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DERMATOLOGICAL MANIFESTATIONS
OF SYSTEMIC DISEASES
guide: dr.rahul nagar
candidate: dr.smit chopra
CONNECTIVE TISSUE
DISORDERS
LUPUS ERYTHEMATOSUS
Discoid lupus erythematous
Subacute cutaneous lupus erethematosus
Systemic lupus erythematosus
DISCOID LUPUS ERYTHEMATOSUS
• Most patients have limited disease to head and
neck (localised DLE) but rarely may present as
disseminated disease potentially affecting any
area of skin (Disseminated DLE)
• It presents as rash (erythematous patches) with
adherent scale over it,
• Raynaud’s phenomenon found in 14% of
cases
Localised DLE-
• Face is most commonly affected area f/b
scalp, ears, arms, legs, and trunks to a
lesser extent
• Discoid erythematous rash appear most
commonly over the cheeks, bridge of the
nose, ears , scalp and side of the neck
• Alopecia occurs in 1/3rd of the patients
Disseminated DLE
• Characteristic lesions of DLE may occur in
widespread pattern on trunk and limbs
• This occur almost always in women and
they are cigarette smokers
• This variety tends to be persistent ,
resistant to therapy and associated with
psychological upset
SUBACUTE CUTANEOUS LUPUS
ERYTHEMATOSUS
• Lesions usually occur above the waist
particularly around neck, on the trunk and on
the outer aspects of arm.
• Predominantly affects adult age group
• Skin lesions comprises of non scarring
papulosquamous (two thirds) and annular
polycystic lesions (one third)
SYSTEMIC LUPUS ERYTHEMATOSUS
Skin manifestation occurs approximately in
80% of the cases
• Cutaneous erythema on light exposed area is the most common
feature
• Butterfly blush with fine scaling on butterfly area of the cheeks is
frequently found
• Epidermal necrosis may occur in some cases
• Alopecia occurs in around 50% of cases , hair becomes coarse
dry and fragile called ‘lupus hair’
• Persistent non itching urticaria like wheals are common
• Livedo reticularis(a mottled or bluish red discoloration, which
blanches on pressure and is not affected by temperature
changes) may develop over outer aspects of arms
SCLERODERMA
• At first lesions are indurated and faintly purplish later they lose their
colour especially in centre and appear as thickened waxy areas with
smooth and shiny surface , hairs are usually absent over these
surfaces
• Morphea i.e patches of hardened skin, appears over face, hands, and
feet which lacks sweat gland and ability to make skin folds
• Skin induration initially affects the fingers (sclerodactyly) and extend
proximally
• Raynauds phenomenon is a common finding
• Infarction and dry gangrene of fingers may occur sometimes due to
severe vasospasm
DERMATOMYOSITIS
A purplish-red or heliotrope erythema occurs on
the face, especially involving the eyelids, the
upper cheeks, forehead and temples
Oedema of the eyelids and periorbital tissues
may occur
Small erythematous or violaceous, flat papules
(Gottron’s papules) occur over the knuckles, on
the dorsa of the finger and around the nail folds .
RHEUMATOID ARTHRITIS
Rheumatoid nodules these are subcutaneous
nodules affecting the extensor surfaces of the
forearms, back of hand, occipital region, auricular
region more commonly
Cutaneous small vessel vasculitis may lead to
purpura, bruise or ulcerative lesions over the skin.
