This chapter summarizes the different cutaneous
manifestations of certain systemic diseases.
The skin is a clear mirror of the human
body
where internal diseases may be reflected on the skin surfaces.
There are different internal diseases that can
cause skin manifestations . These signs may appear on the skin
surface with different clinical features depending mainly on the
primary internal disease:
Skin color
Different skin colors are associated with certain
skin diseases.
Pallor : as in anemia .
Earthy yellowish discoloration of the skin:
occurs in chronic intestinal infestations such as in bilharziasis.
Plethoric: due to hyperkinetic circulation as in
erythroderma and congestive heart failure.
Dryness of the skin occurs in chronic
debilitating diseases .
Thinning of the skin: is due to exhaustion of
dermal collagen such as in cachexia or locally due to topical potent
steroids.
Stria of the skin: occur in Cushing‘s
disease, after topical and systemic steroids for a long period,
dupuytren‘s contracture and in chronic
liver diseases.
Shape: changes in the form and shape of the
skin such as moon face due systemic steroids and lymphangitis and
gynecomastia that is related to increased circulating estrogens.
Hair changes: fine , lanugo hair covering the skin may become
pigmented as in some tumors mainly carcinomas .
Hirsutism: this is caused by increased
circulating androgens and cortisol due to Cushing‘s
disease or systemic steroid treatment and certain ovarian tumors .
Alopecia: may develop due to increased
circulating androgens or changes in the sensitivity of androgen and
estrogens receptors in the skin.
Changes in the color of hair: metabolic and
deficiency diseases such as Kwashiorkor and porphyries may cause
change in the color of hair.
Falling of hair: in anemia, hormonal disorders,
after chemotherapy or psychic trauma .
Nail changes: this occurs in chronic diseases
such as pernicious anemia, liver cirrhosis leading to white bands
and clubbing of nails.
Xanthomatosis, acne and seborrheic like
dermatitis occurs in hepatobiliary diseases.
Pruritus is a common manifestation of liver
diseases that is believed to be related to bile salt stasis and
increase in its concentration in the blood. Cholestyramine increases
fecal loss of bile salts and thus relieves itching.
Edema of skin may be due to hypoalbuminaemia,
increased venous pressure and increases capillary permeability.
Erythroderma: erythema and exfoliation of
skin may result from drug eruption and Papulosquamus diseases such
as psoriasis.
Urticarial lesions and alopecia areata: is
related to deep psychic trauma.
SKIN MANIFESTATIONS OF LIVER DISEASES
The pathological changes in the skin and its
appendages in liver disease are:
-
Jaundice in chronic liver diseases .
-
Diffuse hyperpigmentation of the skin due to
hepato-cellular damage.
-
Spider naevi, telengectasia, palmer flush,
livedo reticularis and vasculitis are common manifestations in
children.
-
Purpuric rashes are due to vitamin K
deficiency.
-
Hair :is fine in liver diseases.
-
Seborrhea and acniform eruption on the upper
part of the body is common manifestations.
-
Decreased
testicular androgens due to
hepatic dysfunction leads to fine hair in adults and gynecomastia.
-
Bier‘s spots:
white areas appear on the lower extremities when cooled .
-
Nails: changes in nails with absent lanula
and nail clubbing in liver cirrhosis.
SKIN MANIFESTATIONS OF RENAL DISEASES
Pruritus: is a common manifestation of renal
failure . The exact mechanism is not clear and may be related to
secondary hyperparathyroidism that leads to mast cells
proliferation.
Dryness of the skin: dryness of skin in renal
disease may be related to different factors mainly:
Excretion of nitrogen containing compounds on to
the skin surface. Decreased sebaceous gland activity leads to more
dryness and also increases the viscous cycle of itching .
Impaired androgen metabolism: increases dryness
of the skin. This also causes fine scalp hair, with falling of
axillary and pubic hair after puberty.
Skin color changes: This is due to increase
of melanocyte stimulating hormone occurring in chronic renal failure
since the kidney is the major site of metabolism of this hormone .
The skin color in renal failure varies from pallor due to anemia and
hyperpigmentation due to increased melanocyte activity .
SKIN MANIFESTATIONS
OF ENDOCRINE DYSFUNCTIONS
PITUITARY DISEASES
Different skin manifestations are related to
pituitary dysfunction :
Acromegaly : The skin is thick due to
increased collagen related to increase in circulating growth hormone
leading to coarse features and tendency of keloids formation, skin
tags and folds on the scalp (cutis verticis gyrata ).
The skin in acromegalic
patient is greasy and
pigmented and covered by thick coarse dark hair.
