Teaching Clinic: Cutaneous Manifestation Of Systemic Disease Flashcards
SLE cutaneous presentation
***Specific manifestations:
Acute SLE:
- Malar butterfly rash
Subacute SLE:
- Annular urticarial like eruption (Ring-like insect bite)
- Erythematous scaly plaques with psoriasiform appearance
(vs Psoriasis: Chronic inflammatory dermatosis that affect extensor surface + scalp)
Discoid LE:
- Scaly erythematous patch with ***scarring, pigmentary changes, follicular plugging
(Scarring: smooth shiny skin without hair follicle)
***Non-specific manifestations:
1. Palms with skin atrophy, erosion, hyperkeratosis
2. Mucosal ulceration and nose bleeding
3. Diffuse / Scarring alopecia
4. Raynaud’s phenomenon
5. Nail fold telangiectasia and infarct
6. Splinter haemorrhages, Chilblain LE
7. Acquired C1 esterase deficiency
8. Urticaria vasculitis
9. Purpura
10. Thrombophlebitis
11. Livedo reticularis
12. Phospholipid syndrome
13. Bullous LE
14. LE profundus
15. Erythema multiforme (Rowell’s syndrome)
16. LE / Lichen planus overlap
Medium vessel vasculitis
- Polyarteritis nodosa (with systemic involvement)
- Livedo vasculitis(cutaneous only)
- Tender nodules, 5-10mm in diameter
- Often livedo reticular pattern
- Ulceration not uncommon
Treatment:
1. NSAID
2. Colchicine
3. Dapsone
4. Antiplatelet
5. Immunosuppressive agents
Panniculitis
- Inflammation of Subcutis
Histopathological classification:
1. Lobular with vasculitis
2. Lobular without vasculitis
3. Septal with vasculitis
4. Septal without vasculitis
Septal panniculitis
With vasculitis:
1. Superifical migratory thromophlebitis
2 ***Polyarteritis nodosa
3. Livedo vasculitis
Without vasculitis:
1. **Erythema nodosum
2. Scleroderma panniculitis
3. **Lipodermatosclerosis
4. Eosinophilic fasciitis
5. Eosinophilic myalgia syndrome
Lobular panniculitis
With vasculitis:
1. ***Erythema induratum
Without vasculitis:
1. Weber Christian (Idiopathic condition, associated with liver derangement; requires prompt steroid)
- Systemic
- Pancreatic
- SLE
- Sarcoidosis
- Calcifying panniculitis of renal disease
- Lymphoma / Leukaemia
- Infection - Physical
- Cold
- Traumatic
- Chemical
- Factitial - α1-antitrypsin deficiency
- Neonatal
- Sclerema neonatorum
- Neonatal subcutaneous fat necrosis
Erythema nodosum
- Septal without vasculitis
- Immunological reaction triggered by a wide range of factors
- Erythematous, tender nodules in anterior shins and lower limbs
- Associated with systemic symptoms (fever, malaise)
- Resolve with bruise in 3-6 weeks
- Can ulcerate
Causes:
1. Infective
- Strept
- TB
- Mycoplasma
- Leprosy
- Yersinia
- Leptospirosis
- Histoplasmosis
- Infectious mononucleous
- Drug
- Sulfonamides
- Bromide
- Oral contraceptive - IBD
- Crohn’s
- UC - Malignant
- Lymphoma
- Post-RT
- Behcet’s
Treatment:
1. Treat underlying cause
2. NSAID
3. Immunosuppressive therapy
4. Potassium iodine
Erythema induratum / Nodular vasculitis
- Lobular with vasculitis
- Known association with ***TB
- ?Immune complex deposition
- Tender nodules in calf + lower limbs
Investigations:
1. Mantoux test
2. Early morning urine
3. CXR
Treatment:
- Triple therapy for all patients in HK
- If mantoux test -ve: Immunosuppressive therapy
(Cutaneous TB:
- Erythema induratum: 79.5%
- Lupus vulgaris: 6.3%
- Tuberculosis verrucosa cutis: 4.5%)
Approach to Chronic recurrent painful nodules
- CBC, LRFT
- ANA, ANCA (to exclude Polyarteritis nodosa)
- Incisional deep skin biopsy + Immunofluorescence
- CXR
- Screen for TB: Mantoux test, Early morning urine, Sputum for AFB (if Erythema induratum)
***DM cutaneous presentation
Insulin
- Regulates glucose deposition into skin cells
- Affect growth and differentiation of keratinocytes (Promote growth and induce comedo)
- Affect dermal fibroblastic function therefore dermal collagen formation
- Acute metabolic derangement
- **Infections
—> Staphylococcal pyodermas
—> Candidiasis
—> Erythrasma
—> Epidermophytosis
- **Xanthomatosis - Chronic degenerative changes
- ***Diabetic dermopathy
- Erythema + Necrosis
- Bullous lesions
- Scleredema adultorum (of Buschke)
- Thickened skin and stiff joints - Others
- **Necrobiosis lipoidica diabeticorum
- Granuloma annulare
- **Acanthosis nigricans
- Perforating disease (Kyrle’s disease) - ***Cutaneous reaction to diabetic treatment
- Sulfonylurea
- Insulin
Erythrasma
- Superficial infection due to Corynebacterium minutissimum
- Presents as well-defined brownish patches in the intertriginous areas (axilla, toe web)
- 20% of subjects could be affected in tropical countries
Treatment:
- Erythromycin (2 week course)
Epidermophytosis
- Prevalence not increase
- More resistant to treatment
