occurs in about 10% of patients with primary thyrotoxicosis.
usually diffuse and is broadly of Addisonian pattern but
involvement of the mucous membranes is uncommon and pigmentation of
nipples and genital skin is less striking.
pigmentation appears at birth in the infant of a thyrotoxic mother.
are occasionally conspicuously pigmented.
patients show chloasmal rather than diffuse pigmentation.
incidence of vitiligo is increased.
associated with other diseases.
pigmentation is an unconstant feature of a wide variety of systemic
disorders and may be associated with malignant disease.
may occur in many chronic infections, but it is difficult to
determine the relative responsibility of the infection, malnutrition
and other factors. It has been suggested that the activity of the
reticulo-endothelia system (RES) is inversely related to
adrenocortical activity. With the stimulation of the RES by these
chronic infections and a consequent reduced adrenocortical activity,
this would lead to an enhanced pigmentation of the skin.
may accompany certain chronic infections as malaria, kala-azar,
schistosomiasis and tuberculosis.
light-brown pigmentation is an indication of the later stages of sub
acute bacterial endocarditis.
diseases associated with hyperpigmentation:
skin diseases may be associated with hypermelanosis. Some of these
include the following:
photodermatitis and long time exposure to direct sunlight.
diseases such as Gaucher‘s and Niemenn-Pick disease.
states, there may be diffuse hyperpigmentation of the skin as in
ectopic ACTH syndrome, which may occur in patients with oat-cell
carcinoma of the bronchus, pigmentation is usual. The tumor has been
shown to produce a distinct MSH-like compound.
In an adult,
acanthosis nigricans is associated usually with an internal
malignancy, almost invariably an adenocarcinoma.
hypermelanosis affects the axillae, nipples and umbilicus, which
also show a warty papillomatosis.
changes may later become generalized. The mucous membranes are
dermal melanosis, having a slaty-blue color, can occur secondary to
is an uncommon manifestation of the lymphomas, occurring in 10% of
cases of Hodgkin‘s disease and in 1 or 2% of cases of
lymphosarcoma and lymphatic leukemia. The pigmentation is of
Addisonian type, but without involvement of the mucous membranes.
may be a factor in increasing skin pigmentation.
scratching due to chronic skin itchy diseases.
diffuse progressive hyperpigmentation.
drugs used for the treatment of these disorders also can produce
increased pigmentation of the skin.
the nervous system.
the nervous system particularly those involving the diencephalon and
the substantia nigra show pigmentations of the addisonian type.
pigmentation is a feature of Schilder‘s disease but some increase
in pigmentation is not uncommon in schizophrenia, emotional stress,
Parkinsonism, hepatolenticular degeneration and in ependymomas.
attacks may be preceded by 3 or 4 days of diffuse darkening of the
pigmentation in scleroderma may be intense and diffuse or of
Addisonian type, but without mucous membrane involvement.
may be a conspicuous feature of morphoea and may indeed be the
and systemic lupus erythematosus may be accompanied by
is usually generalized occasionally observed in rheumatoid arthritis
and Still‘s disease.
renal disease with nitrogen retention is frequently accompanied by
of the skin occurs in vitamin B12 deficiency and is more common in
dark-skinned races . The pigmentation often has a rather dappled and
mottled appearance, and particularly affects the face, hands and
feet. Sometimes only the fingers are affected. Treatment with
vitamin B12 reverses the pigmentation of the skin back to normal.
pernicious anemia there is an increased incidence of vitiligo and of
premature grayness of the hair.
hemolytic anemia, hypermelanosis and hemosiderosis may develop on
the lower legs.
bronzed or slaty-gray in color, involves first the exposed skin but
later it is generalized. It is present in some 90% of cases, but may
not be conspicuous. The diagnosis should be suspected when
pigmentation of this pattern occurs in middle-aged men, and will be
supported by the finding of an enlarged liver and diabetes and
confirmed by the high level of serum iron.
hypermelanosis is seen in patients with liver cirrhosis.
planus may be associated with primary biliary cirrhosis and as
lesions resolve they leave slaty-brown hypermelanotic macules.
