Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a rare skin condition that is characterized by the formation of granulomas, which are small, organized clusters of immune cells that form in response to a variety of stimuli. These granulomas are typically composed of neutrophils, which are a type of white blood cell that is involved in the body’s immune response.
PNGD can occur in a wide range of individuals, but it is most commonly seen in middle-aged women. The condition is often associated with underlying medical conditions, such as rheumatoid arthritis, systemic lupus erythematosus, and other autoimmune disorders. However, in some cases, the cause of PNGD is unknown.
The term “palisaded” refers to the way in which the neutrophils are arranged in the granulomas. In PNGD, the neutrophils are arranged in a palisade formation, which means that they are aligned in a parallel fashion along the edge of the granuloma. This arrangement is thought to be due to the release of cytokines, which are signaling molecules that are produced by immune cells in response to infection or injury.
There are several different types of PNGD, which are defined based on the underlying cause of the condition. These types include:
- Idiopathic PNGD: This type of PNGD occurs in individuals without an underlying medical condition. The cause of this type of PNGD is unknown, but it is thought to be related to an abnormal immune response.
- Rheumatoid arthritis-associated PNGD: This type of PNGD is associated with rheumatoid arthritis, which is an autoimmune disorder that causes inflammation of the joints. In individuals with rheumatoid arthritis-associated PNGD, the granulomas are often found in the skin around the joints.
- Lupus erythematosus-associated PNGD: This type of PNGD is associated with systemic lupus erythematosus, which is another autoimmune disorder that can affect many different parts of the body. In individuals with lupus erythematosus-associated PNGD, the granulomas are often found on the face or scalp.
- Paraneoplastic PNGD: This type of PNGD is associated with an underlying cancer, such as lymphoma or leukemia. The granulomas are often found on the skin around the cancerous tumor.
- Drug-induced PNGD: This type of PNGD is associated with the use of certain medications, such as tumor necrosis factor (TNF) inhibitors or interferon. The granulomas are often found on the skin around the injection site.
Causes
The cause of this condition is not yet fully understood, but it is believed to be multifactorial, with a number of different factors potentially contributing to the development of the condition. In this article, we will discuss the potential causes of PNGD and explain each one in detail.
- Autoimmune disease: Autoimmune diseases occur when the immune system attacks the body’s own tissues, mistaking them for foreign invaders. There is some evidence to suggest that PNGD may be associated with autoimmune diseases such as lupus, rheumatoid arthritis, and Sjogren’s syndrome.
- Infections: Certain bacterial, fungal, and viral infections may be associated with PNGD. These include streptococcal infections, tuberculosis, and hepatitis C virus infection.
- Medications: Certain medications, such as tumor necrosis factor (TNF) inhibitors and antibiotics, have been reported to cause PNGD. TNF inhibitors are used to treat autoimmune diseases such as rheumatoid arthritis, psoriasis, and Crohn’s disease.
- Cancer: There have been reports of PNGD occurring in patients with various types of cancer, including lymphoma and leukemia.
- Connective tissue diseases: PNGD has been reported in patients with connective tissue diseases such as scleroderma, systemic lupus erythematosus (SLE), and dermatomyositis.
- Genetic factors: There is some evidence to suggest that PNGD may have a genetic component, although no specific genes have been identified as yet.
- Hormonal changes: Hormonal changes, such as those that occur during pregnancy, may trigger PNGD.
- Sun exposure: There have been reports of PNGD occurring in areas of the skin that are exposed to the sun.
- Trauma: PNGD has been reported to occur at sites of previous trauma or surgery.
- Insect bites: There have been reports of PNGD occurring at sites of insect bites.
- Vaccinations: There have been reports of PNGD occurring following vaccination with certain vaccines, such as the hepatitis B vaccine.
- Environmental factors: Exposure to certain environmental factors, such as chemicals or pollutants, may be associated with the development of PNGD.
- Immune system dysfunction: Dysregulation of the immune system may play a role in the development of PNGD.
- Vitamin deficiencies: Deficiencies in certain vitamins, such as vitamin D, may contribute to the development of PNGD.
- Allergic reactions: Allergic reactions to certain substances, such as medications or chemicals, may trigger PNGD.
- Parasitic infections: Certain parasitic infections, such as leishmaniasis, may be associated with PNGD.
- Food allergies: Food allergies may be associated with the development of PNGD in some cases.
- Stress: Psychological stress may exacerbate PNGD symptoms in some individuals.
- Smoking: There have been reports of PNGD occurring more frequently in smokers than in non-smokers.
- Alcohol consumption: There have been reports of PNGD occurring more frequently in individuals who consume alcohol than in those who do not.
