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THE SKIN MANIFESTATION OF SYSTEMIC DISEASES

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Title: THE SKIN MANIFESTATION OF SYSTEMIC DISEASES


1
THE SKIN MANIFESTATION OF SYSTEMIC DISEASES
  • Presented by
  • OGUNDIPE A.J

2
OUTLINE
  • Introduction
  • Common Systemic Diseases
  • Aetiology
  • Pathogenesis and Pathology
  • Clinical Features( including cutaneous
    manifestations)
  • Diagnosis
  • Management
  • Prognosis

3
INTRODUCTION
  • The skin is the mirror of the human body.
  • It is also the window through which we can look
    inside the body.
  • There is virtually no disease of the body that
    does not give itself away on the skin.
  • The skin is the greatest system since it can
    reveal the secrets of all body systems

4
COMMON SYSTEMIC DISEASES
  • GIT diseases Liver cirrhosis,Inflammatory bowel
    dxs, Carcinoid syndrome
  • Endocrine disorders- DM, Thyroid diorders
  • Cardiovascular diseases
  • Renal disease- CRF
  • Haematologic diseases- HIV/AIDS,etc
  • Respiratory diseases-Sarcoidosis,etc
  • Neurological diseases

5
Endocrine system diseases
6
Diabetes mellitus
  • It is disease of chronic hyperglycaemia due to
    relative insulin deficiency, resistance or both.
  • Insulin is a endocrine secretion from the beta
    cells of the paencreatic islet of Langerhans.
  • Insulin mediates the transportation of glocose
    molecules into hepatocytes, adipocytes and muscle
    cells.
  • It achieves this by binding to specialized
    transmembrane glucose-transporter proteins (
    GLUT,especially GLUT 4)on these cells.
  • This chronic debillitating disease results when
    there is either deficiency of insulin to bind to
    the receptors or the receptors are resistant to
    insulins advances

7
classification
  • There are 2 common types type 1 and type 2
  • Type 1- formerly juvenile-onset DM. Accounts
    for 10 of all cases.
  • Type 2- formerly maturity-onset DM. Accounts
    for 80-90 of cases.
  • Other types are Infection-induced
    DM,Drug-induced DM and DM in association with
    genetic syndromes

8
Clinical features
  • Symptoms are polyuria, polydypia, polyphagia,
    nocturia
  • Signs of over weight, dehydration may be present.
    There also be evidence of ketoacidosis( breathe
    smells fruity).

9
Non-infectious skin manifestations
  • Acanthosis nigricans
  • Diabetic dermopathy
  • Granuloma annulare
  • Lipodystrophy
  • Necrobiosis lipoidica
  • Pruritus (due to dry skin, yeast infection or
    poor blood flow)
  • Scleroderma diabeticorum
  • Xanthoma
  • Vitiligo(associated with type 1 than type 2)
  • Stiff hand syndrome
  • Lichen planus
  • Yellow nails

10
GRANULOMA ANNULAE
11
Infectious skin manifestations
  • Pyodermas
  • Malignant external otitis
  • Necrotizing fascitis
  • Erythrasma
  • Dermatophytosis
  • Candidiasis

12
Features of vascular insufficiency
  • Ischaemic ulcer
  • Digital gangrene
  • Erysipelas-like erythema
  • Periungual telangiectasia (microvascular
    engorgement and tortuosity involving the proximal
    nail fold.)
  • Red Skin and Rubeosis Facei(a function of the
    degree of engorgement of superficial veins)
  • Pigmented Purpura(salt and pepper appearance)

13
(No Transcript)
14
Periungual Telangiectasia
Gangrene
Pigmented Purpura. Salt and Pepper type of
yellow-tan hyperpigmentation of the shin
15
Erysipelas-like erythema
  • Microcirculatory compromise in diabetic patients
    leads to the development of well demarcated
    erythema on the lower leg or dorsum of the foot
    that correlates with radiological evidence of
    underlying bone destruction, and incipient
    gangrene.
  • The condition was at first mistaken for
    erysipelas (hence the name erysipelas-like
    erythema), but there was no associated pyrexia,
    elevated erythrocyte sedimentation rate, or
    leukocytosis.
  • This erythema would seem to be functional
    microangiopathy localized to an area of
    macrocirculation compromise