pigmentation, often symmetrical, is seen in both lichen and macular
type of amyloidosis is often mistaken for post inflammatory
hyperpigmentation, but the lesions often have a distinctive
“ripple“ pattern and microscopic studies will reveal the
presence of amyloid. Melanophages are to be found in the papillary
dermis. The melanin contained in these dermal cells is derived from
degenerating basal keratinocytes and melanocytes.
deficiency of vitamin A, generalized pigmentation may occur, as well
as pigmentation of the horny follicular papules.
pigmentation has been noted particularly in the Mongoloid races and
may be striking, especially in the bulbar conjunctiva.
and folate deficiency
and folate deficiency show diffuse, brown pigmentation is seen
occasionally in patients with folic acid deficiency and also in
vitamin B12 deficiency. Pigmentation of the fingertips and nails of
a patient with B12 deficiency is reported.
is frequent in pellagra. It is often of Addisonian type but the
pigmentation is accentuated on the face and hands and on areas of
friction or pressure, or as a sequel to pellagrous dermatitis.
causes purple pigmentation or slate colored pigmentation.
causes bluish black hyperpigmentation
causes skin melanosis
such as phenothiazine, antipyrine, tetracycline cause skin
causes gum hyperpgimentation
causes metallic blue pigmentation
of ascorbic acid is sometimes associated with pigmentation, which is
usually of Addisonian type.
In sprue and
other malabsorption syndromes pigmentation is of common occurrence.
It may be of Addisonian type but without involvement of the mucous
membranes, or may occur in well-defined patches on the face and neck
and occasionally on the trunk. The scaly inflammatory plaques, which
may develop in these syndromes, are usually followed by intense
disease classically occurs in those in whom lack of food is combined
with lack of cleanliness, and heavy infestation with pediculi. The
pigmentation is basically of Addisonian pattern and the mucous
membranes may be involved.
is probably post inflammatory and related to the scratching from the
pediculosis infestation. Areas of hypomelanosis occur and there is a
decrease in the number of melanocytes, which show degenerative
commonly follows acute or chronic inflammatory processes in the skin
as in lichen planus, herpes zoster, dermatitis herpetiformis,
papular urticaria and collagen diseases.
intensity and persistence of the hypermelanosis are greater in
hyperpigmentation may occur following trauma to the skin. It can
occur following dermabrasion and particularly in those who are
racially pigmented, of skin type 111 & 1V.
diseases: such as
discoid lupus erythematosus.
hyperpigmentation particularly central cyanosis occurs secondary to
congenital heart disease.
alba is a non-specific dermatitis of unknown origin that
characteristically produces erythematous scaly patches, which
subsides to leave areas of depigmentation.
cause of pityriasis alba is not clear.
that there are different predisposing factors for pityriasis
alb.These include the following:
exposure to sunlight.
spending long time in the swimming pools containing excess chlorine
and later long exposure to direct sunlight.
recurrent infections such as chronic tonsillitis.
children are more susceptible.
especially at night and sleeping immediately after the night meal.
cosmetics and topical preparations.
hyperactive and irritable children.
alba is usually a chronic skin eruption affecting predominantly
children. The clinical picture of the skin lesions varies from scaly
erythematous patches to depigmented plaques. The site involved is
mainly the face . Other sites like shoulders and the limbs may be
affected but to a lesser extent.
lesion is usually asymptomatic and the patients usually seek advice
due to cosmetic changes, due to the depigmentation at the site of
is variable; healing of the patches may take few days or weeks and
may last for more than a year. Remission and relapse of the skin
lesions are not uncommon, where the new patches may appear at the
Fig. 327. Pityriasis Alba
Fig. 328. Pityriasis Alba
Fig. 329. Pityriasis Alba
Fig. 330. Pityriasis Alba
pityriasis alba depends on the clinical picture .
lesions appear as erythematous, well demarcated covered by fine,
branny adherent scales or as a depigmented patches of a chronic
course eliciting minimal symptoms and affecting mainly children.