Pyodermagangrenosum sterile pustules which
rapidly evolve over painful ulcers charactrized by
raised edges with erythematous purple covering
INFECTIOUS DISEASE
TUBERCULOSIS
MODE OF
INOCULATION
DISEASE
DIRECT INOCULATION
Tubercular
chancre(primary
inoculation)
CONTIGUOUS
SPREAD
Scrofuloderma
MULTIBACILLARY AUTOINOCULATION
Orofacial
tuberculosis
HEMATOGENOUS
SPREAD
Acute military
tuberculosis,
Tuberculous
gumma(abcess)
MODE OF
INOCULATION
DISEASE
DIRECT INOCULATION
Warty
tuberculosis
(verruca cutis)
PAUCIBACILLARY
HEMATOGENOUS
SPREAD
Lupus vulgaris,
Lichen
scrofulosorum,
Nodular
tuberculid,
Erethema
induratum
PRIMARY INOCULATION
Tubercular chancre skin
lesion may vary from brownish
papule ,nodule to an ulcer with
undermined edge. On
diascopy it shows apple jelly
nodules
Warty tuberculosis (verruca
cutis) leisions occur on area
exposed to trauma.Starts with
a small indurated warty
papule. Irregular extension
may cause finger like
SCROFULODERMA
At first bluish-red nodule
overlying the infected
gland forms which breaks
down to form undermined
ulceration with granulating
tissue at the base
LUPUS VULGARIS
Plaque form
Vegetative form Tumor like form
LEPROSY
CLINICAL TYPE PERCATAGE DISTRIBUTION
Tuberculoid (TT) 8
Borderline tuberculoid (BT) 41.33
Borderline (BB) 5.3
Borderline lepromatous (BL) 26.66
Lepromatous (LL) 18.66
HIV
seborrhoea dermatitis
psoriasis
herpes simplex
viral warts
mollusca
oral and vaginal
candidiasis
scabies
squamous cell
carcinoma
kaposis sarcoma
ENDOCRINE
DISORDERS
THYROID DISORDERS
Cutaneous features of hyperthyroidism
• SKIN- palmar erythema ,facial flushing
,increased skin temp.,increased sweating,
hyper pigmentation , pretibial myxoedema
• NAILS- soft nails ,koilonychia,distal onycholysis
(plummer’s nail)
• HAIR- fine thin hair ,diffuse alopecia
Cutaneous features of Hypothyroidism
• SKIN-Pale, cold, scaly and wrinkled skin, Ivory-
yellow skin colour, Xerosis, Absence of sweating,
puffy oedema of hands, face and eyelids
• NAILS- Brittle and striated nails
• HAIR- Coarse sparse scalp hair, Loss of pubic,
axillary and facial hair, Loss of lateral eyebrows
(madarosis)
ADRENAL DISORDERS
Cutaneous manifestation of cushing’s syndrome
• Truncal obesity (classically deposits of fat over the clavicles and back of
the neck, the ‘buffalo hump’)
• Facial fullness and plethora (‘moon facies’)
• Slender limbs
• Skin atrophy
• Fragility, bruising and poor healing leading to Striae (typically white and
red)
• Hirsuties,Acneiform lesions
• Male-pattern baldness in women
Cutaneous manifestation of Addison’s disease
• Hyperpigmentation of the skin, due to increased secretion of
pituitary MSH and ACTH as a response to low adrenal
corticosteroid levels, is the cardinal dermatological feature
• Patterns of addisonian pigmentation -
1. Light-exposed areas i.e.face, dorsa of hands,
2. Areas subject to friction—elbows, knees, waistline
3. Accentuation of normally high pigmentation areas—genital,
perineum, axillae, areolae, umbilicus
4. Palmar creases
5. Tongue and mucous membranes
DIABETES MELLITUS
Periungual talengectasia-
A microangiopathic
complication seen in upto
49% of diabetics
Acanthosis nigricans- a
verrucous hyper pigmented
plaques seen over axilla ,
nape of neck which is due to
hyperinsulinemia
Granuloma annulare-
annular leisions with
raised skin colour having
symmetrical distribution
on arms,neck and upper
half of the trunk
Necrobiosis lipodica-
sharply demarcated
slightly depressed yellow
waxy plaques with
erythematous raised
border
Insulin reactions
(Lipodystrophy)- soft
nodules resembling
lipoma are formed, these
are local response to
lipogenic action of
insulin, preventable by
rotating injection sites
GASTROINTESTINAL
DISEASES
LIVER CIRRHOSIS
Skin lesions associated with chronic liver disease
•Spiderangiomas , telangiectasis
•Palmar erythema
•Dilated abdominal/chest veins (including periumbilical
caput medusae)
•Jaundice
•Increased melanin pigmentation
•Thin ‘paper-money’ skin
•Loss of secondary sexual hair in males
ACUTE PANCREATITIS
Grey Turner sign (Turner
sign)—tracks from the
pararenal space to the edge
of the quadratus lumborum
muscle then through a
defect in the fascia to the
subcutaneous tissues of the
flank (left sided in
pancreatitis)
Bryant’s sign—tracks to
the scrotum to produce
the ‘blue scrotum’ sign.