Symptoms
The symptoms of PNGD can vary from person to person, and some people may not experience any symptoms at all. The most common symptoms of PNGD are skin lesions, joint pain, and fever. Here are the most common symptoms of PNGD:
- Skin lesions: The most common symptom of PNGD is skin lesions. These lesions are usually red, raised, and may be tender to touch. They can be found on the face, neck, arms, legs, or trunk.
- Joint pain: Joint pain is another common symptom of PNGD. The pain may be mild to severe and can affect any joint in the body. The joint pain is usually symmetrical, meaning it affects both sides of the body.
- Fever: Fever is a common symptom of PNGD. It may be low-grade or high-grade and may be accompanied by other symptoms such as chills and sweating.
- Fatigue: Fatigue is a common symptom of PNGD. It may be mild to severe and can affect a person’s ability to perform daily activities.
- Weight loss: Weight loss is a common symptom of PNGD. It may be mild to severe and can be a sign of underlying systemic disease.
- Arthritis: Arthritis is a common symptom of PNGD. It may be inflammatory or non-inflammatory and can affect any joint in the body.
- Myalgia: Myalgia is a common symptom of PNGD. It is characterized by muscle pain and can affect any muscle in the body.
- Erythema nodosum: Erythema nodosum is a type of skin lesion that is a common symptom of PNGD. It is characterized by red, painful nodules on the legs.
- Palpable purpura: Palpable purpura is a type of skin lesion that is a common symptom of PNGD. It is characterized by red or purple spots on the skin that can be felt.
- Uveitis: Uveitis is a common symptom of PNGD. It is inflammation of the uvea, the middle layer of the eye.
- Vasculitis: Vasculitis is a common symptom of PNGD. It is inflammation of the blood vessels and can affect any organ in the body.
- Lymphadenopathy: Lymphadenopathy is a common symptom of PNGD. It is swelling of the lymph nodes and can be a sign of underlying systemic disease.
- Splenomegaly: Splenomegaly is a common symptom of PNGD. It is enlargement of the spleen and can be a sign of underlying systemic disease.
- Hepatomegaly: Hepatomegaly is a common symptom of PNGD. It is enlargement of the liver and can be a sign of underlying systemic disease.
- Abdominal pain: Abdominal pain is a common symptom of PNGD. It may be mild to severe and can be a sign of underlying systemic disease.
Diagnosis
The diagnosis of PNGD requires a combination of clinical, histological, and laboratory findings. Here are 20 diagnostic tests and procedures that can aid in the diagnosis of PNGD:
- Skin biopsy: A skin biopsy is the most important diagnostic test for PNGD. The histological features of PNGD include palisading granulomas, neutrophilic infiltrates, and eosinophilic collagenolytic degeneration.
- Immunofluorescence: Direct immunofluorescence studies can help to rule out other autoimmune blistering diseases such as bullous pemphigoid or pemphigus vulgaris.
- Blood tests: Blood tests are not specific for PNGD but can be useful to rule out other autoimmune or infectious causes. These include a complete blood count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), antinuclear antibody (ANA), rheumatoid factor (RF), and serological testing for infectious agents such as hepatitis B, hepatitis C, and HIV.
- Skin culture: Skin cultures can be helpful in ruling out bacterial, fungal, or viral infections.
- Chest X-ray: A chest X-ray may be useful in ruling out underlying lung disease or malignancy.
- CT scan: A CT scan may be needed to evaluate for underlying malignancy or other systemic diseases.
- Ultrasound: An ultrasound may be helpful in evaluating for lymphadenopathy or other associated abnormalities.
- PET scan: A PET scan may be helpful in evaluating for underlying malignancy.
- Histopathology: Histopathological examination of the skin biopsy is essential for the diagnosis of PNGD. The hallmark of PNGD is the presence of palisading granulomas with surrounding neutrophilic infiltrates.
- Gram stain: A Gram stain can be used to rule out bacterial infections.
- Fungal culture: Fungal cultures can be used to rule out fungal infections.
- Acid-fast bacilli stain: An acid-fast bacilli stain can be used to rule out mycobacterial infections.
- Polymerase chain reaction (PCR): PCR can be used to detect DNA of infectious agents such as Mycobacterium tuberculosis, Borrelia burgdorferi, and other microorganisms.
- Skin prick test: A skin prick test can be used to rule out hypersensitivity reactions to drugs or other allergens.
- Skin patch test: A skin patch test can be used to rule out allergic contact dermatitis.
- Biopsy of lymph nodes: A biopsy of lymph nodes may be necessary to evaluate for lymphadenopathy.
- Serological testing: Serological testing can be used to rule out infectious diseases such as Lyme disease, syphilis, and tuberculosis.
- Immunohistochemistry: Immunohistochemical staining can be used to detect specific antigens and can be helpful in ruling out malignancy.
