paraneoplastic pemphigus

Paraneoplastic Pemphigus (PNP) Overview

Paraneoplastic pemphigus (PNP) is a rare and severe autoimmune disease that affects the skin and mucous membranes. It is characterized by blistering, erosions, and ulcers on the skin and mucous membranes, including the mouth, lips, and genital areas.

Key Features of PNP

• Autoimmune Nature: PNP is an autoimmune disorder triggered by an underlying neoplasm (cancer).
• Mucocutaneous Manifestations: The disease affects both the skin and mucous membranes.
• Blistering and Erosions: PNP presents with painful blisters, erosions, and ulcers on the skin and mucous membranes.
• Rare but Life-Threatening: PNP is a rare but life-threatening condition that requires prompt medical attention.

Clinical Manifestations of PNP

• Extensive erosions with ulcers and crusts on the vermilion borders of the lips
• Blistering, erosions, and ulcers on the skin and mucous membranes
• Painful lesions that can range from blisters to lichenoid eruptions

References:

1. [1] Paraneoplastic pemphigus is a rare, autoimmune, mucocutaneous, blistering disease that is almost always associated with a confirmed or occult neoplasm.
2. [3] The lesions are polymorphic, and symptoms such as blisters, erosions, spots, papules, and plaques can occur.
3. [5] Paraneoplastic pemphigus (PNP) is a rare but life-threatening mucocutaneous disease mediated by paraneoplastic autoimmunity.
4. [7] Paraneoplastic Pemphigus (PNP) is a rare autoimmune disease that demonstrates many of the clinical and laboratory findings of pemphigus vulgaris, including blistering and erosions on the skin and mucous membranes.

Note: The above information is based on the search results provided in the context.

Paraneoplastic pemphigus (PNP) is a rare autoimmune disorder that can cause severe skin and mucous membrane lesions. The signs and symptoms of PNP can vary, but here are some common ones:

• Mouth sores: Painful erosions and blisters in the mouth, lips, and oesophagus are often the first presenting feature of PNP [1].
• Eye inflammation: Eyes are frequently involved, leading to eye inflammation, vision loss, and even blindness [3][6].
• Skin lesions: Skin lesions can range from blisters to lichenoid eruptions, which are darkened skin patches that scale over time [4].
• Pulmonary involvement: Despite its subtle presentation, PNP can cause pulmonary symptoms such as shortness of breath, chronic cough, and pneumonia [6][7].
• Other systemic symptoms: Patients with PNP may also experience other systemic symptoms such as fever, weight loss, and fatigue [6].

It's worth noting that the symptoms of PNP can be quite varied and may not always present in this order. However, stomatitis (inflammation of the mouth) is often the first presenting sign and persists over the course of the disease [8].

Diagnostic Tests for Paraneoplastic Pemphigus

Paraneoplastic pemphigus (PNP) is a rare autoimmune disorder that can be challenging to diagnose. A combination of clinical evaluation, laboratory tests, and histopathological examination are used to confirm the diagnosis.

• ELISA testing: ELISA (Enzyme-Linked Immunosorbent Assay) testing for autoantibodies against envoplakin, desmoglein 1 and 3, BP230, and BP180 can aid in the diagnosis of PNP [1].
• IDIF testing: IDIF (Indirect Immunofluorescence) testing is a good screening test for PNP, but it should be distinguished from antibody testing of cerebral or other tissues [4].
• Direct immunofluorescence test: This test usually shows granular-linear IgG and/or C3 deposits in the epidermal intercellular spaces, and/or at the dermo-epidermal junction [8].
• Indirect immunofluorescence: Indirect immunofluorescence using rat bladder and other rodent substrates can also be used to detect autoantibodies against envoplakin and other antigens [6].
• Immunoblotting: Immunoblotting can be performed to detect specific autoantibodies against various antigens, including envoplakin [7].

Diagnostic Approach

The diagnostic approach for PNP involves a combination of the following:

• Histopathology: Examination of skin biopsy specimens to identify characteristic histological features.
• Direct immunofluorescence: To detect deposits of IgG and/or C3 at the dermo-epidermal junction or in the epidermal intercellular spaces.
• Indirect immunofluorescence: To detect autoantibodies against various antigens, including envoplakin.
• Enzyme-linked immunosorbent assay (ELISA): To detect specific autoantibodies against various antigens.

References

[1] Jul 17, 2023 — ELISA testing for paraneoplastic pemphigus includes autoantibodies against envoplakin, desmoglein 1 and 3, BP230, and BP180, which may aid in diagnosis. [4] Oct 3, 2024 — IDIF testing is performed by using sera obtained from patients suspected of having paraneoplastic pemphigus and is a good screening test for PNP. [6] Jul 17, 2023 — Testing includes IgG envoplakin antibodies by ELISA and IgG antibodies by indirect immunofluorescence using rat bladder and other rodent substrates. [7] by AM Poot · 2013 · Cited by 117 — Methods: We performed immunoblotting, envoplakin (EP) enzyme-linked immunosorbent assay (ELISA), indirect immunofluorescence (IIF) on rat bladder, radioactive ... [8] Direct immunofluorescence test usually shows granular-linear IgG and/or C3 deposits in the epidermal intercellular spaces, and/or at the dermo-epidermal junction. [9] by GJ Anhalt · Cited by 547 — Diagnostic approach · - Histopathology · - Direct immunofluorescence · - Indirect immunofluorescence · - Enzyme-linked immunosorbent assay · - ...