16
  • Acanthosis nigricans is characterized by
    symmetric, velvety to verrucous, hyperkeratotic
    and hyperpigmented plaques that have a
    predilection for the axillae, the nape, and other
    flexural areas. Insulin binds to the receptors
    composed of insulin-like growth factors with
    growth effect, hence the hyperkeratosis. Light
    microscopy distinguishes the disorder by the
    presence of hyperkeratosis, papillomatosis, area
    of acanthosis that may alternate with areas of
    atrophy, and increased amounts of melanin in the
    basal epidermis. insulin-like growth factors
    which have growth promoting effect, hence the
    hyperkeratosis.

17
Acanthosis nigracans
18
  • Atrophic hyperpigmented macules on the shins,
    so-called diabetic dermopathy, has been termed
    the most common cutaneous finding in diabetes.It
    is usually noted as irregularly round or oval,
    circumscribed, shallow lesions vary in number
    from few to many, which are usually bilateral but
    not symmetrically distributed. They are
    asymptomatic and often overlooked. Many patients
    who develop these depressed hyperpigmented
    lesions relate antecedent trauma or mild pyoderma
    such as folliculitis. "Diabetic dermopathy"
    probably represents post-traumatic atrophy and
    post-inflammatory hyperpigmentation in poorly
    vascularized skin.

19
Diabetic dermopathy
20
  • Granuloma annulare can occur on any site of the
    body and is occasionally quite widespread. Skin
    coloured bumps occur in rings often over joints,
    particularly the knuckles. The centre of each
    ring is often a little depressed. It usually
    affects the fingers or the backs of both hands,
    but is also common on top of the foot or ankle,
    and over one or both elbows.

21
Granoloma annulare
22
NECROBIOSIS LIPOIDICA
  • The primary lesion of NLD is a well-defined,
    small, firm, dusky-red papule topped with a fine
    scale. By slow enlargement or coalescence, these
    lesions form indurated plaques that are round or
    oblong when small and have an irregular
    geographic configuration when larger. The border,
    which sometimes is slightly elevated, and the
    adjoining skin are reddish-blue, whereas the
    center is yellow, indicating lipid accumulation.

23
Necrobiosis Lipoidica
24
Necrobiosis lipoidica
25
SCLEREDEMA ADULTORUM
  • Scleredema adultorum of diabetes is a syndrome
    characterized by a marked increase in dermal
    thickness on the posterior back and upper neck in
    middle aged, overweight, poorly controlled type
    II diabetic subjects. It is not recognized a
    being related to digital sclerosis, and we found
    no correlation by ultrasound measurements of back
    skin and hand skin thicknessThe examiner is
    demonstrating the inability to tent the skin on
    the dorsum of the neck

26
Scleredema diabeticorum
  • .

27
Diabetic bullae
  • Adult onset diabetic patient may have spontaneous
    onset of multiple bullae on his lower
    extremities, which are illustrated here. These
    lesions were not secondary to trauma or infection
    and they healed without special intervention.

28
THYROID DISORDERS
  • Hypothyroidism
  • Diffuse hair loss,vitiligo,dry
    skin,itching
  • Hyperthyroidism
  • Diffuse hair loss,itching
  • Graves disease-pretibia myxoedema

29
Pretibia myxoedema
30
Cushings syndrome
  • Results from hypercortisonism
  • Cutaneous symptoms include-acne,striae,skin
    atrophy

31
striae
32
Acromegaly
  • Results from growth hormone excess
  • Leads to excessive soft tissue hypertrophy
  • Cutaneous symptoms-skin tags,seborrhoea

33
Skin tags
34
Addisons disease
  • Occurs as a result of lack of cortisol
  • Leads to increase in ACTH level
  • Cutaneous symptom-hyperpigmentation