Cullen’s sign—tracks
into the falciparum
ligament then through the
connective tissues of the
round ligament to the
periumbilical area
NUTRITIONAL AND
METABOLIC DISORDERS
IRON DEFICIENCY DISORDER
• Paleness of skin, palm creases and conjuctiva
• Painful cracks at corner of mouth (angular
cheilitis)
• Shiny smooth tongue (atrophic glossitis)
• Spoon shaped nails (koilonychiya)
• Dry and brittle hair
VITAMIN B COMPLEX DEFICIENCIES
VitB3 (niacin)deficiency - Pellagra
• Red skin with large blebs or blisters that exfoliates
• Erethema over the dorm of hands
• Scaling over the sun exposed area of the skin
• Dry ,scaly and hyperkeratotic darkly pigmented skin
• Site- mostly over face, neck ,arms, hands and feet
B12 Deficiency -
• Skin hyperpigmentation with accentuation
in flexural areas ,palms ,soles and oral
cavity
• Angular stomatitis , glossitis(hunters
glossitis), xerostomia
• Vitiligo
VITAMIN C DEFICIENCY
SCURVY
• Perifollicular hemmorage with blood
pigment discolouration especially on the
trunk and lower limbs (purpuric rash)
• Swollen bleeding gums
• Epistaxis may occur sometimes
SYSTEMIC AMYLOIDOSIS
Myeloma associated cutaneous amyloidosis
(AL amyloid)
Secondary systemic amyloidosis (AA amyloid)
Dialysis associated amyloidosis
Myeloma associated cutaneous amyloidosis (AL
amyloid)
• Almost 40% of patients with AL amyloidosis
have skin manifestation
• leisions tend to aggregate around
mucocutaneous junctions like orbit, lips and
genital skin
• purpura is also a common finding which
results from vascular fragility due to amyloid
deposition in blood vessels
Secondary systemic amyloidosis (AA amyloid)
• The amyloids are composed of fibrils of a protein
designated as AA
• AA protein is an acute phase reactant
• secondary amyloidosis occurs as a complication of
many chronic inflammatory diseases
• Skin lesions are wage and are non specific
• Most common are renal manifestation leading to
nephrotic syndrome
Dialysis associated amyloidosis
• cutaneous involvement is rare
• manifests as hyper pigmentation
,lichenoid eruptions,or nodules that
demonstrate amyloid deposition on HPE
examination
TAKE HOME MESSAGE
CUTANEOUS MANIFESTATIONS CAN HELP IN
EARLY DIAGNOSIS AND INTERVENTION OF MANY
HIDDEN SYSTEMIC DISEASES
IT ALSO HELPS IN UNDERSTANDING THE
PROGNOSIS AND MANAGING THE DISEASE
THANK YOU

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Dermatological manifestation of systemic diseases

  • 1. DERMATOLOGICAL MANIFESTATIONS OF SYSTEMIC DISEASES guide: dr.rahul nagar candidate: dr.smit chopra
  • 3. LUPUS ERYTHEMATOSUS Discoid lupus erythematous Subacute cutaneous lupus erethematosus Systemic lupus erythematosus
  • 4. DISCOID LUPUS ERYTHEMATOSUS • Most patients have limited disease to head and neck (localised DLE) but rarely may present as disseminated disease potentially affecting any area of skin (Disseminated DLE) • It presents as rash (erythematous patches) with adherent scale over it, • Raynaud’s phenomenon found in 14% of cases
  • 5. Localised DLE- • Face is most commonly affected area f/b scalp, ears, arms, legs, and trunks to a lesser extent • Discoid erythematous rash appear most commonly over the cheeks, bridge of the nose, ears , scalp and side of the neck • Alopecia occurs in 1/3rd of the patients
  • 6.
