Cutaneous manifestations of systemic disease

CUTANEOUS MANIFESTATIONS OF SYSTEMIC DISEASES

DUPUYTEN’S CONTRACTURE

• Fibromatous hyperplasia of the palmar aponeurosis, nodular thickening of fascia with associated flexion contractures of 1 or more digits.

• Familial,alcoholism, cirrhosis, epilepsy and diabetes mellitus,TB.

• Polyfibromatosis syndrome• Periarthritis of shoulder,chronic lung

disease,gout,trauma and ulnar nerve damage

DIABETIC DERMOPATHY(DIABETIC SHIN SPOTS)

• Shins, forearms, thighs, bony prominences

• Initial lesion oval dull red papule evolves slowly producing superficial scale, leaving an atrophic brownish scar.

THE DIABETICFOOT SYNDROME

• The cutaneous lesion is a plantar ulcer, often accompanied by soft tissue and bone infections which can require amputation.

• TRIGGERS include poorly fitting shoes, poor foot care, or overlooked foreign bodies, coupled with a structural foot deformity.

PATHOGENESIS OF DIABETIC FOOTSYNDROME

THERAPEUTIC GOALS

• The most important is optimal control of the diabetes mellitus

• Relieving pressure points and

• Avoiding or reducing callus formation

Association- 0.3 % of diabetic patients. Relatively asymptomatic lesions, F:M=3:1Characteristically found on the anterior and lateralsurfaces of the lower legs.

- Bullae appear spontaneously, usually on the extremities, especially the feet.- Heal in several weeks without significant scarring, although they may recur.

• Development of stiff, thick skin that resembles the changes in scleroderma

• Most apparent at acral sites, and may be associated with limited joint mobility (also known as cheirarthropathy) due to the abnormal collagen.

• The proposed mechanism is an increased cross-linking of collagen, known as non-enzymatic glycosylation (NEG), which produces stiffer collagen.

DIABETIC CHEIROARTHROPATHY

ACANTHOSIS NIGRICANS

• Cutaneous marker, most commonly of insulinresistance and less frequently of a malignancy.

• Hyperpigmented, hyperkeratotic, verrucous plaques that bestow a velvety texture on involved skin.

• Typically symmetric in distribution• Involves intertriginous areas, including the neck, axillae,

groin, antecubital and popliteal fossae, and umbilicus • Occasionally involves the oral, esophageal, pharyngeal,

laryngeal, conjunctival, and anogenital mucosae

INSULIN RESISTANCE–RELATED

OBESITY

ENDOCRINOPATHIES: acromegaly, cushing’s, hypo and hyperthyroidism, Polycystic ovarian disease (Stein-Leventhal syndrome)

MALIGNANCY-RELATED • Adenocarcinoma: gastric• Endocrine: phaeochromocytoma,testicular, thyroid,carcinoid• Melanoma, Sarcoma• Lung• Lymphomas • Other: cervical, urinary bladderDRUG-INDUCED: Testosterone, nicotinic acid, diethylstilbestrol, oral

contraceptives, triazinate, glucocorticoids, and with topical application of fusidic acid.

IDIOPATHIC• Familial• Nonfamilial

DIABETIC FINGER PEBBLES

• An exaggeration of the skin markings over the knuckles and on the dorsal aspect of the terminal phalanges.

• They are more cobblestoned than knuckle pads.

REACTIVE PERFORATING COLLAGENOSIS

SCLEROEDEMAAreas of induration frequently after infection, spontaneously clear in months or years.

CUTANEOUS FEATURES ASSOCIATED WITH DIABETES MELLITUS

• Infections• Peripheral vascular and small-vessel disease • Neuropathy • Pruritus• Necrobiosis lipoidica • Granuloma annulare variants • Perforating disorders • Xanthomas • Scleredema • Drug-related • Features of associated autoimmune disease

DIAMOND TRIAD

The combination of acropachy with

exophthalmos and pretibial myxedema.

PRETIBIAL MYXEDEMA

• Localized oedematous and thickened pretibial plaques

• 1-10% hyperthyroidism

• Erythema and slight edema are the early signs, with subsequent peau d'orange appearance as the degree of infiltration increases.

THYROID ACROPACHY

• Clubbing of fingers and toes in Grave’s disease associated with soft tissue swelling of hands and feet with periosteal new bone formation.

• A number of other causes, including bronchial neoplasia.

CUTANEOUS FEATURES RELATED TO ABNORMALITIES OF THYROID

HORMONE LEVELS (THYROTOXICOSIS)SKIN • Soft, smooth, velvety, warm, Palmar erythema, facial

flushing • Pruritus • Hyperpigmentation• Pretibial myxedema• May also be features of associated autoimmune

diseases, e.g. vitiligo, urticaria   SWEATING: Increased NAILS: Fast growth, Soft nails, koilonychia, distal

onycholysis,Thyroid acropachy HAIR: Fine thin hair, diffuse alopecia,Loss of pubic, axillary,

and facial hair,Loss of lateral eyebrowsORAL: Large tongue, gingival swelling, oral candidosis.

HERTOG’S SIGN

CUTANEOUS FEATURES RELATED TO ABNORMALITIES OF THYROID

HORMONE LEVELS (HYPOTHYROIDISM)SKIN • Pale, cold, scaly, wrinkled• Puffy edema of hands, face, and eyelids• Purpura and ecchymoses• Pruritus (due to xerosis or asteatotic eczema)• Ivory-yellow color (in part due to carotenemia)• Xanthomatosis (secondary to hyperlipidemia)SWEATING: DecreasedNAILS: Slow nail growth, brittle and striated nailsHAIR: Coarse hair, diffuse alopecia

ESTABLISH THE DIAGNOSIS AND RULE OUT DIFFERENTIAL DIAGNOSES

• PALPATE PURPURA

• CHECK TEMPERATURE and assess if patient is unwell

• Complete blood examination (CBC) platelets

• SKIN biopsy for HP&direct IF

D/D OF NONPALPABLE PURPURAI. PROCOAGULANT

DEFECT

A. Hemophilia

B. Anticoagulant use

C. Disseminated intravascular coagulation

D. Vitamin K deficiencyE. Hepatic insufficiency with

poor procoagulant synthesis

II. POOR DERMAL SUPPORT OF VESSELS

A. Actinic (senile) purpura

B. Glucocorticoid therapy, topical or systemic

C. Vitamin C deficiency (scurvy)

D. Systemic amyloidosis (light chain–related, some familial types)

E. Ehlers-Danlos syndrome (some types)

F. Pseudoxanthoma elasticum

IDENTIFY THE CAUSE

• pANCA• Streptococcal serology or throat swab

(especially in children)• Erythrocyte sedimentation rate (ESR), urea and

electrolytes (U&E), liver function tests (LFTs)OTHER INVESTIGATIONS• Findings on history including hepatitis serology,

rheumatoid factor, cryoglobulins, immunoglobulins, antinuclear factor (ANF), total complement levels and serum protein electrophoresis.

ASSESS THE EXTENT OF THE VASCULITIS

Further investigations should be directed toward

investigating relevant systems:

• RENAL – urinalysis looking for proteinuria and haematuria

• GASTROINTESTINAL – abdominal pain, gastrointestinal bleeding

• MUSCULOSKELETAL – nonerosive polyarthritis

• PULMONARY – pleural effusion

• CARDIAC – pericardial effusion.

MANAGEMENT

• General principles of management for vasculitis: Rest, elevation of the legs and compression hosiery.

• Systemic therapy indicated if the vasculitis is extensive, painful, ulcerating, or if there is renal involvement.

• The cause of the vasculitis should be managed as appropriate.