected skin is covered
by papillomatous elevations, which give it a velvety texture.
different clinical types of acanthosis nigricans:
benign type: affects
children and may appear after birth.
are scaly, dry, gray brown or black patches that are thickened and
covered by small papillomatous elevations giving the velvety texture
of the lesions.
resembles ichthyosis and is not associated with endocrine
disturbances or internal malignancy.
Fig. 371b. Acanthosis negricans
(Dark, scaly patches &
skin thickening and the skin lines are further accentuated. The
surface becomes mammillated or rugose, and larger warty
excrescencies develop. The intertriginous areas show warty lesions
where the lesions may become generalized and cover an extensive
areas of the skin surface.
occurs in adults and older age groups. This type is very rare in
lesions are rapidly progressive with involvement of the mucous
membranes, which are useful warning signs.
pseudo acanthosis type:
is a common type that appears after puberty and may be associated
with hypertrophic ostearthropathy.
DUE TO OTHER DERMATOSES
skin diseases may give rise to palmo planter hyperkeratosis.
Hyperkeratosis of the palms and soles are associated with the
characteristic psoriatic lesions with the silvery scales.
disease. The lesions are compact, heaped up and resemble the
heads of nails (keratoderma blenorrhagica).
Pityriasis rubra pilaris.
The palms and soles are thickened with even yellow hyperkeratosis.
The kerato-derma is associated with an acute follicular eruption
in adults and by the typical lesions of pityriasis on the knees
and elbows in juvenile types.
Hyperkeratotic eczema may be extensive and may be difficult to
differentiate by both the clinical and histological features.
Marked itching suggests eczema.
show prominent palmer lesions. These may be dry and either
atrophic or hypertrophic. Skin and laboratory changes are
lesions look rather warty and may be mistaken for viral warts and
for punctate keratoderma. The flat topped polygonal violaceous
papules with Wickman‘s stria help in the differential diagnosis.
The histopathological changes are characteristic.
immuno-compromised patients may be confluent on the palms or
Hyperkeratosis due to dermatophytes.
fungal infections especially lesions of Trichophyton rubrum may be
accompanied by marked hyperkeratosis of soles and palms.
disease: firm, warty, dirty ,symmetrical follicular papules
involving the face, trunk, flexures and extremities. The papules
form papillomatous or vegetative lesions. The histopathological
features are diagnostic.
Syphilis may involve the palms and soles leading to hyperkeratosis.
Hyperkeratotic lesions of late syphilis may be very warty or focal.
In the so-called ‘crab‘ yaws the gross kerato-derma of the late
lesions induces a peculiar crab-like gait in those affected.
multiple, irregular warty keratoses.
Occasional lesions of keratoderma are seen following ingestion of
medications such as iodine. In these cases the ‘keratoderma‘
peels off in a continuous sheet within days or weeks.
of abnormal keratinization may be familial which is inherited as an
autosomal dominant disorder or an acquired in the course of certain
may be single or multiple appearing as annular, dry plaques
surrounded by a raised, fine keratotic wall and sometimes show
furrow on the surface. The center is often atrophic but may be
common sites involved are the limbs, the face and genitalia that
show a tendency of centrifugal spread.
membranes may be affected as that of oral mucosa and cornea.
lesions affect mainly the limbs.
porokeratosis may be found together with disseminated actinic
superficial actinic Porokeratosis:
This is a
very common variety occurs in middle-aged individuals who have a
history of over exposure to sun where the lesions are mainly on the
sun exposed sites without malignant changes.
such as 2 per cent salicylic acid ointment.
5-fluorouracil ointment is also an effective medication.
dioxide laser can be used to extensive lesions.
is another line of treatment but side effects as erythema and
irritation may sometimes limit its use.
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