  • 7. Disseminated DLE • Characteristic lesions of DLE may occur in widespread pattern on trunk and limbs • This occur almost always in women and they are cigarette smokers • This variety tends to be persistent , resistant to therapy and associated with psychological upset
  • 8.
  • 9. SUBACUTE CUTANEOUS LUPUS ERYTHEMATOSUS • Lesions usually occur above the waist particularly around neck, on the trunk and on the outer aspects of arm. • Predominantly affects adult age group • Skin lesions comprises of non scarring papulosquamous (two thirds) and annular polycystic lesions (one third)
  • 10.
  • 12. Skin manifestation occurs approximately in 80% of the cases
  • 13. • Cutaneous erythema on light exposed area is the most common feature • Butterfly blush with fine scaling on butterfly area of the cheeks is frequently found • Epidermal necrosis may occur in some cases • Alopecia occurs in around 50% of cases , hair becomes coarse dry and fragile called ‘lupus hair’ • Persistent non itching urticaria like wheals are common • Livedo reticularis(a mottled or bluish red discoloration, which blanches on pressure and is not affected by temperature changes) may develop over outer aspects of arms
  • 14.
  • 15.
  • 16. SCLERODERMA • At first lesions are indurated and faintly purplish later they lose their colour especially in centre and appear as thickened waxy areas with smooth and shiny surface , hairs are usually absent over these surfaces • Morphea i.e patches of hardened skin, appears over face, hands, and feet which lacks sweat gland and ability to make skin folds • Skin induration initially affects the fingers (sclerodactyly) and extend proximally • Raynauds phenomenon is a common finding • Infarction and dry gangrene of fingers may occur sometimes due to severe vasospasm
  • 17.
  • 18.
  • 19. DERMATOMYOSITIS A purplish-red or heliotrope erythema occurs on the face, especially involving the eyelids, the upper cheeks, forehead and temples Oedema of the eyelids and periorbital tissues may occur Small erythematous or violaceous, flat papules (Gottron’s papules) occur over the knuckles, on the dorsa of the finger and around the nail folds .
  • 20.
  • 21. RHEUMATOID ARTHRITIS Rheumatoid nodules these are subcutaneous nodules affecting the extensor surfaces of the forearms, back of hand, occipital region, auricular region more commonly Cutaneous small vessel vasculitis may lead to purpura, bruise or ulcerative lesions over the skin. Pyodermagangrenosum sterile pustules which rapidly evolve over painful ulcers charactrized by raised edges with erythematous purple covering
  • 22.
  • 24. TUBERCULOSIS MODE OF INOCULATION DISEASE DIRECT INOCULATION Tubercular chancre(primary inoculation) CONTIGUOUS SPREAD Scrofuloderma MULTIBACILLARY AUTOINOCULATION Orofacial tuberculosis HEMATOGENOUS SPREAD Acute military tuberculosis, Tuberculous gumma(abcess)
  • 25. MODE OF INOCULATION DISEASE DIRECT INOCULATION Warty tuberculosis (verruca cutis) PAUCIBACILLARY HEMATOGENOUS SPREAD Lupus vulgaris, Lichen scrofulosorum, Nodular tuberculid, Erethema induratum
  • 26. PRIMARY INOCULATION Tubercular chancre skin lesion may vary from brownish papule ,nodule to an ulcer with undermined edge. On diascopy it shows apple jelly nodules Warty tuberculosis (verruca cutis) leisions occur on area exposed to trauma.Starts with a small indurated warty papule. Irregular extension may cause finger like
  • 27. SCROFULODERMA At first bluish-red nodule overlying the infected gland forms which breaks down to form undermined ulceration with granulating tissue at the base
  • 30.