35
GIT DISEASES
  • Liver cirrhosis
  • Gardnersyndrome
  • Peutz-jeghers syndrome

36
Liver cirrhosis
  • This is the end stage of chronic disease of the
    liver.
  • It characterized by replacement of the normal
    liver parenchyma with fibrous tissue-the process
    of wound healing.
  • CAUSES of LC
  • Alcoholic liver disease
  • Viral hepatitis
  • Non-alcoholic Steatohepatitis, Primary
    sclerosing cholangitis, Autoimmune hepatitis,
    Hereditary hemochromatosis, Alpha 1-antitrypsin
    deficiency(AAT), Cardiac cirrhosis(due to chronic
    right sided heart failure which leads to liver
    congestion.
  • Galactosemia
  • Glycogen storage disease type IV
  • Cystic fibrosis
  • Hepatotoxic drugs or toxins

37
pathophysiology
  • The central pathogenetic processes in cirrhosis
    are progressive fibrosis and reorganization of
    the vascular architecture of the liver.
  • Activation of perisinosoidal stellate cells in
    the space of Disse
  • Stellate cells ususlly store and metabolize vit
    A, but transform into collagen-producing
    myofibrils during inflammation.
  • Activated stellate cells express growth and
    tissue repair factors
  • Normal liver matrix is replaced by collagen types
    1 and 3
  • Subendothelial fibrosis leads to loss of
    endothelial fenetrations, there impairing liver
    function.
  • Before fibrosis, inflammationregenerationdecomp
    ensation.

38
Clinical features
  • It may be clinically silent.
  • Progression from non-specific symptoms to hepatic
    failure (pptd by metabolic load, systemic
    infection or GI bleeding).
  • The features are
  • Due to portal hypertension ascites,
    splenomegaly, hepatomegaly, caput medusae
    (portosystemic venous shunt),
  • Due to defective bilirubin metabolism and
    transport jaundice.
  • Due to failure of hepatic functions fluffiness
    of the hair, fetor hepaticus, gynaecomastia,
    spider naevi, dupuytrens contracture, palmar
    erythema, nail changes, hypogonadism(feminism),ped
    al oedema.

39
Cutaneous manifestations
  • Fluffiness of the hair
  • Spider angiomata (spider nevi)
  • Caput medusae
  • Dupuytrens contracture
  • Palmar erythema
  • Generalized pruritus
  • Nail changes
  • Finger clubbing
  • Terrys nails-prox 2/3 of nails are white due to
    hypoalbuminaemia(leuconychia)
  • Muehrckes nail-prox paired horizontal bands
    seperated by area of normal colour.

40
Gardeners syndrome
  • Gardner syndrome is a variant of the disease
    familial adenomatous polyposis (FAP), an
    inherited disease that is characterised by
    gastrointestinal polyps, multiple osteomas
    (benign bone tumours), and skin and soft tissue
    tumours.
  • Polyps tend to form at puberty with the average
    age of diagnosis around 25 years of age.
  • In almost all patients, polyps will progress to
    malignancy, resulting in colorectal cancer so
    that timely detection is essential.
  • The most noticeable cutaneous feature of Gardner
    syndrome is the appearance of epidermoid cysts.

41
Peutz-Jeghers syndrome
  • Peutz-Jeghers syndrome (PJS) is a rare inherited
    disease that is characterised by gastrointestinal
    polyps in association with pigmentation affecting
    skin and mucous membranes.
  • The most noticeable cutaneous feature of PJS is
    the appearance of melanocytic macules (pigmented
    spots) in 95 of patients.
  • Tan, dark brown, or bluish black flat patches 1
    to 5 mm in size are seen around the mouth, lips,
    gums, inner lining of the mouth, eyes, hands and
    feet, fingers and toes, anus and genital areas.
  • Pigmentation usually appears before 5 years of
    age and may fade after puberty.

42
Caput medusae
43
RENAL DISORDERS
44
Chronic renal faiilure
  • It is a slowly progressive loss of renal function
    over a period of months to years.
  • It usually arises from multiple aetiologies,
    resulting in the reduction of the number and
    function of nephrons.
  • It usually presents with several skin
    manifestions.