- Increase risk to secondary bacteria infection
- Prompt treatment is necessary to prevent complication
Treatment:
- Terbinafine (Lamisil) is very effective after microbiological confirmation
Xanthomatosis
- Eruptive xanthoma
- Itchy, multiple red papules in extensor surface and buttock
- Contain lipid rich histocytes - Xanthelasma
- Tuberous xanthoma
Treatment:
1. Control metabolic derangement
2. Topical / Intralesional steroid
3. Chemical peel
4. Laser resurfacing
Causes of Chronic degenerative changes
- Microangiopathy leads to reduce in cutaneous blood flow
- Changes occurs in dermal connective tissue
- Cutaneous innervation abnormality
- Changes correlate with other systemic degeneration
Diabetic dermopathy
- Atrophic brownish patches in lower limbs
- Asymptomatic
- Appears and disappear over 12-18 months
- Constant appearances of new lesions, create a stationary impression
- M:F = 2:1
Erythema + Necrosis
- Rubeosis faciei (facial erythema)
- Erythema in limbs: even erysipelas appearance
- Can proceed to necrosis and ulceration
- Edema
- Signs of arterial insufficiency
- Gangrene
Bullous lesions
- Rare
- On extremities
- Subepidermal
- Appear spontaneously and heal without scarring
- Causes unknown but 75% have significant diabetic retinopathy
Scleredema adultorum (of Buschke)
- Skin induration in back and neck
- Spread to other area such as face, shoulder, anterior neck
- Well demarcated from normal skin
- Due to ?Altered metabolism of collagen and mucopolysaccharide as a result of insulin insufficiency
- Can be associated with ***streptococcal infection
- ***Asymptomatic in most cases but can associated with pain and discomfort
- Need to rule out **Scleroderma + **Dermatomyositis
- Treatment: disappointing
Thickened skin and stiff joints
- Mainly in IDDM and NIDDM
- Due to altered soft tissue formation
- Increase skin thickness due to thickened basement membrane and collagen deposition
- Can occurs in hands and over joint
- Joint contracture
Necrobiosis lipoidica diabeticorum
- Not specific to DM
- 3/1000 of DM patients
- 90% of patients with NLD have DM or positive FH of DM
- Hyperglyceraemia not necessary for NLD (Hba1c could be normal)
- ?Microangiopathy
- ?Vasculitic
- ?Delayed hypersensitivity
Clinical features:
- Translucent base and able to see vessels underneath
- Erythematous papules that coalesce into large plaques in the pretibial area
- Yellow atrophic center with telangiectasia
- Could be multiple
- 1/3 ulcerated
- 50% of DM cases have neuropathy / retinopathy
- 10-20% spontaneously resolution
Treatment:
1. ?Benefit of tight control
2. Topical potent / Intralesional steroid
3. Antiplatelet agents (Aspirin, Dipyridamole)
4. Fibrinolytic therapy (Nicotinic acid derivative)
Granuloma annulare
Ring-like granuloma
Types:
- Localised
- Generalised
- Perforating
- Subcutaneous
Clinical features:
- Erythematous dermal papules arranged in an annular pattern
Treatment:
1. Topical / Intralesional steroid
2. PUVA therapy
Acanthosis nigricans
- Papilloma with hyperpigmentation in axilla, inguinal, inframammary folds and neck
Severe form:
- Could also involve the palms, sole and mucosal area
- Linked to malignant disease
Benign form:
- Associated with insulin resistance
- Insulin acts on IGF receptors
Associated diseases:
Benign:
1. Acromegaly
2. DM
3. Obesity
4. Cushing’s disease
5. Polycystic ovary disease
Malignant:
1. Lung, Breast, Uterus, Ovarian, GI
- associated with more extensive disease and could also involve the mucosa
- associated with Leser-Trelat sign
Perforating disease (Kyrle’s disease)
- Transepidermal elimination of degenerative collagen or elastin (Immune system attempts to clear collagen off in CKD patients)
- End-stage DM nephropathy
- Hyperkeratotic, pruritic papules on the legs
- Coalescing into large plaques
Treatment:
1. Topical steroid
2. UV therapy
Cutaneous reaction to diabetic treatment
Sulfonylureas:
- First generation agents such as chlorpropamide or tolbutamide
- Allergic or toxic
- Chlorpropamide alcohol flushing (10-30%)
- Maculopapular, morbilliform rashes
- Urticaria
- Photoallergic
- Lichenoid
- EM
Insulin:
- Insulin allergy
- Lipoatrophy
- Lipohypertrophy
- Keloid
- Pigmentation at injection site
Neoplasm cutaneous presentation
Circulating factors leads to paraneoplastic syndrome:
1. Acanthosis nigricans
2. Acquired icthyosis
3. Amyloidosis
4. Carcinoid
5. Clubbing
6. Coagulopathies
7. Cryoglobulinaemia
8. Cushing
9. Dermatomyositis
10. Erythema gyratum repens
11. Hypertrichosis lanuginosa (increased hair growth in pancreatic Ca)
12. Leser-Trelat (explosive onset of multiple seborrhoeic keratoses)
13. Pemphigus
14. Necrolytic migratory erythema
