Granulomatous Facial Dermatitis - Symptoms, Diagnosis and Treatment

Introduction:

Granulomatous facial dermatitis is a skin condition characterized by eruptions on the face. It generally occurs in Afro-Caribbean children at pre-pubertal age. It is usually resolved by itself without the need for treatment. They appear as yellow-brown patches. It is considered to be different from perioral dermatitis.

What Are the Clinical Features of Granulomatous Facial Dermatitis?

• The patches are limited to areas around the mouth, nose, and eyes.

• The condition is self-limiting.

• There is usually no systemic involvement.

• The lesions are asymptomatic.

• The papules are dome-shaped.

• It is yellow-brown or red in color.

• Location at perioral, periocular or perinasal.

Granulomatous periorificial dermatitis is considered to be different from perioral dermatitis. Perioral dermatitis has red papules, pustules, vesicles, and redness around the mouth, nose, and eyes. However, in granulomatous periorificial dermatitis, pustules (pus-filled blisters) are absent. The lesions are yellow-brown patches. Redness and scaling are less prominent. The lesions can also be differentiated based on biopsy.

How Is Granulomatous Dermatitis Classified?

Granulomatous dermatitis can be classified into three depending on its histological appearance. Such as:

• Palisading neutrophilic granulomatous dermatitis.

• Interstitial granulomatous dermatitis.

• Interstitial granulomatous drug reaction.

1. Interstitial Granulomatous Dermatitis:

A skin disorder where a particular pattern of granulomatous inflammation is seen is known as interstitial granulomatous dermatitis. The characteristic sign is called the ‘rope sign’; that is, the linear red cords that can be palpated on the lateral side of the trunk of the body.

The clinical features of interstitial granulomatous dermatitis are,

• The clinical signs of this condition are variable.

• Shape: round, cord-like, or annular.

• Color: red or skin color.

• Appearance: Papules and plaques, that is, tiny bumps or patches.

• The lesions appear and disappear over days to months, and the shapes vary.

• They are usually asymptomatic but, in some cases, may itch or produce a burning sensation.

• They are generally found on the trunk but may also appear on the side of the limbs.

• Gender: usually affects women who are middle-aged.

• The affected individuals usually have associated autoimmune diseases.

The diagnosis of interstitial granulomatous dermatitis is,

• The diagnosis of this variant is based on skin biopsy.

2. Palisading Neutrophilic Granulomatous Dermatitis:

Palisading neutrophilic granulomatous dermatitis was first seen arising in rheumatoid arthritis patients on their elbows called Winkelmann granuloma. Different rashes may have the same pathological appearance. Lesions may ulcerate and are often tender.

Therefore they are diagnosed based on skin biopsy. The histological appearance includes:

• Histiocytic infiltration.

• Degeneration of collagen.

• Intense neutrophilic infiltration.

• Leukocytoclastic vasculitis.

• Based on the stage of the eruption, the histological appearance varies.

• Palisading granulomas are also described as miniature ‘Churg-Strauss granulomas.’ Here the eosinophils envelop the degenerated collagen resembling a flame. Therefore they are also called flame figures.

3. Interstitial Granulomatous Drug Reaction:

Interstitial granulomatous dermatitis that is triggered by the use of mediation is known as an interstitial drug reaction. It is considered a separate entity.

The features of interstitial granulomatous drug reaction are,

• Annular plaques (patches) are seen on the trunk, thighs, skin folds, and arms.

• The skin lesion resolves when the drug is withdrawn.

Like the other two histological variants, diagnosis is based on skin biopsy. The histopathological features include:

• Lichenoid changes.

• Vacuolation of basal epidermal cells.

• Neutrophil absence.

• Collagen necrosis and floating sign.

Medications Known to Cause Interstitial Granulomatous Drug Reaction:

• Beta-blockers.

• TNF-alpha inhibitors.

• Angiotensin-converting enzyme inhibitors.

• Calcium channel blockers.

• Statins.

• Lipid-lowering agents.

• Frusemide.

• Anticonvulsants.

• Antidepressants.

How to Differentiate Interstitial Granulomatous Dermatitis From Granuloma Annulare?

Clinically and through pathological comparison, both these diseases seem similar. Granuloma annulare also appears as bumps and patches (papules and plaques), but their location varies from that of interstitial granulomatous dermatitis. For example, granuloma annulare appears on the hands or feet on the back side, whereas interstitial granulomatous dermatitis appears on the trunk. Another difference is that granuloma annulare is associated with autoimmune diseases.

What Diseases are Associated with Granulomatous Dermatitis?

Certain conditions have been known to arise in association with granulomatous dermatitis, especially interstitial granulomatous dermatitis. However, it is less common or less known about palisading neutrophilic granulomatous dermatitis.

Autoimmune Disorders:

Granulomatous dermatitis has been seen in people with autoimmune disorders. This suggests that an immune response may be associated with the origin of the condition. The autoimmune disorders known to be associated with this condition are:

• Systemic lupus erythematosus (the body’s immune system attacks its own cells).

• Arthritis: Both rheumatic and non-rheumatoid arthritis. In patients with arthritis, fingers, elbows, wrists, and shoulders are involved. The joints become inflamed and painful (arthralgia). Joint involvement can happen before, after, or during the skin condition.

• Chrug-Strauss syndrome (blood vessels’ inflammation).

• Thyroiditis (inflammation of the thyroid gland).

• Vitiligo (loss of skin color in patches).

Malignancy:

In some cases, interstitial granulomatous dermatitis is seen as associated with malignancy. It has been reported that the lesions cleared after lung cancer was treated. It has also been associated with lymphoma, leukemia, breast cancer, neoplasm of the endometrium, and hypopharyngeal squamous cell carcinoma.

How to Treat Granulomatous Dermatitis?

The skin lesions generally have a period of flare-ups and remission. Treatments include:

• In cases associated with rheumatoid arthritis, Etanercept has been used and has shown complete clearance of skin lesions and improvement in arthritis.

• In cases associated with SLE, systemic steroids have been beneficial.

The cessation of all topical coticosteroids should be the first step in therapeutic management. Granumomatous facial dermatitis may respond well to Tetracycline, Metronidazole and Erythromycin treatment. Most importantly it is important to reassure parents and patients that the disease is benign, self limiting condition.

Conclusion:

Granulomatous facial dermatitis is a skin condition that affects children of pre-pubertal age. The skin lesions affect areas around the face, nose, and eyes. The lesions are self-limiting and resolve without treatment. There are other extra-facial variations or forms of the disease, which are sometimes associated with systemic disorders.