  • 31. LEPROSY CLINICAL TYPE PERCATAGE DISTRIBUTION Tuberculoid (TT) 8 Borderline tuberculoid (BT) 41.33 Borderline (BB) 5.3 Borderline lepromatous (BL) 26.66 Lepromatous (LL) 18.66
  • 32.
  • 33.
  • 34.
  • 35. HIV seborrhoea dermatitis psoriasis herpes simplex viral warts mollusca oral and vaginal candidiasis scabies squamous cell carcinoma kaposis sarcoma
  • 36.
  • 37.
  • 39. THYROID DISORDERS Cutaneous features of hyperthyroidism • SKIN- palmar erythema ,facial flushing ,increased skin temp.,increased sweating, hyper pigmentation , pretibial myxoedema • NAILS- soft nails ,koilonychia,distal onycholysis (plummer’s nail) • HAIR- fine thin hair ,diffuse alopecia
  • 40.
  • 41. Cutaneous features of Hypothyroidism • SKIN-Pale, cold, scaly and wrinkled skin, Ivory- yellow skin colour, Xerosis, Absence of sweating, puffy oedema of hands, face and eyelids • NAILS- Brittle and striated nails • HAIR- Coarse sparse scalp hair, Loss of pubic, axillary and facial hair, Loss of lateral eyebrows (madarosis)
  • 42.
  • 43. ADRENAL DISORDERS Cutaneous manifestation of cushing’s syndrome • Truncal obesity (classically deposits of fat over the clavicles and back of the neck, the ‘buffalo hump’) • Facial fullness and plethora (‘moon facies’) • Slender limbs • Skin atrophy • Fragility, bruising and poor healing leading to Striae (typically white and red) • Hirsuties,Acneiform lesions • Male-pattern baldness in women
  • 44.
  • 45. Cutaneous manifestation of Addison’s disease • Hyperpigmentation of the skin, due to increased secretion of pituitary MSH and ACTH as a response to low adrenal corticosteroid levels, is the cardinal dermatological feature • Patterns of addisonian pigmentation - 1. Light-exposed areas i.e.face, dorsa of hands, 2. Areas subject to friction—elbows, knees, waistline 3. Accentuation of normally high pigmentation areas—genital, perineum, axillae, areolae, umbilicus 4. Palmar creases 5. Tongue and mucous membranes
  • 46.
  • 47. DIABETES MELLITUS Periungual talengectasia- A microangiopathic complication seen in upto 49% of diabetics Acanthosis nigricans- a verrucous hyper pigmented plaques seen over axilla , nape of neck which is due to hyperinsulinemia
  • 48. Granuloma annulare- annular leisions with raised skin colour having symmetrical distribution on arms,neck and upper half of the trunk Necrobiosis lipodica- sharply demarcated slightly depressed yellow waxy plaques with erythematous raised border
  • 49. Insulin reactions (Lipodystrophy)- soft nodules resembling lipoma are formed, these are local response to lipogenic action of insulin, preventable by rotating injection sites
  • 51. LIVER CIRRHOSIS Skin lesions associated with chronic liver disease •Spiderangiomas , telangiectasis •Palmar erythema •Dilated abdominal/chest veins (including periumbilical caput medusae) •Jaundice •Increased melanin pigmentation •Thin ‘paper-money’ skin •Loss of secondary sexual hair in males
  • 52.
  • 53. ACUTE PANCREATITIS Grey Turner sign (Turner sign)—tracks from the pararenal space to the edge of the quadratus lumborum muscle then through a defect in the fascia to the subcutaneous tissues of the flank (left sided in pancreatitis)
  • 54. Bryant’s sign—tracks to the scrotum to produce the ‘blue scrotum’ sign. Cullen’s sign—tracks into the falciparum ligament then through the connective tissues of the round ligament to the periumbilical area
  • 55.
  • 57. IRON DEFICIENCY DISORDER • Paleness of skin, palm creases and conjuctiva • Painful cracks at corner of mouth (angular cheilitis) • Shiny smooth tongue (atrophic glossitis) • Spoon shaped nails (koilonychiya) • Dry and brittle hair
  • 58.