45
causes
  • Congenital and Inherited
  • Medullary cystic dx
  • TuberoUS sclerosis
  • Congenital uropathy
  • Vascular disease
  • Hypertensive nephrosclrerosis
  • Renovascular disease

46
contd
  • Glomerular disease
  • Primary glomerulophhritidies
  • Secondary glomerulophritidis
  • Urinary tract obstruction
  • Calculus disease
  • Prostatic disease
  • Schistosomiasis

47
presentation
  • Malaise
  • Loss of appetite
  • Nocturia and polyuria
  • Nausea and projectile vomiting
  • Anaemic symptoms
  • Bone pain
  • Peripheral and pulmonary edema
  • Paraesthesia and tetany

48
Skin manifestations
  • Pruritus
  • Pallor
  • Pigmentary changes
  • Nodular prurigo
  • Ureamic frosts
  • Purpura
  • Metastatis calcification(calcinosis nd
    calphylaxis)
  • Ecchymoses
  • Reccurent viral warts
  • Half-on-half nail
  • Koilonychia
  • Onychomycosis
  • Splinter haemorrhages
  • Beaus lines
  • Subungal hyperkeratosis

49
Renal transplant
  • Pxs are at high risk of developing viral
    infections and squamous cell ca ,the progression
    of which may be aggressive

50
Hiv/aids
51
Hiv/aids
  • Acquired immune deficiency syndrome or acquired
    immunodeficiency syndrome (AIDS) is a disease of
    the human immune system caused by the human
    immunodeficiency virus (HIV).
  • This condition progressively reduces the
    effectiveness of the immune system and leaves
    individuals susceptible to opportunistic
    infections and tumors.
  • HIV is transmitted through direct contact of a
    mucous membrane or the bloodstream with a bodily
    fluid containing HIV, such as blood, semen,
    vaginal fluid, preseminal fluid
  • AIDS is now a pandemic

52
Clinical features
  • The symptoms of AIDS are primarily the result of
    conditions that do not normally develop in
    individuals with healthy immune systems.
  • Most of these conditions are infections caused by
    bacteria, viruses, fungi and parasites that are
    normally controlled by the elements of the immune
    system that HIV damages.
  • Opportunistic infections are common in people
    with AIDS.13 HIV affects nearly every organ
    system.
  • People with AIDS also have an increased risk of
    developing various cancers such as Kaposi's
    sarcoma, cervical cancer and cancers of the
    immune system known as lymphomas.
  • Additionally, people with AIDS often have
    systemic symptoms of infection like fevers,
    sweats (particularly at night), swollen glands,
    chills, weakness, and weight loss.
  • The specific opportunistic infections that AIDS
    patients develop depend in part on the prevalence
    of these infections in the geographic area in
    which the patient lives.

53
Skin manifestations
  • Viral infections
  • Acute morbilliform rash due to HIV infection
  • Herpes simplex
  • Varicella zoster
  • Molluscum contagiosum
  • Human papillomavirus
  • Oral hairy leukoplakia

54


Herpes simplex labialis
Molluscum contagiosum
Herpetic whitlow
55
contd
  • Fungal infections
  • Tinea
  • Blastomycosis
  • Candidiasis
  • Cryptococcosis
  • Histoplasmosis
  • Pityrosporum folliculitis
  • Pityriasis versicolor
  • Systemic mycoses
  • Pneumocystosis(previously classified as protozoa)

56


Patch stage
Kaposi sarcoma
Malassezia folliculitis
Plaque stage
Classic disease
57
contd
  • Bacterial infections
  • Cellulitis
  • Ecthyma
  • Impetigo
  • Folliculitis
  • Bacillary angiomatosis

58
contd
  • Arthropod infestations
  • Insect bites
  • Scabies
  • Demodicosis
  • Inflammatory conditions
  • Seborrhoeic dermatitis
  • Psoriasis
  • Eczema
  • Pruritic papular eruption of HIV

59
contd
  • Malignancies
  • Kaposi's sarcoma
  • Cutaneous B-cell lymphoma and cutaneous T-cell
    lymphoma
  • Skin cancer including melanoma, squamous cell
    carcinoma, basal cell carcinoma and anal
    carcinoma