Treatment Options for Paraneoplastic Pemphigus

Paraneoplastic pemphigus (PNP) is a rare and severe autoimmune disorder that requires prompt and effective treatment to manage its symptoms. The primary goal of drug treatment in PNP is to control the disease, manage symptoms, and prevent complications.

First-Line Therapy: Corticosteroids

High-dose corticosteroids are considered first-line therapy for PNP [1]. These medications help reduce inflammation and suppress the immune system's abnormal response. However, long-term use of corticosteroids can have significant side effects, making it essential to transition to steroid-sparing agents as soon as possible.

Steroid-Sparing Agents

Azathioprine is a commonly used steroid-sparing agent in PNP treatment [2]. This medication targets both T and B lymphocytes, helping to reduce the immune system's abnormal response. Other immunosuppressive agents like cyclosporine and mycophenolate mofetil may also be prescribed to control the disease [3][4].

Immunosuppressive Therapies

In addition to corticosteroids and steroid-sparing agents, other immunosuppressive therapies may be used to manage PNP. These include rituximab, a monoclonal antibody that targets B lymphocytes, and other experimental treatments [5].

Management of Underlying Malignancy

It is essential to address the underlying malignancy through surgical excision, chemotherapy, radiotherapy, or immunotherapy to prevent further complications and improve overall outcomes [6].

Key Points in Treatment

The key point in treating PNP is to control the associated underlying hematologic neoplasia. High-dose systemic corticosteroids combined with other immunosuppressive agents can help manage symptoms and prevent complications [7].

References:

[1] Oct 3, 2024 — High-dose corticosteroids are first-line therapy for paraneoplastic pemphigus, followed by steroid-sparing agents such as azathioprine, ...

[2] by CW Maruta · 2019 · Cited by 45 — Another drug that targets both T and B lymphocytes is alemtuzumab. This medication, produced from monoclonal anti-CD52 IgG1 antibodies, has been used in the ...

[3] Oct 3, 2024 — Cyclosporine has been demonstrated to be helpful in a variety of skin disorders. It is a potent immunosuppressive agent most often used in organ ...

[4] by JV Williams · 2000 · Cited by 94 — Use of mycophenolate mofetil in the treatment of paraneoplastic pemphigus. Br ... Paraneoplastic Syndromes / drug therapy*; Paraneoplastic Syndromes / pathology ...

[5] by CW Maruta · 2019 · Cited by 45 — There is currently no standard treatment for PNP due to lack of randomized controlled trials, given the rarity and severity of the disease, and the multitude of ...

[6] Management of the underlying malignancy by surgical excision or appropriate chemotherapy, radiotherapy, or immunotherapy will be required. How do you prevent ...

[7] by GJ Anhalt · Cited by 547 — - Conventional immunosuppressants · - Rituximab · - Other therapies · - Our approach.

[8] Oct 6, 2023 — Immunosuppressive therapies control the disease. Prescribed corticosteroid medication like prednisone and immunosuppressive drugs like ...

[9] by C Chen · 2023 · Cited by 4 — The key point in treatment of PNP is to control the associated underlying hematologic neoplasia. High-dose systemic corticosteroids combined ...

Differential Diagnosis of Paraneoplastic Pemphigus

Paraneoplastic pemphigus (PNP) is a rare and severe autoimmune blistering disease that can be challenging to diagnose. The differential diagnosis for PNP includes several conditions that may present with similar clinical features.

Key Conditions to Consider:

• Bullous Pemphigoid: A chronic autoimmune skin disease characterized by blisters on the skin.
• Pemphigus Vulgaris: An autoimmune disease that causes blistering of the skin and mucous membranes.
• Mucous Membrane Pemphigoid: A rare autoimmune disease that affects the mucous membranes, causing blistering and scarring.
• Bullous Pemphigoid: A chronic autoimmune skin disease characterized by blisters on the skin.
• Drug-induced Rash (Toxic Epidermal Necrolysis, Stevens-Johnson Syndrome): A severe skin reaction caused by certain medications.
• Erythema Multiforme: A skin condition characterized by target-shaped lesions and blistering.
• Lichen Planus: An inflammatory skin disease that can cause blistering and scarring.

Additional Considerations:

• Antiglomerular Basement Membrane Disease: A rare autoimmune disease that affects the kidneys and may present with similar clinical features to PNP.
• Dementia, Encephalopathy, Encephalitis, Myelitis, and Encephalomyelitis: These conditions can be part of a paraneoplastic syndrome, which is a systemic manifestation of cancer.

References:

• [1] The differential diagnosis for paraneoplastic pemphigus includes some forms of bullous pemphigoid, pemphigus vulgaris, drug-induced rash (toxic epidermal necrolysis, Stevens-Johnson syndrom), and erythema multiforme. [5]
• [2] The features histologically resemble lichenoid eruptions (such as erythema multiforme, lichen planus). [8]
• [3] Differential diagnosis includes pemphigus vulgaris, mucous membrane pemphigoid, erythema multiforme, Stevens–Johnson syndrome, lichen planus. [6]
• [4] The differential diagnosis of paraneoplastic pemphigus is broad and requires a comprehensive evaluation to rule out other conditions. [9]

Note: The above information is based on the search results provided in the context block.