Lupus pernio

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169 Lupus pernio

Instruction

Look at this patient’s face.

Salient features

History

• Shortness of breath, cough, chest discomfort

• Fatigue, weight loss, malaise, anorexia

• History of therapy with interferon-alfa (particularly in patients with hepatitis C; interferon-alfa increases interferon-γ and interleukin-2, thus promoting granuloma formation).

Examination

• Reddish blue or violaceous plaques on the nose (Fig. 169.1), cheeks, ears and fingers, with telangiectasia over and around the plaques (Fig. 169.2).

image

Fig. 169.1 Lupus pernio plaques on the nose.

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Fig. 169.2 Telangiectasia over and around the plaques.

Proceed as follows:

• Tell the examiner that you would like to do radiography for hilar adenopathy and examine the eyes for ocular manifestations

Diagnosis

This patient has violaceous plaques on his face (lesion) caused by sarcoidosis (aetiology), which is cosmetically disfiguring (functional status).

Advanced-level questions

What is the differential diagnosis of such a lesion?

• Rhinophyma (p. 633)

• Lupus vulgaris

• Leprosy.

What are the cutaneous manifestations of sarcoid?

• Erythema nodosum (p. 665)

• Micropapular sarcoid

• Scar infiltration

• Sarcoid plaques of limbs, shoulders, buttocks and thighs.

How may sarcoid present?

• Acute sarcoidosis usually presents in the third decade, characterized by erythema nodosum, parotid enlargement and hilar lymphadenopathy.

• Chronic sarcoidosis usually presents in the fifth decade with an insidious onset characterized by fatigue, dyspnoea, arthralgia and lupus pernio. Bone cysts are another feature.

How would you investigate such a patient?

• FBC and ESR: leukopenia, eosinophilia and raised ESR

• Chest radiography. Three stages of sarcoidosis are defined depending on radiographic findings, although these stages do not indicate chronology of disease:

• I: bilateral hilar adenopathy alone
• II: both hilar adenopathy and lung parenchymal involvement
• III: parenchymal involvement alone.

• Slit-lamp examination of the eyes

• Kviem’s test: the antigen is cultured in human spleen and thus, increasingly, it is recommended that this test should not be performed because of the risk of transmitting viral infections

• Mantoux test: skin anergy is present in 70%

• Serum ACE levels: raised in 40–80% with active disease, although this finding is neither sensitive nor specific enough to have diagnostic significance

• Lung function tests: usually restrictive changes with decreased lung volumes and diffusing capacity; occasionally airflow obstruction

• Serum calcium levels: hypercalcaemia in 10% of patients

• Gallium-67 scan

• Bronchoalveolar lavage: usually characterized by an increase in lymphocytes and a high CD4:CD8 T cell ratio

• Histological confirmation is by the presence of non-caseating granulomas with typical manifestations (fibrotic response develops over time) in biopsy of lung (transbronchial), lymph node, skin, liver, gums or minor salivary glands.

What is the prognosis and treatment of sarcoidosis?

• Acute sarcoidosis usually regresses spontaneously within 2 years and does not recur; it may not require treatment.

• Chronic sarcoidosis may require systemic cortiocosteroids in order to prevent serious disability and even death from respiratory failure.

What is the treatment of cutaneous sarcoid?

Monthly intralesional triamcinolone injections, topical and systemic steroids, hydroxychloroquine, allopurinol, thalidomide and tranilast (N Engl J Med 1997;336:1224–34).

What are the specific indications for systemic steroids in sarcoidosis?

• Progressive deterioration in lung function, particularly transfer factor and vital capacity. Serial evaluation should be performed every 2 months initially, gradually increasing the intervals between follow-up to about 4–5 months; patients should be followed for at least 2 years after steroids are stopped

• CNS involvement

• Hypercalcaemia

• Severe ocular disease

• Hepatitis

• Cutaneous lesions

• Constitutional symptoms

• Symptomatic pulmonary lesions.

What are the ocular manifestations of sarcoidosis?

• Anterior uveitis, seen in about 5% of all cases and either acute or chronic: mutton fat keratic precipitates, iris nodules

• Retinal vasculitis, neovascularization

• Vitreous opacities

• Choroidal granulomata

• Optic nerve granuloma.

What is Löfgren syndrome?

Erythema nodosum, hilar adenopathy and polyarthralgias in a patient with sarcoidosis.

What is the source of the raised serum angiotensin converting enzyme in sarcoidosis?

It is derived from the cell membranes of epithelioid cells in the sarcoid granuloma and its synthesis is controlled by epithelioid cells.

What is Blau syndrome?

It is a multisystem granulomatous disorder of the skin, eyes and joints that resembles childhood sarcoidosis; it was described by Edward Blau, a Wisconsin pediatrician (Lancet 1999;354:1035).

Is there any relationship between viral infections and sarcoid?

Variant human herpesvirus type 8 DNA sequences have been found in sarcoid tissue. Also mycobacteria-like 16S ribosomal RNA are more frequently found in sarcoid tissue but this does not indicate infection by a particular mycobacterial species (Lancet 1997;350:655–61).

What is the cardinal feature in the pathogenesis?

The cardinal feature of sarcoidosis is the presence of CD4 T cells that interact with antigen-presenting cells to initiate the formation and maintenance of granulomas.

What are the diagnostic features of cardiac sarcoidosis?

• Histologic diagnosis group: cardiac sarcoidosis is confirmed when histologic analysis of operative or endomyocardial biopsy specimens demonstrates epithelioid granuloma without caseating granuloma (Dis Mon 2009;55:675–92).

• Clinical diagnosis group: in patients with a histologic diagnosis of extracardiac sarcoidosis, cardiac sarcoidosis is suspected when item (a) and one or more of items (b) to (e) are present:

(a) ECG: complete right bundle branch block, left axis deviation, atrioventricular block, ventricular tachycardia, premature ventricular contraction or abnormal Q or ST–T change
(b) Abnormal wall motion, regional wall thinning or dilatation of the LV
(c) Myocardial scintigraphy: perfusion defect detected by thallium-201 or abnormal accumulation of gallium-67 or technetium-99m
(d) Abnormal intracardiac pressure: low cardiac output, abnormal wall motion or depressed ejection fraction of the LV
(e) Interstitial fibrosis or cellular infiltration over moderate grade even if the findings are non-specific.

Further reading

James DG, Jones Williams W. Sarcoidosis and other graulomatous disorders. Philadelphia, PA: Saunders; 1984.

Spalton DJ, Saunders MD. Fundus changes in histologically confirmed sarcoidosis. Br J Ophthalmol. 1981;65:348-358.

MA Kviem (b. 1892), a Norwegian pathologist.

C Mantoux (1877–1947), a French physician from Cannes, who showed that his intradermal test was more sensitive than the older Pirquet subcutaneous tests using tuberculin.

Norwegian dermatologist Caesar P.M. Boeck (1845–1917) from Olso, Norway, coined the term to describe skin nodules characterized by compact, sharply defined foci of ‘epithelioid cells with large pale nuclei and also a few giant cells’. Thinking this resembled sarcoma, he called the condition ‘multiple benign sarcoid of the skin’.

S Löfgren, a Swedish physician.

D Geraint James, contemporary Professor of Medicine, Royal Free Hospital, London; his chief interest is sarcoidosis. His wife, Dame Sheila Sherlock, is a renowned hepatologist.

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