Hypopituitrism: The skin is dry, thin,
atrophic leading to wrinkles, which are apparent mainly on the face
with hypopigmented, faint yellowish brown patches.
CUSHING‘S
DISEASE
The different skin manifestations are:
-
Hyperpigmentation :which is due to increase
in the melanocyte-stimulating hormone (MSH).
-
Acne: due to excessive androgen secretion
forming keratotic plugs occluding the pilo sebaceous gland
orifices is a common manifestation of Cushing‘s
syndrome. The lesions are in the form of superficial papules and
pustules with minimal black heads. This type covers different
areas of the skin surface and unlike acne vulgaris, which affects
seborrheic areas of the face, back, and upper chest.
-
Hirsutism : This is due to increased
circulating androgen that is related to increased androgen
production .
Fig .350 Cushing's disease
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Fig 351. Cushing's disease
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Striae: this is due to the increased
circulating glucocorticosteroids. Skin lesions are pinkish in
color arranged usually in linear shapes .Old striae due to Cushing‘s
disease retains its blue- pink color in contrast to the other
types of striae which become faint whitish streaks later on.
-
Purpura : is a common manifestation .
Atrophy of dermal collagen leads to less support of the dermal
vessels , which become liable to rupture .
The presence of purpura in children and young
ages should raise the possibility of Cushing‘s
syndrome .
-
Moon face : this may be due to hydration of
subcutaneous fat .
-
Superficial fungal infections : Tinea
versicolor is also one of the manifestations seen in Cushing‘s
syndrome.
SKIN MANIFESTATIONS OF THYROID DYSFUNCTION
Skin manifestations of Hyperthyroidism
Pretibial myxedema: is the most characteristic
features of thyrotoxicosis appearing as shiny waxy papules and
plaques having orange-skin appearance on the chin of the tibia.
Increased hair of the areas involved.
Eczema : the lesions simulate atopic dermatitis
in children and adults.
Skin thickness: is increased leading to coarse
folds which is apparent more around the eyes. The skin in myxedema
appears as a coarse, dry, scaly, puffy and pale with coarse hair
possibly due to increased circulating TSH.
Warm skin and increased sweating due to increased
basal metabolic rate.
Pruritus .
Hyperpigmentation or vitilligo .
Premature hair graying .
Alopecia and hair loss on the eye browse .
The nails :become brittled and disfigured .
SKIN MANIFESTATIONS OF PARATHYROID DYSFUNCTION
Hyper parathyroidism: may cause pruritus,
cutaneous calcification, hemorrhage and infarction.
Hypoparathyroidism: cutaneous lesions may
simulate that of muco-cutanous candidiasis.
SKIN MANIFESTATIONS DUE TO ADRENAL DYSFUNCTION
Addison‘s
disease:
Skin manifestations of Addison‘s
disease are due to increased melanin and androgen .These may cause
different skin manifestations mainly:
-
Diffuse hyperpigmentation
: of the
buccal mucosa and skin usually on the sun exposed areas of the
face, neck and extremities , due to increased production of
melanin. Skin creases of the palms ,sites of friction , old scars
and previous pigmented areas become darker.
Virilism : due to increased circulating
androgens leading to hirsutism , male pattern baldness.
Increased thickening of the skin
: this
is due to increased dermal collagen.
Acniform eruption and increased seborrhea of the
skin and scalp due to increased androgens .
SKIN
MANIFESTATIONS DUE TO PANCREATIC DISEASES
-
Necrobiosis lipoideca diabeticorum:
Skin lesions are granulomatous, firm, sharply
demarcated, oval plaques of different sizes with shiny atrophic
surface and characteristically yellow center. The lesions appear
on the skin of diabetics mainly on the shins of the tibia due to
collagen degeneration . The course is very chronic and healing is
with scarring.
-
Bacterial and fungal skin infections mainly
candidiasis.
SKIN MANIFESTATIONS OF DIABETES
Skin manifestations of diabetes include the
following:
-
Necrobiosis lipodeca diabeticorum.
Fig.352 Necrobiosis
lipodeca diabeticorum
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Granuloma annulare: The lesions are pale or
flesh colored papules forming rings which blanche with pressure,
showing characteristic beaded ring of dermal white papules mainly
on the back of the fingers and hands . Granuloma annulare can be
caused also by tick bites and drug eruption .
-
Vitilligo : there is an increased incidence
of depigmentation of the skin in diabetics, which is lasting for a
long period.
-
Diabetic dermopathy : The skin lesion is in
the form of dull red , oval papules and may show small blisters,
which ulcerate leaving small erosions healing with atrophic,
pigmented patches.
