Cutaneous manifestation of

systemic disorders

Dr.S.Ahamed Uwyse
Consultant Dermatologist
NCTH
Ragama
 Cutaneous manifestations of internal malignancies

Acanthosis nigricans
• velvety thickening and hyperpigmentation of the skin mainly involving the major
flexures , but can affect any area of the body
• if it occurs in both palm called ‘Tripe palm’.
• Commonly associated with GI malignancy
Erythema gyratum repens
• Erythematous, serpiginous and concentric scaly
rash (like wood grain) occurring on trunk .

• Associated with lung and GI malignancy

 Acquired hypertrichosis lanuginosa

• Widespread growth of fine lanugo hair mainly in face and on the body due to internal
malignancy.

• associated with lung, breast, colorectal malignancies

Necrolytic migratory erythema
• Figurate erythema with moving crusted edge.

• Associated with pancreatic carcinoma.

Dermatomyositis
• DM in adults may be associated with internal malignancy (30% cases).

• Ovarian CA may commonly be associated

Acquired ichthyosis

Internal malignancy may cause acquired ichthyosis , commonly associated with

lymphoma

Generalised pruritus
• It may be associated with internal malignancy, particularly with lymphoma
Paraneoplastic pemphigus
it’s look like pemphigus vulgaris , but with extensive mucosal ulceration.
associated with myeloproliferative malignancies and other internal malignancies as well

Sweet syndrome
associated with erythematous, painful plaque lesions (sometimes bullous lesions)with fever,
raised neutrophil count and ESR
 Skin manifestation of diabetes mellitus

Necrobiosis lipoidica .
 If it is associated with diabetes NLD (NL diabeticorum)
 less than 3% of diabetics develop NLD.
 Non diabetic pts with NL may develop DM later.
 NL may occur in rheumatoid arthritis .
 Atrophic, shiny , brownish red or slightly yellowish plaque lesion develop over the shin
commonly.
It may become ulcerated .
 It’s a necrobiotic condition, where degeneration of collagen of the skin and
subcutaneous fat occurs, probably due to microangiopathy. This necrobiotic process
induces granulomatous inflammation.
Treatment .
 Difficult to treat.
 Superpotent topical steroid therapy or intralesional steroid injection may be tried.
 Topical calcineurin inhibitors may be useful.
 Aspirin , dipyridamole, pentoxyphiline may be tried to improve the circulation
Granuloma annulare
it’s also a necrobiotic lesion.
GA occurring in adults may be associated with DM.
it’s a anular type of a lesion which commonly occurs over the knuckels , dorsum of the
hand and feet , but may occur anywhere.
May clear spontaneously after few years.
Can be treated with topical or intralesional steroid

Pallisading granuloma
Diabetic dermopthy
asymptomatic, atrophic, hyperpigmented patchy lesions found on the shins of both
legs due to microvascular complication
it occurs in 40% of diabetic pts , commoner in men
treatment is not necessary and no treatment is effective
when it heals , leaving pigmented, depressed and atrophic scar
it may occur in non diabetics as well.
Diabetic bullae
painless, noninflamatory, spontaneous small or large bullae (>10cm) occurs in the
acral region, mainly lower in the limb.
in many cases, it may heal spontaneously in 4-5 weeks without scarring.
chronic ulcer may occur develop in some cases

Acanthosis nigricans
Limited joint mobilities and waxy skin
skin becomes thick and waxy mainly in hands and
digits, then spread to the upp.limb, trunk and lower
limb as well.
stiffness of the joints , particularly in the small joints of the hands, giving the’ prayer sign’
Neuropathic ulcers
 Pyoderma gangrinosum