It is not
difficult to differentiate pityriasis alba from psoriasis, where the
latter patches show more dense silvery scales and may involve the
scalp and the extremities which are not the sites for pityriasis
the patches are more depigmented and whitish in color and may affect
extensive areas and if the scalp is involved the affected area shows
usually grayish hair.
eczema may simulate pityriasis alba clinically but the lesion of
discoid eczema is erythematous showing some vesiculation in the
acute stage, while in the chronic cases of discoid eczema the dry
scaly lesions, especially when it is solitary on the face may
simulate pityriasis alba.
such as itching are more in discoid eczema.
of discoid eczema and the skin changes are more prominent.
pityriasis alba is non-specific.
creams may be helpful.
such as hydrocortisone combined with salicylic acid, vioform or tar
is some times helpful.
15% oil of
pergamount applied in the morning may help repigmentation.
Dermatosis of Ramirez)
This is a
rare syndrome of unknown etiology that affects children and old age.
The disease simulates lichen planus clinically and histologically. .
It is sometimes called lichen pigmentosus. The exact relationship to
lichen planus is uncertain but there are close similarities and both
conditions may coexist.
manifest with numerous macules of varying colors, of gray with a
red, slightly raised and palpably infiltrated margin. They vary in
size and tend to coalesce over extensive areas of the trunk, limbs
general grayish background are macules of hypomelanosis or
degeneration of the basal layer.
epidermis contains much pigment and there is pigmentary
incontinence. The dermal vessels are sleeved with an infiltrate of
lymphocytes and histiocytes, and there are many melanophages.
pigmented macules resemble very closely the lesions of the late
pinta, but the negative dark-field examinations; negative
serological tests for syphilis and lack of response to penicillin
are important features.
involving predominantly the face and the neck is relatively common
and often presents a complex diagnostic problem.
Fig. 331. Facial
type III & IV skin types, are more susceptible to
factors play a major
role in cloasma and are implicated to some degree in other
melanosis. Addisonian pigmentation, the condition is more frequent
agents: light and
photodynamic chemicals are essential factors.
melanosis, erythrosis and poikiloderma of Civatte.
frequently a cause of facial melanosis.
compounds in cosmetics.
and other factors may be involved. Some cases occur in children and
in others there is no history of contact with cosmetics.
certain types of facial melanosis may be of unknown cause.
pigmentation develops quite rapidly over the greater part of the
face but is more intense on the forehead and temples.
pigmented macules, often perifollicular, lie beyond the indefinite
pigmentation may extend to the chest, neck and scalp, and
occasionally involves hands and forearms. Horny plugs fill the
follicles and there may be some scaling.
hyperpigmentation that usually involves the periorbital areas and
may extend to the eyelids and cheeks .The pigmented areas are either
deep brown or appear as black halos around the orbits.
factors may predispose to periorbital hyperpigmentation mainly:
to the periorbital muscles as a result of reading, TV and others.
- Stress and
Fig. 332. Periorbital Hyperpigmentation
compresses applied to the periorbital area twice daily followed by a
bleaching agent such as hydroquinone (Eldoquine 2%).
disorder occurs in middle-aged women on the sun-exposed areas. The
age incidence suggests that an unknown endocrine factor or age
change may also play some part.
reticulate pigmentation with telangiectasia and atrophy develops in
irregular more or less symmetrical patches on the lateral cheeks and
the sides of neck but spares the area shaded by the chin.
seen in 90% of Mongoloid babies and less frequently in Negroid
babies, and usually affect the lumbosacral region. These are poorly
circumscribed areas of slate-brown or blue-black that is sometimes
very extensive and may be mistaken for bruises.
fade in early childhood, but the aberrant extrasacral spots can
persist. The dermal melanocytes in the persistent Mongolian spots
have an extracellular sheath as is also seen in the nevus of Ito.
Fig. 333. Mongolian spots
NEVUS OF OTA
hyperpigmentation affects one side of the face in the area supplied
by the ophthalmic and maxillary divisions of the trigeminal nerve.
Occasionally it is bilateral. It is usually congenital but may
appear later in life. It is more prevalent in the Japanese but is
observed in other races.
Fig. 334. Nevus of ota
The color is
variable, but usually is either slate-brown or blue.
is involved and there may be hyperpigmentation of the cornea, iris,
retina, ocular muscles and orbit. Sometimes there is pigmentation of
the hard palate.
occurring in a patient with nevus of Ota has been reported.
