A Case History with Photos [EN and Sarclodosis]

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Clinical case series

Doctors admitted this 45 year old woman into hospital with a six

week history of arthralgia and night sweats and a painful rash over the

anterior aspects of the ankles. She had no complaints of shortness of

breath or cough. Neither had she travelled abroad recently.

On examination she had tender and swollen elbows, wrists, and

ankles bilaterally. Her chest examination was unremarkable. The rash

seen on examination is shown in fig 1.

Doctors did a number of blood tests on admission. One of these

found angiotensin converting enzyme (ACE) at a concentration of 113 U/l

(normal range 27-100 U/l).

Doctors also did a chest radiograph shown in fig 2.

Four months later at follow up she had symptomatically improved and her serum ACE level had fallen to 24 U/l.

Questions

Describe the skin lesion(s). What is the most likely diagnosis?

What is the significance of the abnormal blood result?

Describe the findings on the chest radiograph.

What is the diagnosis and what other skins lesions may be seen in this condition?

What further tests may be indicated, and how is this condition managed?

Answers

There is a large well demarcated area of erythema (redness) over

the dorsum (front) of both feet extending over the lateral and medial

malleoli. It appears slightly raised. A similar lesion was noted over

the extensor aspect of the left knee. It was tender to touch on

examination. The most likely diagnosis is erythema nodosum. This is a

panniculitis (inflammation of the subcutaneous fat).

Potential causes of this include:

Tuberculosis

Inflammatory bowel disease

Throat infection (streptococcal)

Sarcoidosis, sulphonamides, and other drugs (including the oral contraceptive pill)

Less commonly viral, fungal and atypical bacterial infections can be the cause.

An elevated angiotensin converting enzyme (ACE) concentration

is consistent with the diagnosis of sarcoidosis but is non-specific.

ACE concentrations can be used to monitor disease activity and response

to treatment. This woman's ACE concentration returned to normal within

four months. This corresponded with her symptomatic and clinical

improvement. The other most commonly abnormal blood result in sarcoidosis is

serum calcium. Hypercalcaemia is a common finding--this again is

suggestive of sarcoidosis if found alongside other typical clinical

features--but is non-specific. The hypercalcaemia of sarcoidosis is

responsive to steroids.

There is evidence of bilateral hilar lymphadeno-pathy

with a slightly bulkier left hilum. There is no evidence of

interstitial pathology of the lungs. The differential diagnosis for these findings includes:

Malignancy (especially bronchial carcinoma)

Lymphoma

Tuberculosis

Sarcoidosis

Lung

involvement in sarcoidosis can be divided into four stages--0: normal

chest radiograph; 1: bilateral hilar lymphadenopathy; 2: diffuse

infiltrative lung disease and bilateral hilar lymphadenopathy; 3:

diffuse infiltrative lung disease.

This woman's clinical features and investigation results

indicate a diagnosis of sarcoidosis. This is an idiopathic multisystem

disorder characterised pathologically by the presence of granulomas.

This is a mass of chronically inflamed tissue. The triad of erythema

nodosum, arthralgia and bilateral hilar lymphadenopathy are typical of

acute sarcoidosis. These features along with a swinging fever are

referred to as Lofgren's syndrome. In addition to erythema nodosum, as

seen in this woman, there are a number of skin conditions associated

with sarcoidosis including: Lupus pernio (this is a violet coloured nodular rash typically over the nose)

Scarring alopecia (areas of hair loss following scarring of the skin)

Scar sarcoid (infiltration of scar tissue)

Further investigations should be largely based on clinical

features. Pulmonary function tests, including transfer factor and high

resolution computed tomography of the chest are important in the

presence of any respiratory symptoms. Transfer factor is a measure of

the diffusion capacity of the lungs. A small amount of carbon monoxide

is used to test the quality of gas transfer from the lungs to the

blood. Pulmonary function tests were normal in this patient. With stage

two and three lung disease the picture is typically restrictive with a

reduced transfer factor. Treatment of sarcoidosis is largely steroid

therapy in chronic disease. Acute disease will often resolve

spontaneously. The prognosis for this woman is good given her acute

presentation with erythema nodosum and bilateral hilar lymphadenopathy.

Ian Bickle, senior house officer

Email: clonvara@...

beth McCausland senior house officer, Royal Hospital, Belfast

We thank M Finch, consultant rheumatologist, Musgrave Park Hospital, Belfast.

studentBMJ 2005;13:45-88 February ISSN 0966-6494