Download ٍٍSkin Failure "A loss of normal temperature control with inability to

Skin Failure
"A loss of normal temperature control with inability to maintain the core temperature; failure to prevent pe
percutaneous loss of fluid electrolytes and protein with resulting imbalance and failure of the mechanical b
barrier to penetration of foreign materials" Irvine (1991) J. Royal Soc. Med. 84:412, 1991
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"Severe loss of functions as occurs in other organ system e.g. heart failure, liver failure, renal failure and re
respiratory failure" better to be changed to Skin disability Ryan (1991) Br. J. Hosp. Med. 46: 33, 1991
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Skin failure from genetic point view
I. Development defect:
- Hair: Atrichia congenital
-Nail: Anonychia
-Congenital absence of skin.
-Sweat gland: Anhdrotic ectodermaL dysplasia
- Subcutaneous tissue Generalized lipodystrophy - partial lipodystrophy.
II Genetic factor:
-Chromosomal number: Down's syndrome, Turner syndrome, Klein filter syndrome.
–Deletion: X-linked ichthyosis.
– Mutation: Darier's disease, Hailey & Hailey disease.
-III .Metabolic:
-Porphyria
- Mucopolysacharidosis .
-Disorders of lipid metabolism
_ Disorders of amino acid metabolism.
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Diseases Cause Skin failure
1. Burn
2.Toxic epidermal necrolysis
3. Steven –Johnson syndrome.
4. Pustular Psoriasis.
5. Erythrodermas
6.Pemphigus vulgaris & bullous pemphigoid.
7. Graft versus host disease.
8. Epidermolysis bullosa.
9. Recurrent miliaria: @ Heat stroke
@ anhydrotic ectodermal dysplasia
1O. Aids.
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Skin Diseases which require ICU
1. Toxic epidermal necrolysis (TEN) 2. Staphylococcal scalded skin syndrome (SSSS).
3. Blistering diseases: pemphigus vulgaris, bullous pemphigoid.
4 Excessive viral infections: @ Disseminated herpes simplex (Kaposi varcilliform ereuptioon0
@Disseminated herpes zoster
@Varicella
5. Skin necrosis: @ Necrotizing & gangerous cellulites.
6. Exfoliative dermatitis.
7. Heavy therapeutic modalities:
@ Heavy chemo therapeutic regimen for treatment of melanoma, lymphoma….
.
@ Extra corporal phototherapy.
Roujeau 7 Revuz. Recent advances in Dermat. Vol., 8 (199O)
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Dermatologic Emergencies
(Skin diseases need ER)
1. Toxic epidermal necrolysis 2.Steven Johnson syndrome. 3. Pemphigus vulgaris
4. Infectious diseases: @ Chronic gonococcal septicemia, Mountain spotted eruption
@ Neonatal herpes simplex, Kaposi varcilliform eruption
@ Disseminated candidacies.
@ Staphylococcal scalded skin syndrome.
5. Necrotizing fascitis.
6. Auto immune disorders: @ Acute cutaneous & systemic lupus erythematosis
@ Dermatomyositis
@ Leucocytoclasatic vasculitis
7. Inflammatory disorders: @ Pyoderma gangrenosum
8. Environmental disorders: @ Heat stroke @ Child abuse
@ light stroke
Scleroderma nenatorum
Benn ion: Dermatology secrets (1996)
I would like to add: 1. Urticaria & angioedema
2. Anaphylaxis: @ introduction of an antigen given parent rally (penicillin)
@ Ingestion of food or drug (aspirin, Cheese, fish, strawberry...)
@ Topical application (Bactiracin) @ Stings & bites
3. Deep venous thrombosis
-Toxic shock syndrome
.
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Suicide in dermatology
In a report in Br. J. Derm. 137, 246-25O, the following diseases were described:
1. Psoriasis
2. Bed Sores
3. Acne vulgaris
4, Scabies
5. Delusion of parasitoids
6.Systemic sclerosis
Functions of the skin
1. Barrier between the body & the environment. The barrier function is mainly located in the
Skin within the lipid layer of stratum corneum. It will help the body to protect its fluids & its electrolytes.
Moreover, the barrier function will protect against infection which is the most common cause of death in c
diseases e.g. TEN, blistering disease, erythroderma, graft versus host disease, aids. Damaged skin &
exudates, support the growth of wide spectrum of microorganism despite the isolation & dressing
2. Regulation of body temperature:
A.@Erythroderma ---Elevation of skin temperature- total thermal loss = decrease core temp.-->
Sensation of chill.
@ Erythroderma = raised blood flow to skin - High output failure
@ Erythroderma -Fluid loss by transpiration - Thrombophlebitis
B. Fever & shivering due to IL1 (released by lyses epidermal cells). (TEN, Steven Johnson syndrome,
. Blistering disease, burn).
C. Heat stroke e.g. recurrent miliaria, hypohidrotic ectodermal dysplasia.
3. Respond to the mechanical forces. If epithelium is disrupted (Blistering disease, TEN, GVHD)
-Mechanical barrier is lost -Failure to prevent penetration of foreign material.
4. Immunological functios. : There are various alterations of immune response
A. Humoral: Serum immunoglobulin: depletion (TEN, burn) but increased ( Erythroderma, DVHD)
B. Cellular: @ Decrease CD4 T lymphocytes
@ Decrease cytotxic T cells
@Decrease of natural killer cells.