Melanomas may develop in the skin, eye and brain of these patients,
more frequently in white patients than in the Japanese.
NEVUS OF ITO
condition the increased pigmentation affects the area supplied by
the posterior supra clavicular and lateral brachial cutaneous
Fig. 335. Nevus of Ito
commonly occur on the hands and feet, usually early in life.
cellular and elevated lesions may rarely undergo malignant change.
Fig. 336. Blue Nevus
congenitale is a rare disease, appears early in infants usually in
the first six months after birth, characterized by telengectasia,
atrophy, and skin pigmentation.
lesions appear first on the face and hands and extend to neck and
trunk where, the lesions at the end stage closely resemble chronic
are first and most severely involved, but the forehead, chin, neck
and ears seldom escape. The hands, forearms and lower legs are next
affected and the buttocks and thighs are frequently involved.
Fig. 337. Poikeloderma
sensitivity is a feature of many cases. Light sensitivity may be so
severe provoking a bullous response that tends to diminish after
early childhood; it may persist into adult life.
keratoses develop on exposed skin from adolescence onwards
presenting with large warty keratoses of hands, wrists, feet and
carcinoma may develop in the keratoses or in the surrounding
Eyebrows and lashes, pubic and axillary hair is often sparse or
is often sparse, fine and may be absent.
are normal or small and dystrophic.
are often normal, but microdontia and early caries have been
bilateral cataracts have developed, usually between the fourth and
frequently retarded; most patients are of small stature and some are
dwarfs. The dwarfism is proportionate with slender, delicate limbs,
small hands, feet, and short stubby fingers. The skull may be small
and the features bird-like, sometimes with a saddle-nose.
Expectation of life appears to be normal.
of slight or severe degree is frequent.
parathyroidism appears also to be increased.
development is usually normal, but some cases may be retarded.
essential features in differential diagnosis are:
The age of
distribution of the lesions.
combination of atrophy, telangiectasia and mottled pigmentation is
more intense on light-exposed skin.
changes are essentially sclerodermatous, and both skin and ocular
lesions develop later than in the Rothmund-Thomson syndrome.
pigmentation develops between the ages of 5 and 13, and is most
marked on the neck, trunk and thighs. Atrophy and telangiectasia may
appear later. The nail changes are constant and severe.
child is often small but otherwise normal during the first year,
thereafter development is retarded. The scalp hair, brows and lashes
are lost and the skin assumes an increasingly senile appearance.
sensitivity is a conspicuous feature after the first year, but there
is no poikiloderma.
combinations of conical teeth, hypotrichosis and partial or complete
anhidrosis. The skin is atrophic but not poikilodermatous.
In the mild
forms there is only freckle-like macules.
is the conspicuous feature.
is often irregular, linear and present at birth.
and not poikiloderma, is the essential change.
syndrome, probably is determined by an autosomal dominant gene Clinical
Vesiculopustular eruption of hands and feet in infancy and
Extensive eczema from 3 or 6 months to 5 years;
Persistent poikiloderma, sparing only the face, scalp and ears,
papules on hands, feet, elbows and knees.
excessive exposure to sun.
can be used before exposure to sunlight.
camouflage may also be indicated.
preparation: hydroquinone (Eldoquine 2-4%) may help in some cases.
be taken in using strong concentrations especially in children and
those with delicate skin. Local irritation is very common and
hypopigmentation, pigmented colloid milium and even vitilligo like
reactions may develop with the long use of this potent hydroquinone.
It should be
noted that hydroquinones are not very effective for post
inflammatory hyperpigmentation. The monobenzyl ether of hydroquinone
is responsible for many therapeutic and cosmetic disasters and the
compound should be used only to bleach away the remaining pigmented
areas in patients with extensive vitiligo.
other substituted phenols such as 4-isopropylcatechol can produce
cutaneous depigmentation: however, this compound and others are
irritant and may produce local sensitization.
recently used effectively and safely in treatment of pigmented skin
lesions. Q-switched ruby, Pulsed dye laser and other types can be
used with variable degrees of improvement. The results are better in
those who have fair or white skin color.
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