 it’s a neutrophilic dermatosis

 commonly occurs in adults, 40-60 age
 typically present in the lower extremities and trunk but may occur anywere
 Start as a pustule,. then suddenly enlarge and ulcerate. Painful ulcer with well
defined, undermined edge . Border of the ulcer looks bluish or purplish disclouration.
satellite lesions may develop at the border and may coalesce with the main ulcer.
ulcer heals with atrophic scar.
 bullous PG, presents with large bullae
 vegetative PG, least aggressive form.
 50% of the cases are associated with internal diseases
inflammatory bowel diseases(2-5% of the IBD presents with PG), ulcerative colitis, crohn’s
polyarthritis -- rheumatoid arthritis , seronegative arthritis , some time mono arthritis
haematological malignancies – leukemia, multiple myeloma,IgA monoclonal gammopathy
polycythemiarubra vera
chronic active hepatitis , SLE, pregnancy
Histology
Suppurative inflammation with dense neutrophilic infiltrate
lymphocytes and plasma cells also be present
secondary leukocytoclastic vasculitis present (about 40% cases)
granulomatous inflammation may also be present in PGs associated with IBD
Treatment
mild cases potent topical or intralesional steroid therapy
oral steroid therapy (1-2 mg/kg bw/day)
cyclosporine , azathioprine, oral tacrolimus , mycophenalate mofetil
infliximab
 Erythema nodosum
 It’s a inflammatory septal panniculitis
 Common in young adults , but may occur at any age
commoner in female than male
 The lesions develop as erythematous, tender nodules or plaques, more commonly over the shins
symmetrically, but may occur anywhere in the body.
may be associated with fever, arthritis or arthralgia, leg oedema,
conjunctivitis and episcleritis, and various GI symptoms
 Causes
infection – streptococcal (common in children), TB , intestinal infection(salmonella,shigella)
fungal infection and some other infection as well
inflammatory bowel diseases (ulcerative colitis, crohn’s disease)
behcet’s syndrome
sarcoidosis
drugs (sulphonamides, iodides ,bromides, oestrogen – OCP , NSAIDS)
haematological malignancies rarely
in many cases , idiopathic
 Histology
septal panniculitis
neutrophil infiltrate in early stage, lymphocytes and mononuclear cells in later stages,
in chronic lesions , granulomatous inflammation may occur.
leukocytoclastic vasculitis is not the feature in EN
 Lesions heal without scarring after weeks , ulceration doesn’t occur
 Chronic form of EN occurs in elderly (women), lesions are unilateral or asymmetrical ,
painless, not associated with systemic symptoms , not associated with systemic disorders,
have a prolong course for months or years.
 Treatment
bed rest , analgesics like NSAIDS
oral steroid therapy in severe cases
colchicine may be useful in acute cases
intralesional steroid therapy in resistant cases
in chronic cases saturated solution of potassium iodide (SSKI) often effective
 Skin and liver
 Generalised pruritus
this is due to obstruction to bileduct, resulting in increased level of bile salts deposited in
the skin
 Hyperpigmentation of the skin
haemachromatosis (increased amout of melanin rather than iron deposition) and cirrhosis (PBC)– increased
amount of melanin widely dispersed in the epidermis and dermis
 Spider naevi and palmar erythema
due to increased level of oestrogen, dilating the blood vessels
 Licchen planus in hepatitis c infection
 Cryoglobulinaemic vasculitis in hep c infection
 Xanthomas in primary biliary cirrhosis
 Hair loss may occur in alcoholic cirrhosis (zn deffiency)
 White nail due to hypoalbuminaemia
haemochromatosis White nail

Spider naevi
 Skin and endocrine diseases
Cushing syndrome
hypertrichosis , AN, acne , erythema of cheeks and ‘V’ area of the chest
skin atrophy, atrophic striae , hyperpigmentation (if ACTH is increased)

Acromegaly
diffuse hypertrophy of the skin
Cutis verticis gyrate (30% 0f the cases)
hypertrichosis, hyper pigmentation and hyperhidrosis occurs in many pts
Addison’s disease
diffuse hyperpigmentation of the skin due to increased ACTH level , predominantly in sun
exposed areas
darkening of the palmar creases, darkening of the existing naevus
hyperpigmentation of the scar, darkening of the nail
decreased pubic and axillary hair in female

Hyperandrogenism
seborrhea and acne, hirsutism, androgenetic alopecia, masculine features in female
Hypothyroidism
skin becomes dry and thick
periorbital puffiness and facial puffiness and
madarosis
hair becomes coarse and brittle and loss of hair ,
onycholysis
Hyperthroidism
pretibial myxedema and thyroid acropachy (soft tissue swelling of the hand and clubbing
of the fingers )
palmar erythema and facial flushing
hair is thin and diffuse loss of hair
onycholysis
 Skin manifestation of renal diseases
 Skin becomes dry and scaly with pruritus , uremic frosting of the skin
 Looks pale due to anaemia
 Half and half nail (Lindsay’s nail) – proximal half is white and distal half is pink
 Acquired perforating dermatosis (perforating folliculitis)
 Skin and hyperlipidaemia
 xanthomas occurs in the skin due to primary (type 1- V ) or secondary hyperlipidaema
(diabetes , hypothyroidism )
 xanthelesma palpebram – yellowish plaque lesion on the eye lids, can occur in the pts with
normal level of serum lipids
 tuberous xanthoma -- tuberous lesions commonly occurs on the ext.aspect of the
knees and elbow
 tendinous xanthoma -- nodular lesions attached to the tendons of the hands and feet
 eruptive xanthoma -- sudden onset of widespread yellowish papules on the skin due to
primary and secondary (diabetes) HL
 plane xanthoma -- macular yellowish lesions over the palmer creases or on the any
part of the skin
 Cutaneous Porphyria
 Due to accumulation of porphyrins (precursor molecules of haem synthesis) in the skin.
 Porphyrins are photosensitive agents which causes photosensitive reaction in the skin.
 Congenital erythropoietic porphyria
severe photosensitive reaction occurs in the newborn, causing blistering and scarring in
the exposed area of the skin.
increased hair growth also occurs in these areas
it is an AR condition
 Erythrohepatic protoporphyria
it is an AD condition
photosensitive reaction occurs during infancy , skin become reddish, odematous, blistering
and crusted
 Porphyria cutanea tarda (cutaneous hepatic porphyria)
it occurs in pts with liver disease (alcoholic liver disease)
exposed areas of the skin (mainly face and dorsum of the hand)become damaged with
blistering , scarring, post inflamatory hyperpigmentation and excessive hair growth.

Porphyria cutaneous tarda Erythropoietic protoporphyria

Thank you