60
Internal malignancies
61
Internal malignancies
  • Skin changes can often be the first sign of a
    deeper problem including an internal malignancy.
  • Signs of skin disease may precede, occur with, or
    follow the detection of an associated cancer.
  • These skin diseases can be a feature of an
    undiagnosed cancer and may be the prompt for a
    thorough examination in patients.
  • Cutaneous markers can be classified into 2
    major types
  • Genetically determined syndromes with a cutaneous
    component (genodermatoses) that predispose at
    risk individuals to develop cancer.
  • Paraneoplastic syndromes which occur as a result
    of circulating factor(s) or presumed factors
    produced by the underlying cancer.

62
genodermatosis
  • Cowden's disease
  • Gardner syndrome
  • Gorlin's syndrome
  • Multiple endocrine neoplasm type 2B
  • Neurofibromatosis
  • Peutz-Jeghers syndrome
  • Torre-Muir syndrome

63
Cowdens disease
  • Benign lesions of the skin and mucosa, known as
    hamartomas (benign tumours made up of a mixture
    of mature cells normally found in that tissue).
    At least one of the four types of skin lesions
    are present in nearly all cases.
  • Facial papules flesh-coloured flat-topped dry
    or warty 1-5mm papules around the mouth, nostrils
    and eyes.
  • Oral lesions numerous 1-3 mm smooth whitish
    spots on the gums and palate that join together
    to create a cobblestone appearance known as
    papillomatosis.
  • Acral keratoses flesh-coloured or slightly
    pigmented smooth or warty papules on the upper
    surface of hands and feet. These occur in more
    than 60 of patients.
  • Palmoplantar keratoses scaly spots on the palms
    and soles occur in about 40 of patients.
  • Other skin lesions occurring less frequently
    include lipomas, neuromas and haemangiomas.

64
CD is seen in
  • Breast cancer
  • Genitourinary tumours
  • Thyroid cancer,especially follicular thyroid
    carcinoma
  • Skeletal abnormalities such as bone cysts.
  • Central nervous system development of
    Lhermitte-Duclos disease caused by hamartomatous
    growths of the cerebellum (rare).
  • Gastrointestinal polyps

65
Facial papule
Acral keratoses
Close-up
66
Neurofibromatosis
  • It is classified into 2 distinct types,
    neurofibromatosis 1 (NF1) and neurofibromatosis 2
    (NF2).
  • NF1, also known as von Recklinghausen disease, is
    characterised by the presence of
  • 6 or more café-au-lait spots (defined oval-shaped
    light brown patches greater than 0.5cm in
    diameter)
  • Multiple neurofibromas (tumours on, under, or
    hanging off the skin)
  • Freckling (under the armpits and areas of skin
    folds such as the groin)
  • Lisch nodules (tiny tumours on the iris of the
    eye)

67
Freckling in the armpit
Café-au-lait mark
Neurofibromas
68
Paranoeplastic syndromes
  • Papulosquamous
  • Acanthosis nigricans
  • Acquired ichthyosis
  • Acrokeratosis paraneoplastica
  • Extramammary Paget disease
  • Florid cutaneous papillomatosis
  • Palmoplantar keratoderma
  • Pityriasis rotunda
  • Sign of Leser-Trelat
  • Tripe palms

69
Sign of Leser-Trélat
  • The sign of Leser-Telat is a skin disorder
    characterised by the abrupt appearance of
    multiple seborrhoeic keratoses that rapidly
    increase in their size and number.
  • It is caused by an associated cancer and often
    occurs with malignant acanthosis nigricans.
  • The sign of Leser-Trélat has been linked to a
    variety of cancers but is mostly associated with
    an adenocarcinoma of the stomach or colon.
  • Other cancers where the condition has occurred
    include squamous cell carcinoma, lymphoma and
    leukaemia.
  • The sign of Leser-Trélat is rare, even among
    patients with cancer.

