Systemic lupus erythematosus

162 Systemic lupus erythematosus



Instruction


Examine this patient’s face (usually a young woman).



Salient features



History




• Fatiguability and tiredness: suggests anaemia


• Joint symptoms: particularly small joints (90% of patients)


• Gangrene of the digits: vasculitis


• Skin rash (butterfly rash on face; Fig. 162.1) in sun-exposed areas, livedo reticularis, alopecia, Raynaud’s phenomenon


• Mouth ulcers


• Hypertension, oedema (suggesting renal involvement)


• Fever, enlargement of lymph nodes


• Bleeding from gums excessive menstrual bleeding, purpura (caused by thrombocytopenia)


• Neuropsychiatric symptoms, seizures


• History of remissions and exacerbations


• Drug history (hydralazine, procainamide, minocycline).


image

Fig. 162.1 Malar rash in a patient with systemic lupus erythematosus. Note that the rash does not cross the nasolabial fold.


(With permission from Klippel, Dieppe 1998.)



Examination




• Butterfly rash: follicular plugging, scales, telangiectasia and scarring affecting the bridge of the nose and cheeks (the patient may be cushingoid because of steroids)


• Examine the following:


• Conjunctiva for anaemia (often Coombs’ test positive)

• Mouth ulcers (seen in one-third of cases; Fig. 162.2)

• Scalp for alopecia

• Sun-exposed areas and elbows for vasculitic rash, subcutaneous nodules

• Nails for splinter haemorrhages, nailfold capillaries and periungal infarcts

• Hands for palmar erythema, Raynaud’s phenomenon (Fig. 161.1), arthritis

• Knees for vasculitic rash

• Feet for secondary oedema (secondary to nephrotic syndrome).

• Tell the examiner that you would like to examine the urine for proteinuria.


image

Fig. 162.2 Oral mucosal ulcerations: generally painless and can present on other cutaneous tissues.


(With permission from Powers 2008.)


Note: SLE principally affects skin, joints, kidney and serosal membranes.



Diagnosis


This patient has a butterfly rash of the face with telangiectasia (lesion) caused by systemic lupus erythematosus (aetiology) and has renal failure, probably caused by lupus nephritis as evidenced by the haemodialysis catheter (functional status).



Advanced-level questions




What is the histology of the skin rash in systemic lupus erythematosus?




• Liquefactive degeneration of the basal layer of the epidermis together with oedema at the dermoepidermal junction. Immunofluorescence microscopy shows deposition of immunoglobulin and complement along the dermoepidermal junction.


• Oedema of the dermis accompanied by infiltrates of perivascular mononuclear cells.


• Vasculitis with fibrinoid necrosis of the vessels.



What are the skin manifestations of systemic lupus erythematosus?


Butterfly rash, periungal erythema, nailfold telangiectasia, alopecia, livedo reticularis, hyperpigmentation, urticaria, purpura, scarring eruption of discoid lupus.



What are the criteria for diagnosis of systemic lupus erythematosus?


Any four of the following 11 criteria are required to make a diagnosis:



• Malar rash


• Discoid rash


• Photosensitivity


• Oral ulcers


• Arthritis: non-erosive arthritis


• Serositis: pleuritis, pericarditis


• Renal involvement: proteinuria, cellular casts (nephritis is more common in patients with anti-native DNA)


• Neurological involvement: seizures, psychosis


• Haematological involvement: haemolytic anaemias, leukopenia, thrombocytopenia


• Anti-nuclear antibody (ANA): seen in over 95% of patients


• Immunological disorder: positive for LE cell (a specific type of cell found in SLE), anti-DNA antibody, false-positive syphilitic serology.

Related posts:

  1. Dermatomyositis
  2. Arteriovenous fistula
  3. Vitiligo
  4. Anaemia

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Dec 4, 2016 | Posted by in GENERAL & FAMILY MEDICINE | Comments Off on Systemic lupus erythematosus

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