5.
Diabetic skin gangrene
Fig.353 Diabetic skin gangrene
Anhidrosis: is patchy due to diabetic neuropathy
leading to heat intolerance.
Manifestations due to diabetes therapy
Lipodystrophy: at the sites of insulin injections
.
Urticarial reactions due to insulin .
Drug reactions: this is due to the oral
medications as sulphonylureas leading to erythema multiforme and
phototoxic reactions.
Fig. 353b. Drug reaction due to
diabetic therapy(Sulphonylurea)
Fig. 353b. Drug reaction due to
diabetic therapy(Sulphonylurea)
Xanthomatosis: The lesions appear in later stages
of diabetics due to increased serum lipids .
Trophic ulcers and bullous lesions : due to
diabetic neuropathy mainly on the feet .
SKIN MANIFESTATIONS OF INTESTINAL
MALABSORPTION SYNDROME
The clinical features are due to malabsorption of
the essential fatty acids. These manifestations are more common in
adults than in children.
-
Skin manifestations:
Dry scaly skin .
Dermatitis herpetiformis .
Fine hair .
Skin pigmentation of the mucous membrane of the
buccal cavity and skin creases are increased in some cases of
intestinal malabsorption.
Other rare manifestations:
Bowel lymphoma and skin blistering due to
epidermal necrosis in patients with carcinoma of the pancreas.
In children, the same manifestations may appear
in those fed on linoleic acid deficient diet . The common skin
manifestations are:
Psoriasiform rash.
Dryness , cracking and fissuring of the skin.
These cases improve with topical application of
sunflower seed oil.
Acrodermatitis enteropathica.
This is a genetic disorder that may be due to
zinc deficiency as in malabsorption syndrome. The condition may be
fatal in infants and young children.
Clinical Features.
Skin lesions.
Candidiasis like lesions appear on peri-oral,
around the genitalia, scalp, elbows and fingers. The skin eruption
is small blisters, pustules, erosions, crusting and scaling lesions.
Hair and nail loss
General manifestations
Acrodermatitis enteropathica may be accompanied
by severe diarrhea leading to cachexia.
Diagnosis depends on the clinical picture and the
decrease in the circulating zinc.
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Vasculitis
Intestinal malabsorption may be associated with
skin and bowel vasculitis.
Dermatitis herpetiformis
Dermatitis herpetiformis is an immunologic
problem due to deposition of IgA at the dermo epidermal junction
.The condition affects all age groups but mainly in middle aged
females.
The skin lesion begins as a small severely
pruritic papules on an erythematous base on the extensor surfaces of
the limbs and trunk. Dermatitis herpetiformis is usually accompanied
by coeliac disease.
Eczema, scabies, erythema multiformis simulate
dermatitis herpetiformis and some times it is not easy to
differentiate skin lesions.
The condition responds well to Dapsone and
Sulphapyridine.
REFERENCES
-
Callen JP, Jorrizo JL, eds. Dermatological
Signs of Internal Disease. Philadelphia: Saunders, 1988.
-
Jones JH, Mason DK, eds. Oral Manifestations
of Systemic Disease. Philadelphia: Saunders, 1980.
-
Braverman IM. Skin Signs of Systemic Disease
2nd edn. Philadelphia: Saunders, 1981.
-
Lang PG. Pituitary disorders. In: Callen JP,
ed. Cutaneous Aspects of Internal Disease. London: Year Book Medical
Publishers, 1981: chapter 39, 463-71.
-
Barth JH, Ng LL, Wojanarowska F et al.
Acanthosis nigricans, insulin resistance and cutaneous virilism. Br
J Dermatol 1988; 118: 613-19.
-
Callen JP. Skin signs of internal malignancy.
Austral J Dermatol 1987; 28: 106-14.
-
Kurwa A, Waddington E. Hepato-cutaneous
syndrome (juvenile cirrhosis, allergic capillaritis of the skin,
proctocolitis and arthritis). Br J Dermatol 1968; 80: 839-40.
-
McElgunn PS. Dermatologic manifestations of
hepatitis B virus infection. J Am Acad Dermatol 1983; 8: 539-48.
-
Isaacs NJ, Ertel NH. Urticaria and pruritus:
uncommon manifestations of hyperthyroidism. J Allergy Clin Immunol
1971;48: 73-81.
-
Brown J, Winkelmann RK, Randall RV. Acanthosis
nigricans and pituitary tumours. Report of eight cases. J Am Med
Assoc 1966; 198: 619-23.
-
Editorial. Pituitary-dependent Cushing‘s
disease. Br Med J 1977; i: 1049-50.
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