@ Langrhan cell depletion (GVHD, aids)
@B cell activation for auto antibodies (GVHD)
C. Phagocytes cell: Granulocyte: Decreased chemo tactic & phagocytes activity (TEN, Steven Johnson syn
GVHD)
5. Sensory functions
6. Socio-sexual communications. Medicine traditionally is concerned with disorders that end life but great
be paid to medical conditions that ruin life without ending it.
A. Acne Vulgaris: Skin of face comprises 9% of total skin surface, yet it is our passport to society.
B. Vitiligo: According to the Egyptian law 9; 1922 divorce can occur legally , if any of the couple has
a disease that cannot be cured e.g. vitiligo, leprosy or mania. The divorce occurs whether he has
the disease before or during marriage.
Impact of skin diseases
Skin disability
Restriction or lack (resulting from impairment) to perform an activity within the range considered
normal for a human being.
E.g. being able to walk
The term is used in more general sense. It includes:
1. Hand dermatitis
2. Sunburn
3. Skin cancer
4. Failure to display due to disfigurement:
a. Vitiligo b. Acne vulgaris c. Alopecia areata. d. Psoriasis
(Ryan (1991): Br. J. Hosp. Med. 46: 33, 1991)
Impairment
Or inability of psychological, physiological or anatomical structure or function
E.g. Broken limb
Handicap
From impairment and disability that limits or prevents the fulfillment of a role that
is normal for that individual
E.g. unemployment.
Body image
The Physical properties of a person carried into the imaginary or himself.
Quality of life
Factors impact on psychological state, social relationship & everyday activities.
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The path physiology of skin functions in diseases causing skin failure will be discussed:
Toxic epidermal necrolysis
Protective Barrier:
Full thickness damage to epidermis 
A. Fluid loss: Daily loss of 3-4 L if
more than 5o% of body surface is
affected  decrease of intravascular
volume  decrease of urinary output
-- > hyperosmolar urine  increase
blood urine nitrogen .creatinine
(functional renal failure0 If not
corrected - renal failure
B. Infection: Severe systemic
infections sare the main cause of
death in TEN
2. Regulation of body temperature:
Fever & shivering due to IL1 released
by lyses of epidermal cells. Shivering
reflects the high level of muscular
catabolism.
3. Immunologic function:
@Granulocyte: Decreased
chemotactic & phagocytic activity.
@ Depletion of serum
immunoglobulin
@ Decreased CD4 T
lymphocytes
@ Decreased cytotoxic T cells
@Decreased natural killer cell
activity
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Acute Generalized Pustular Psoriasis
1. Protective barrier:
Exfoliation of dried pustules & sheats of skin. @ -Loss of plasma protein in the tissue 
Hypoalbuminemia -Hypocalcaemia
@Fluid loss - Oligemia -Renal tubular necrosis
Oligemia can cause - liver damage & cholestatic jaundice
Moreover, fluid loss can cause - deep vein thrombosis -Fatal pulmonary embolism.
2. Regulation of body temperature:
@ Erythroderma - Elevation of skin temperature - Total thermal loss -
Decrease of core temperature - Sensation of chill
@Erythroderma-Raised blood flow to the skin - Increased cardiac output –
 Cardiac function is affected.
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Erythroderma
1. Protective Barrier:
A. Increase exfoliative scales -Pncrease protein loss (23O g /day) + exudates protein rich
Fluid - Hypoalbuminemia (due to increase metabolism & decreased synthesis). This will
lead: @ edema @ High cardiac output failure.
B. cutaneous & subcutaneous infection.
2. Regulation of body temperature: Increase blood flow - Increaser skin temperature
- @ Compensatory hypermetablism - Increase B.M.R.
@ Increase fluid loss by transpiration -Thrombophlebitis
.
3. Immunological function: A. Increased gamma globins.
B. High IgE
C. CD4+ T lymphocytopenia in absence of HIV infection.
Blistering Diseases
(Pemphigus vulgaris- Bullous Pemphigoid)
1. Protective barrier:
The barrier function is mainly located in the skin within the lipid structure of stratum corneum.
A. Fluid loss:
The blister fluid contains around 4O g / L of protein, 12O -15o m mol /l of NA=, 1OO m mol/L of
Cl-, 5 -1O M mol/L of k=. Also there is increased evaporation of 'FREE WATER' without protein or
electrolytes. The total daily cutaneous fluid losses average 3-4 lire. IF 5o% of body surface is
Affected by disease + Hypoalbuminemia - Reduction of intravascular volume - decrease
urinary output - Hyperosmolar urinary content ( Decreased Na, Increase K) - Increase urea
nitrogen & creatinine. - Functional renal failure. If not corrected - organic renal failure.
B. Infection: Damaged skin & exudates support the growth of wide spectrum of microorganisms
despite isolation & dressing. Infection is the main cause of death in these diseases.
2 Regulation of body temperature: Fever & shivering due to IL1 released by lysed epidermal cells.
Graft Versus Host Disease
1. Protective barrier:
Epithelial damage in GVHD is mediated largely by cytotoxic T cells & in which Langerhan cells
are selectively lost.
Infectious complications: (bacteria, virus, fungus…) is the most common cause of death & result
from: @ lymphoid failure
@ Functional asplenism
@ Circulating non specific suppressor cells @ Depression of epithelial barrier
.
2. Immunological function:
i. Cytotoxic T cells lost the controlling influence of regulating suppressor cells.
ii. Langerhan cells depletion in acute GVHD
iii. B cell activation -production of auto antibodies e.g. antinuclear, anti smooth muscle, ant
mitochondrial, anti epithelial.
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