Sarcoidosis is an inflammatory condition that causes small lumps to form in your lungs, lymph nodes, eyes, skin, and other body parts. It is a rare disease that mostly affects black women and can be life-threatening if it affects the heart or lungs.¹

Skin is another part of the body affected by sarcoidosis. Sarcoidosis can cause skin problems such as rashes, bumps, and disfiguring lesions on your skin.²

What is sarcoidosis?

Sarcoidosis is characterized by the formation of lumps of inflammatory cells called granulomas in the body. Lungs and lymph nodes in the chest are most commonly affected by sarcoidosis. Skin, eyes and other parts of the body may also be affected.

Sarcoidosis is a rare disease with less than 200,000 cases occurring at a time in the US. The exact cause of sarcoidosis is unknown. However, sarcoidosis may occur as a reaction of the body's immune system to certain unknown substances.

What are granulomas?

Granulomas are small lumps or nodules formed by a collection of immune cells that infiltrate the lungs, lymph nodes, skin, and ears.²

What causes sarcoidosis?

The exact cause of sarcoidosis is not known. Genetics and environmental factors play important roles in causing sarcoidosis. There is immune system involvement associated with sarcoidosis. Antigens or foreign bodies such as viruses and bacteria can trigger the immune system causing the disease.²

Risk factors for sarcoidosis

Risk factors include:

• African or Scandinavian descent 
• Women aged 30 to 50 years
• People working in dusty environments or using insecticides
• Genetics
• Conditions like Lymphoma
• Medications for the treatment of cancer and HIV¹

Signs and symptoms

Most people affected by sarcoidosis present with no symptoms. Feelings of tiredness, discomfort, and unexplained weight loss are experienced by others.³

Lofgren's syndrome

The classic symptoms of sarcoidosis are together referred to as Lofgren's syndrome. These symptoms usually disappear in 2 years

These include:

• Erythema nodosum
• Rashes or bumps on the head, neck, or legs
• Swollen lymph nodes in chest, armpits, chin, and neck
• Joint stiffness, swelling, and pain
• Pain or redness of eyes and blurred vision
• Fever

Respiratory symptoms 

If sarcoidosis affects your lungs, the following symptoms may be present:

• Shortness of breath
• Dry cough
• Chest pain
• Wheezing

Cardiac symptoms

• Shortness of breath
• Chest pain
• Arrhythmias - Irregular heartbeat
• Palpitations - Rapid heartbeat
• Edema - Swelling due to accumulation of fluid
• Syncope - Fainting

Ocular symptoms

• Eye pain
• Blurred vision
• Itching and redness
• Dry eyes
• Sensitivity to light

Cutaneous symptoms

• Red or purple bumps and rashes on the skin and ankle
• Darker or lighter areas on the skin
• Disfiguring lesions occurring on cheeks, ears, and nose
• Nodules around previous scars or tattoos²

Sarcoidosis and skin

Symptoms of sarcoidosis may appear anywhere on the skin. The most common sites are:

• Face and neck
• Near tattoos, scars and piercings
• Legs and arms
• Upper body⁴

Skin manifestations of sarcoidosis 

Sarcoidosis may manifest as bumps, rashes, patches, discolouration or lumps.

Bumps

One of the common skin manifestations of sarcoidosis is the appearance of small painless bumps on the face and neck regions. These bumps are reddish brown or violet in color and are known as papules.

Patches

Another skin manifestation of sarcoidosis is the presence of raised patches or plaques on the face, scalp, arms, legs, and back. Patches on the scalp can lead to hair loss.

Discolouration

These bumps and patches are sometimes firm and can cause red, pink, or brown discolouration of the skin.

Nodules

Nodules are small painless or slightly tender lumps that appear on arms. They are deep-seated on the skin.

Rashes

Rashes appear on shins and may be slightly tender. These rashes are called erythema nodosum. They resolve themselves in a few weeks to months.

Changes in tattoos, piercings, or scars

Sarcoidosis occurs on scars such as small bumps, lumps, or discolourations. Granulomas infiltrate the scars, tattoos, and tissues with foreign materials.

Nail changes 

Sarcoidosis causes discolouration and deformation of nails and also leads to swelling of fingers and toes.⁴

Symptoms of cutaneous sarcoidosis 

You may or may not have symptoms along with the skin changes in sarcoidosis. The commonly present symptoms are:

• Itching
• Swelling
• Pain or tenderness
• Warm skin on affected regions⁴

Syndromes associated with sarcoidosis

Characteristic skin manifestations in sarcoidosis are:

1. Erythema nodosum

It is a hypersensitivity reaction occurring due to exposure to infectious agents. It is usually self-limiting and does not require treatment. It presents as nodules that are tender and erythematous and usually occur on extremities. It is accompanied by fever, malaise, and arthralgia.