70
Multiple eruptive seborrhoeic keratoses
71
Tripe palms
  • Tripe palms are also known as acanthosis
    palmaris.
  • Tripe palms are characterised by thickened
    velvety palms that have the appearance of tripe,
    the stomach lining of beef, pork, or sheep.
  • Approximately 90 of cases of tripe palms are
    associated with internal malignancy.
  • Tripe palms are frequently seen in conjunction
    with acanthosis nigricans. In these cases, the
    underlying malignancy is most commonly stomach
    (35) or lung (11) cancer.
  • Less commonly associated cancers include head and
    neck tumours, and tumours of the genitourinary
    tract.

72
Tripe palms associated with acanthosis nigricans
73
contd
  • Erythematous
  • Dermatomyositis
  • Erythema gyratum repens
  • Hypertrophic osteoarthropathy and digital
    clubbing
  • Multicentric reticulohistiocytosis
  • Necrolytic migratory erythema
  • Sweet disease

74
Necrolytic migratory erythema
  • Necrolytic migratory erythema is a characteristic
    rash usually occurring in the glucagonoma
    syndrome.
  • Glucagonoma syndrome is due to a slow-growing
    cancerous tumour located in the alpha cells of
    the pancreas.
  • Necrolytic migratory erythema may affect any site
    but it most often affects the genital and anal
    region, the buttocks, groin and lower legs.
  • Initially there is a ring-shaped red area that
    blisters, erodes and crusts over. It can be quite
    itchy and painful. As it heals, it may leave
    behind a brown mark.
  • It also results in a sore smooth tongue, a sore
    mouth, cracked dry lips and ridging of the nails.

75
Necrolytic migratory erythema
76
Sweet disease
  • Sweet disease was named after a Dr Sweet from
    Plymouth, England, who first described this
    condition in 1964. It is also known as acute
    neutrophilic dermatosis.
  • In Sweet disease there is a sudden appearance of
    red tender lumps (plaques) on the skin usually of
    the limbs or neck, sometimes elsewhere
  • Sweet is a reaction to an internal condition. It
    may follow internal cancer usually of bowel,
    genitourinary organ or breast

77
Sweet disease
Neutrophilic dermatosis of the dorsal hands
78
contd
  • Bullous
  • Paraneoplastic pemphigus
  • Miscellaneuos
  • Generalised granuloma annulare (rarely)
  • Carcinoid syndrome
  • Hypertrichosis lanuginosa acquisita
  • Trousseau syndrome

79
Hypertrichosis lanuginosa acquisita
  • Hypertrichosis lanuginosa acquisita is a very
    rare condition characterised by the rapid growth
    of long, fine, lanugo-type (i.e. fetal) hair
    particularly around the eyebrows, forehead, ears
    and nose.
  • It is similar to congenital hypertrichosis
    lanugino except that it appears later on in
    adulthood and in most cases associated with an
    underlying cancer.
  • Although the face is the most commonly affected
    area, some patients have extensive involvement
    that includes the trunk, axillae and extremities.
  • Palms, soles, scalp and pubic regions tend to be
    spared

80
contd
  • The most frequently associated cancers include
  • lung cancer
  • breast cancer
  • uterine cancers
  • colorectal cancer
  • lymphoma
  • bladder cancer.

81
Trousseau syndrome
  • Trousseau syndrome is also referred to as
    migratory superficial thrombophlebitis,
    carcinogenic thrombophlebitis, and
    tumour-associated thromboembolism.
  • Lesions appear as inflamed, reddened lines or
    lumps in the fat under the skin.
  • They may occur on the trunk or extremities and
    are similar to those found in cellulitis,
    erythema nodosum, lymphangitis, and vasculitis.

82
contd
  • Approximately 50 of patients with Trousseau
    syndrome have an associated cancer.
  • Pancreatic cancer appears to be associated with
    the highest risk of Trousseau syndrome.
  • Adenocarcinomas (cancers that develop in the
    lining or inner surface of an organ) can also
    cause the syndrome.
  • Lung cancers are commonly reported.

83
conclusions
  • It is important not to overlook dermatological
    problems. They could be timely clues to
    discovering serious systemic problems.

84
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