- Flow cytometry: Flow cytometry can be used to evaluate lymphoproliferative disorders.
- Electromyography: Electromyography can be used to
Treatment
There is no specific treatment for PNGD, but there are several treatment options that can be used to manage its symptoms. In this article, we will discuss the treatments for PNGD in detail.
- Topical corticosteroids: Topical corticosteroids are the first-line treatment for PNGD. They work by reducing inflammation in the skin and can be applied directly to the affected area. They are available in various strengths and forms, including creams, ointments, and gels.
- Oral corticosteroids: Oral corticosteroids may be used in severe cases of PNGD or when topical corticosteroids are not effective. They work by reducing inflammation throughout the body and are available in various doses and formulations.
- Nonsteroidal anti-inflammatory drugs (NSAIDs): NSAIDs are used to reduce pain and inflammation in PNGD. They are available over-the-counter and in prescription strength and can be taken orally or applied topically.
- Immunosuppressive drugs: Immunosuppressive drugs, such as methotrexate, azathioprine, or mycophenolate mofetil, may be used to treat PNGD. These drugs work by suppressing the immune system, which can help reduce inflammation.
- Biologic agents: Biologic agents, such as adalimumab, infliximab, or rituximab, may be used to treat PNGD. These drugs work by targeting specific components of the immune system, which can help reduce inflammation.
- Colchicine: Colchicine is a medication that is commonly used to treat gout. However, it has also been reported to be effective in treating PNGD. Colchicine works by reducing the activity of inflammatory cells.
- Dapsone: Dapsone is an antibiotic that is commonly used to treat leprosy and dermatitis herpetiformis. However, it has also been reported to be effective in treating PNGD. Dapsone works by reducing the activity of neutrophils.
- Hydroxychloroquine: Hydroxychloroquine is an antimalarial drug that is commonly used to treat rheumatoid arthritis and systemic lupus erythematosus. However, it has also been reported to be effective in treating PNGD. Hydroxychloroquine works by reducing inflammation and suppressing the immune system.
- Cyclosporine: Cyclosporine is an immunosuppressive drug that is commonly used to prevent organ rejection in transplant patients. However, it has also been reported to be effective in treating PNGD. Cyclosporine works by suppressing the immune system.
- Thalidomide: Thalidomide is a medication that was originally used to treat morning sickness in pregnant women. However, it was withdrawn from the market due to its teratogenic effects. Thalidomide has also been reported to be effective in treating PNGD. Thalidomide works by reducing inflammation.
- Interferon-alpha: Interferon-alpha is a medication that is commonly used to treat viral infections and certain types of cancer. However, it has also been reported to be effective in treating PNGD. Interferon-alpha works by modulating the immune system.
- Colchicine: Colchicine is an anti-inflammatory medication that is used to treat gout and other inflammatory conditions. It may be effective in treating PNGD by reducing inflammation and the formation of granulomas.
- Dapsone: Dapsone is an antibacterial medication that is also effective in treating inflammatory skin conditions, including PNGD. It works by reducing inflammation and inhibiting the formation of granulomas.
- Methotrexate: Methotrexate is a chemotherapy medication that is also used to treat inflammatory skin conditions. It works by suppressing the immune system and reducing inflammation.
- Azathioprine: Azathioprine is an immunosuppressant medication that is used to treat a variety of autoimmune and inflammatory conditions, including PNGD. It works by suppressing the immune system and reducing inflammation.
- Cyclosporine: Cyclosporine is an immunosuppressant medication that is used to treat a variety of autoimmune and inflammatory conditions, including PNGD. It works by suppressing the immune system and reducing inflammation.
- Mycophenolate mofetil: Mycophenolate mofetil is an immunosuppressant medication that is used to prevent organ rejection in transplant patients. It may also be effective in treating inflammatory skin conditions, including PNGD.
- Tacrolimus: Tacrolimus is an immunosuppressant medication that is used to treat a variety of inflammatory skin conditions, including eczema and psoriasis. It works by suppressing the immune system and reducing inflammation.
- Interferon alpha: Interferon alpha is a type of protein that is naturally produced by the body in response to infection. It may be effective in treating PNGD by reducing inflammation and inhibiting the formation of granulomas.
- Infliximab: Infliximab is a biological medication that is used to treat a variety of inflammatory conditions, including PNGD. It works by blocking a protein called tumor necrosis factor-alpha (TNF-alpha) that is involved in inflammation.
- Adalimumab: Adalimumab is a biological medication that is used to treat a variety of inflammatory conditions, including PNGD. It works by blocking TNF-alpha and reducing inflammation.
- Etanercept: Etanercept is a biological medication that is used to treat a variety of inflammatory conditions, including PNGD.