Adult-Onset Still’s Disease Associated With Palisaded Neutrophilic Granulomatous Dermatitis (PNGD)

Introduction:

Palisaded neutrophilic granulomatous dermatitis (PNGD) is a neutrophilic skin disease well-reported as a cutaneous marker for several systemic associations, such as rheumatoid arthritis, lupus erythematosus, vasculitis, lymphoproliferative disorders, and medication responses. Tender erythematous to violaceous papules or plaques on the neck and extensor surfaces are a frequent feature of the clinical presentation of PNGD. In addition, Neutrophil infiltration, vasculitis, and collagen entrapment are histological hallmarks.

What Is Palisaded Neutrophilic Granulomatous Dermatitis?

A distinctive histopathologic pattern, PNGD is a subset of neutrophilic dermatoses and cutaneous granulomatous eruptions. Patients of any age may be afflicted, although PNGD most frequently manifests in the fifth decade of life (mean age 47.3 years) and is more prevalent in women. Several systemic diseases might cause PNGD. A range of histologic diagnosis of PNGD is characterized by aberrant collagen deposition, palisaded histiocytes, and tiny granulomas in the absence of leukocytoclastic vasculitis. It is linked to infections, hematologic problems, rheumatoid arthritis, connective tissue illnesses, and tumor necrosis factor inhibitors and is assumed to represent immune complex deposition.

PNGD frequently affects the hands and trunk. In addition, palmar involvement occurs; hence, in the proper clinical setting, PNGD should be considered with differential diagnoses such as Osler nodes, secondary syphilis, erythema multiforme, and lichen planus. Clinical outcomes range from spontaneous remission to persistence despite treatment. Dapsone, topical or systemic corticosteroids, and care of the underlying condition are all used in treatment.

How Is Palisaded Neutrophilic Granulomatous Dermatitis Presented?

With a wide variety of clinical and histological symptoms, PNGD is an unusual pattern of inflammatory dermatologic response. The typical clinical presentations are:

• Asymptomatic, erythematous to violaceous plaques with symmetrical distribution.

• Skin-colored or erythematous papules with crusting, perforation, or umbilication and Skin-colored or erythematous linear cords involving the lateral trunks are typical clinical presentations.

What Are the Conditions Linked to Palisaded Neutrophilic Granulomatous Dermatitis (PNGD)?

• Adult-onset Still’s disease.

• Sarcoidosis.

• Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome).

• Rheumatoid arthritis.

• Systemic vasculitis.

• Takayasu arteritis.

• Chronic myelomonocytic leukemia.

• Hodgkin’s or non-Hodgkin’s lymphoma.

• Allopurinol.

• Chronic uveitis.

• Systemic lupus erythematosus (SLE).

• Ulcerative colitis.

What Are Adult Still-Diseases?

An uncommon form of inflammatory arthritis called Adult Still's disease includes fever, rash, and joint discomfort. Adult Still's illness in some persons only manifests as one episode. However, the problem recurs or continues in other persons. Joints that are afflicted, especially the wrists, might be destroyed by this inflammation. The major risk factor for adult Still's disease is aging, with incidence peaking twice between the ages of 15 and 25 and 36 and 46. Both men and women are equally vulnerable.

What Is the Clinical Presentation of Adult Still Diseases?

The majority of adults with adult Still's disease exhibit some of the following symptoms:

• Fever: For a week or more, one can experience daily fevers of at least 102° F (38.9° C). The fever often reaches its height in the late afternoon or early evening. Two fever spikes per day are possible, with periods of normal temperature in between.

• Rash: With the fever, a salmon-pink rash might appear and disappear. The rash typically shows up on your arms, legs, or trunk.

• Painful Throat: One of the initial signs of adult Still's illness is this. The neck's lymph nodes might be sore and swollen.

• Swelling and Achy Joints: The joints may be painful, stiff, and inflamed, especially the knees and wrists. Shoulders, wrists, elbows, and ankles may also hurt. The pain in the joints often lasts for at least two weeks.

• Muscle Ache: The frequency of muscular discomfort often fluctuates with the fever, although it is occasionally severe enough to interfere with normal tasks.

This disorder's signs and symptoms might resemble those of other illnesses, such as lupus and the disease of lymphoma.

What Are the Complications of Adult Still Diseases?

1. Joint Destruction: The joints might get damaged by persistent inflammation. The knees and wrists are the joints that are affected the most frequently. The neck, foot, finger, and hip joints may also be impacted less commonly.

2. Heart Inflammation: Pericarditis, an inflammation of your heart's sac-like covering, as well as heart muscle inflammation, can both result from adult Still's disease (myocarditis).

3. Extra Fluid Around the Lungs: It may be difficult to breathe deeply if fluid builds up around the lungs due to inflammation.

4. Macrophage Activation Syndrome: Low blood cell counts, extremely high triglyceride levels, and altered liver function can all result from this uncommon but possibly deadly adult Still's disease condition.

How Is Adult-Onset Still’s Disease (AOSD) Diagnosed?

• No one test can accurately diagnose AOSD. As a result, testing may be performed to rule out other conditions, particularly infections and cancer. After considering a patient's medical history and symptoms, including arthritis that lasts at least six weeks, doctors can make a diagnosis.

• Blood testing can rule out other ailments and disorders and may even pick up variations in blood-cell levels consistent with AOSD. As opposed to other disorders of a similar nature, AOSD may have greater ferritin levels.

• X-rays can detect inflammation-related damage to muscles and joints. Unfortunately, the rarity of the ailment makes it susceptible to missed or incorrect diagnoses.

What Is the Treatment Plan For Adult-Onset Still’s Disease?

To treat adult Still's illness, doctors employ a range of medications. The kind of medication taken will depend on how severe your symptoms are and if it has any adverse effects.

• Non-Steroidal Anti-Inflammatory (NSAIDs): Over-the-counter NSAIDs like ibuprofen or naproxen sodium may be helpful for minor joint pain and inflammation. By prescription, stronger NSAIDs are available. As NSAIDs might harm the liver, one might require routine blood tests to monitor liver function.

• Steroids: Prednisone is typically needed to treat adult Still's disease. These potent medications reduce inflammation, but they also raise the chance of developing osteoporosis and may limit the body's ability to fight off infections.

• Methotrexate: Prednisone is often used with methotrexate, which makes it possible to lower the prednisone dose.

• Modifiers of Biologic Responses: The long-term benefits of medications like infliximab, adalimumab, and etanercept have yet to be determined. The doctor could advise trying anakinra, tocilizumab, or rituximab if prior drugs haven't been successful.

Conclusion

After the initial occurrence and treatment, AOSD never returns for around one-third of those with the disorder. Relapses will affect another third of people. However, they often don't worsen over time. Another third will develop a chronic illness that lasts for years. Immunosuppressant medications may be required if one has persistent AOSD to safeguard important organs