  • 59. VITAMIN B COMPLEX DEFICIENCIES VitB3 (niacin)deficiency - Pellagra • Red skin with large blebs or blisters that exfoliates • Erethema over the dorm of hands • Scaling over the sun exposed area of the skin • Dry ,scaly and hyperkeratotic darkly pigmented skin • Site- mostly over face, neck ,arms, hands and feet
  • 60.
  • 61. B12 Deficiency - • Skin hyperpigmentation with accentuation in flexural areas ,palms ,soles and oral cavity • Angular stomatitis , glossitis(hunters glossitis), xerostomia • Vitiligo
  • 62.
  • 63. VITAMIN C DEFICIENCY SCURVY • Perifollicular hemmorage with blood pigment discolouration especially on the trunk and lower limbs (purpuric rash) • Swollen bleeding gums • Epistaxis may occur sometimes
  • 64.
  • 65. SYSTEMIC AMYLOIDOSIS Myeloma associated cutaneous amyloidosis (AL amyloid) Secondary systemic amyloidosis (AA amyloid) Dialysis associated amyloidosis
  • 66. Myeloma associated cutaneous amyloidosis (AL amyloid) • Almost 40% of patients with AL amyloidosis have skin manifestation • leisions tend to aggregate around mucocutaneous junctions like orbit, lips and genital skin • purpura is also a common finding which results from vascular fragility due to amyloid deposition in blood vessels
  • 67.
  • 68. Secondary systemic amyloidosis (AA amyloid) • The amyloids are composed of fibrils of a protein designated as AA • AA protein is an acute phase reactant • secondary amyloidosis occurs as a complication of many chronic inflammatory diseases • Skin lesions are wage and are non specific • Most common are renal manifestation leading to nephrotic syndrome
  • 69. Dialysis associated amyloidosis • cutaneous involvement is rare • manifests as hyper pigmentation ,lichenoid eruptions,or nodules that demonstrate amyloid deposition on HPE examination
  • 70. TAKE HOME MESSAGE CUTANEOUS MANIFESTATIONS CAN HELP IN EARLY DIAGNOSIS AND INTERVENTION OF MANY HIDDEN SYSTEMIC DISEASES IT ALSO HELPS IN UNDERSTANDING THE PROGNOSIS AND MANAGING THE DISEASE

Notes de l'éditeur

  1. Good afternoon everyone today i will be presenting seminar on dermatological manifestation of systemic ds
  2. There are 3 types of lupus
  3. This shows a discoid erythematous rash over the face This is a scalp leision showing alopecia and alopecia is usually permanent. Scalp leision occurs in almost 1/3 rd of pt. This is a typical erethematous rash with adherent scale over it
  4. These are lesions of disseminated dle these are actually classical dle leisions that may occur at any part of the skin
  5. Now here we can see the leisions are occurring typically above the waist
  6. This picture shows typical butterfly rash over cheecks
  7. This shows characterstick lupus hair with alopecia This is levido reticularid
  8. This is an early leision showing central paleness with waxy smooth and shiny surface This is frontopariteal morphea This is a typical scleroderma facies with decreased skin fold
  9. This shows radial furrowing round the mouth and decreased oral apurture This shows infarction and dry gangrene of fingers Due to sevre vasospasm
  10. This is a typical heliotrope erethema seen in dermatomyositis These are gotrons papule
  11. This is rheumatoid nodule This is pyoderma gangrenosum This is cutaneous small vessel vasculitis Showing purpuric leision over the skin
  12. Tubercular skin infection can be divided into multibacillary and paucibacillary types
  13. Lupus vulgaris has 5 forms Plaque form, ulcerative form , vegitative form, tumor like form, papular and nodular form
  14. Other types of tubercular skin leisions are lichen scrofulosorm. Tubercular gumma
  15. Sensory loss
  16. Here we can see a patient of seborrhoic dermatitis Showing itchy scaly patches over face Extensive forms may some times lead to erythroderma in aids pt.
  17. This is a picture showing lady with hypothyroid features