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PCDS home Sarcoid

About the PCDS

Dermatology dictionary
CREATED: 15TH SEPTEMBER 2015 | LAST UPDATED: 3RD MAY 2018
Dermatology: the basics

Diagnostic tables Introduction

Dermoscopy
Sarcoidosis is a rare multisystem granulomatous condition characterised histologically by the presence of
Investigations non-caseating epithelioid granulomas. The lung and lymph nodes, followed by the skin, eyes, liver, spleen,
salivary glands, the small bones of the hands and feet, and the heart are the most commonly affected sites.
A-Z of clinical guidance
A-Z list This chapter is set out as follows:
Simple desktop treatment guide Aetiology
Patients and carers History

Leg dressings and other bandaging

Clinical findings
techniques Images

Skin surgery and cryosurgery Investigations

Management
PCDS guidance and other
guidelines

Quality of Life Measures

Research - keep up to date Aetiology

GPwER & service development The cause is unknown

PCDS educational events

Diplomas and other educational

programmes
History
Resources - websites and books Sarcoidosis is more common in Afro-Caribbeans, those of Irish descent living in England, and Puerto Ricans
living in New York
Latest news and bulletins
It mainly affects people between 20-40 years of age, and is rare in young children
Patient information leaflets The risk is slightly higher in women than in men
Patient support groups

Fundraising - the BSF

Clinical findings
Ethical dermatology
Presentation
PCDS website poster
Although sarcoid can present in many ways, acute and chronic presentations have been described
Affiliated societies

Acknowledgements
Acute - characterised by erythema nodosum, hilar lymphadenopathy, and uveitis
Chronic - showing more in the way of infiltrative, multisystem involvement

Cutaneous features

Approximately 20-35% of patients with systemic sarcoidosis have skin lesions, but cutaneous sarcoidosis can
occur without systemic involvement. Cutaneous sarcoid is one of the great mimickers as it has a wide range of
morphological features.

Lupus pernio
​Large bluish-red-purple infiltrated nodules and plaques
The most commonly affected sites are a roughly symmetrical involvement of the nose, cheeks, and ears.
The hands, fingers and toes can also be affected
Nasal involvement is associated with oedema, crusting, or ulceration of the nasal vestibule, which can
affect breathing
Lesions can be chronic and systemic involvement is common

Maculopapular
Maculopapular sarcoidosis is the most common cutaneous finding, especially in black women
Usually, lesions are asymptomatic, red-brown macules and papules commonly involving the face,
especially the periorbital areas and the nasolabial folds, and/or the extensor surfaces
 Lesions usually resolve without scarring

Papules and nodules

​Lesions can be red-yellow or purple-brown
Papules - can be few or very many. Lesions arise in crops, mainly on the face and extensor aspects of
the limbs. They generally carry a more favourable prognosis
Nodules - are solitary or few. Lesions mainly affect the proximal limbs, trunk and face

Plaques
The limbs and buttocks are the most commonly affected sites
Lesions are red-purple-brown, diffuse, and can grow to many cm in diameter

Annular
​Annular lesions are most common on the face and neck, although other sites can be affected
Lesions have peripheral evolution and central clearing

Scar sarcoidosis
​Sarcoid can cause thickening of old scars, which become red-purple-brown
This may be the only cutaneous feature
Sarcoid has also been seen in tattoos

Erythema nodosum
Mainly affects young women in patients with sarcoidosis
Is often associated with hilar lymphadenopathy, uveitis, and usually a good prognosis, with most cases
resolving within two years

Sarcoid has many other cutaneous presentations including alopecia, hypopigmentation, and ichthyosiform
sarcoidosis. It has also been associated with vasculitis, and necrobiosis lipoidica

Systemic involvement

Approximately two-thirds of patients with cutaneous sarcoidosis have systemic involvement.

