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 sacral 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