2. Lofgren syndrome

It involves a triad of symptoms that usually resolve spontaneously in 6 to 8 weeks but may sometimes take about 2 years from onset to completely resolve. The symptoms of Lofgren’s syndrome are:

• Erythema nodosum
• Polyarthritis
• Hilar adenopathy

3. Lupus pernio

It is characterized by red or purple plaques and nodules mostly on the nose, cheeks, lips, and ears. It may cause severe disfigurement. This is mostly seen in black women with pulmonary sarcoidosis involving the lungs and respiratory tract.⁶ 

Diagnosis of skin manifestations of sarcoidosis

Physical examination

Skin lesions seen in sarcoidosis are classified as specific and nonspecific lesions.

1. Specific lesions

These manifest as granulomas. Examples of specific lesions are:

• Lupus pernio
• Nodular lesions
• Scar lesions
• Plaque lesions
• Maculopapular lesions
• Ulcerative lesions

2. Non-specific lesions 

These lesions do not reveal granulomas. The main non-specific lesion is erythema nodosum.

Skin tests

1. Kveim-Siltzbach Test

In this test, spleen or lymph node tissue from a sarcoidosis patient is injected intradermally to the suspected patient.

After 4 to 6 weeks, the injected area is biopsied and examined. If there is granuloma formation, the test result is positive. This is the recommended test but is not commonly available because of fear of transmitting infection or difficulty obtaining the tissue for injection. 

2. Tuberculin skin test

Tuberculin skin test is a test used to diagnose sarcoidosis and tuberculosis. 

A negative tuberculin skin test is a sensitive test for sarcoidosis. A positive tuberculin skin test indicates that a patient having sarcoidosis might also have tuberculosis. Therefore, a positive result must be followed by a thorough workup for tuberculosis.^{5 7}

3. Biopsy

Skin is biopsied and histopathologically examined to rule out any fungal or mycobacterial infections causing granuloma formation.

Treatment 

Treatment of sarcoidosis involves:

Medications

For patients with cardiac, pulmonary, ocular, or neurologic involvement of sarcoidosis, oral corticosteroids are indicated. 

Symptoms of Lofgren syndrome mostly resolve themselves and do not need immunosuppressive medication. NSAIDs and low doses of corticosteroids can be given for symptomatic relief. 

Mild cutaneous lesions can be treated with topical and intralesional corticosteroids like triamcinolone acetonide. 

In more aggressive cases, systemic corticosteroids, antimalarials such as chloroquine, or low-dose methotrexate are given.

In severe disfiguring or unruly cases of sarcoidosis, therapies using biological agents such as alpha therapy and anti-tumour necrosis factor therapy are used.⁶

Other agents used in the treatment are:

• Cyclosporine
• Thalidomide
• Chlorambucil
• Doxycycline
• Tetracycline 
• Minocycline
• Prednisone and hydroxychloroquine 

Radiation

For cutaneous lesions resistant to medications, radiation therapy has been used.

Surgical treatment

Disfiguring and recalcitrant lesions are treated by surgical resection. However, scarring and recurrence are some of the complications of surgery.

Laser surgery is also used for treating disfiguring lesions. Laser surgery has fewer complications when compared to normal surgery.⁶

FAQs

What is the common skin manifestation of sarcoidosis?

Erythema nodosum is the most common skin manifestation of sarcoidosis. It is associated with the formation of erythematous nodules on extremities which is accompanied by fever, malaise, and arthralgia. 

What is the skin test for sarcoidosis?

A kveim-Siltzbach skin test is used to diagnose sarcoidosis. It involves the injection of infected tissue from a sarcoidosis patient followed by a biopsy of the injected area. The biopsied tissue is examined for granuloma formation, which is characteristic of sarcoidosis.

What is the treatment for cutaneous sarcoidosis?

Skin lesions of sarcoidosis can be treated by medications that include corticosteroids, methotrexate and NSAIDs. Radiation therapy and surgery are also indicated for recalcitrant lesions.

Summary

Sarcoidosis is characterized by the formation of nodules and lumps in different parts of the body. Skin manifestations of sarcoidosis mainly involve the formation of lumps, patches and rashes on the skin called granulomas. Symptoms like itching, swelling, and pain may also be present. Kveim-Siltzbach skin test is used to diagnose sarcoidosis. Management of sarcoidosis usually involves the use of corticosteroids and methotrexate. Recalcitrant lesions are treated by surgery and radiation. 

Most cases of sarcoidosis resolve on their own. Chronic cases of sarcoidosis can be managed with proper monitoring of symptoms along with medications as per the recommendation of your healthcare provider.