General
​Lethargy, arthralgia and weight loss

Respiratory
Cough and/or chest pain
Shortness of breath associated with lung fibrosis
Upper respiratory tract involvement produces hoarseness, nasal obstruction and recurrent or persistent
sinusitis

Ocular
​Most patients with ophthalmic sarcoidosis have evidence of systemic involvement at the time of
presentation
Anterior uveitis is the most common ocular manifestation of sarcoidosis
Other features include lacrimal gland involvement causing dry eyes, conjunctivitis, cataracts, glaucoma
and involvement of the orbital space associated with proptosis or ptosis

Bones and joints

​Lysis and cystic changes of the small bones of the hands and feet
Acute polyarthralgia
Chronic polyarthritis

Cardiac
​Heart block and sudden death may occur
The incidence of ventricular tachyarrhythmias requiring implantable cardioverter-defibrillator therapy is
estimated to be 15% per year in those patients with cardiac involvement

Renal
​Although the kidneys are infrequently involved in sarcoidosis, it can cause serious damage.
Sarcoidosis may either involve the kidneys directly or cause renal failure secondary to hypercalcaemia,
hypercalciuria and nephrocalcinosis

Other organ involvement

 ​Includes the nervous system (optic nerve involvement, cranial nerve palsy, peripheral neuropathy),
deranged LFT, diabetes insipidus, and myopathy

Images
Please click on images to enlarge or download. The PCDS would like to thank Dermatoweb, DermQuest (Galderma),
and others who have contributed images. All named individuals and organisations maintain copyright for the relevant
images. This website is non-profit and holds the images for educational purposes only. Any image downloaded must
only be used for teaching purposes and not for commercial use. Notice and credit must be given to the PCDS or other
named contributor. Please follow this link if you have any high-quality images that you can contribute to the website.

Figure: 1

Lupus pernio

Figure: 2

Lupus pernio

Copied with kind permission from Dermatoweb

Figure: 3

Sarcoid

Figure: 4

Sarcoid

Figure: 5
Sarcoid

Figure: 6

Sarcoid - in a scar

Same patient as above

Figure: 7

Sarcoid

As above

Figure: 8

Sarcoid

Figure: 9

Sarcoid

Figure: 10

Sarcoid
The red-yellow colour gives this a necrobiotic appearance

Figure: 11

Sarcoid

Figure: 12

Papular sarcoid

Copied with kind permission from Dermatoweb

Figure: 13

Sarcoid - papules and plaque

Figure: 14

Samer patient as above - plaque sarcoid

Figure: 15

Plaque sarcoid
Figure: 16

Plaque sarcoid

Same patient as above

Figure: 17

Plaque sarcoid

Figure: 18

Sarcoid - older lesions

Figure: 19

Sarcoid

Same patient as above

Figure: 20

Annular sarcoid

Copied with kind permission from Dermatoweb

Figure: 21

Annular sarcoid

Figure: 22

Sarcoid in a tattoo

Figure: 23

Erythema nodosum
 Figure: 24

Hilar lymphadenopathy

Investigations
Bloods
FBC can show a mild anaemia, lymphopenia and neutropenia
U+E and calcium levels checking for renal impairment and hypercalcaemia
LFT may be slightly elevated
ACE (angiotensin-converting enzyme) levels are raised in 60% of cases and may be useful in disease
monitoring

CXR (and possibly lung function tests)

ECG to look for heart block

Histopathology reveals aggregates of epithelioid cells with pale-staining nuclei, which form the characteristic
discrete non-caseating granulomas. Multinucleate giant cells are usually, but not always, present

Management
Patients with suspected sarcoidosis should be referred to the appropriate specialist

Cutaneous lesions can be very recalcitrant to treatment. Topical treatments sometimes used include:
Potent or super-potent topical steroid creams/ointments
Intralesional steroids
Tacrolimus 0.1% ointment

Systemic treatments
May be required for systemic disease, and in turn may improve skin symptoms
Are not often used for patients with cutaneous involvement alone, unless the lesions are very
problematic, eg involving the face or widespread, and not responding to topical treatments
Include oral corticosteroids, steroid-sparing agents such as methotrexate and mycophenolate mofetil, and
the biologics. There have been case reports of patients responding to tetracyclines, and
hydroxychloroquine, but not mepacrine

Prognosis
​Cutaneous sarcoidosis often runs a prolonged course, with 10-15% of patients having active disease
lasting many years
Papules and nodules tend to resolve over months or years, whilst plaques may be more persistent
Lupus pernio is often present in patients with chronic fibrotic sarcoidosis and is associated with
involvement of the upper respiratory tract, advanced lung fibrosis, bone cysts and eye disease
20-30% of patients have some degree of permanent lung damage