It is hypothesized that antigens associated with the tumor trigger an immune response resulting in blistering of the skin and mucous membranes.

Current treatment focuses on general wound healing and administering corticosteroids, which has not demonstrated a high success rate.

Recent research developments aim to treat the underlying tumor in order to alleviate the symptoms of PNP.

[1] The underlying tumor causes circulating and tissue-bound antibodies to direct themselves against antigens in the plakin family, which are involved in the intracellular attachment structures in various levels of the skin/respiratory tract/membranes (keeping skin tissue together throughout the body).

[citation needed] The precise mechanism for how tumors are able to induce autoantibodies toward the plakin proteins is unknown.

Initially, samples are obtained via skin biopsy for routine microscopy and direct immunofluorescence (DIF) testing.

[1][4][7] PNP is most commonly mistaken for pemphigus vulgaris, due to the extreme similarities of the lesions that develop.

[8] Initial treatment involves addressing any existing infections that may have occurred due to the broken state of the skin.

Existing wounds are treated with warm compresses, non-adherent (non-stick) dressing, and topical antibiotic ointment.

However, a high level of caution is advised in patients with a confirmed malignancy, where immunosuppression is vital and dictates treatment options.

If the initial therapy fails to control the symptoms of PNP, and the condition of the patient deteriorates, a more aggressive approach may be necessary.

The impaired skin barrier function commonly leads to localized infection, which sepsis and death may follow.

It manifests as dyspnea and progresses to bronchiolitis obliterans (non-reversible obstructive lung disease) via an unknown mechanism.

[15] Further use of ELISA testing on these antibodies confirmed the presence of anti-envoplakin and anti-periplakin autoantibodies in patients with PNP.

This piece labeled PNP as a "multiorgan disease characterized by antibodies against plakins, desmogleins and the α2-macroglobulin-like-1 (A2ML1) protein, in association with an underlying neoplasm".

[2] A study concluded in 2009, summarized in 2010, surrounded the surgical removal of the associated tumor as a means to treat PNP.

While 7/22 of the subjects perished due to resulting infection from the body's inability to heal itself after surgery, the other 15 cases survived.

The article warned clinicians to be alert to the possibility that paraneoplastic pemphigus in lymphomas not of B-cell lineage.

[19] The University of Toronto has been working to develop a form of treatment that improves the patient's overall quality of life while remaining economically achievable.

It has proven to be effective among auto-immune diseases, but the correct administration process for treating PNP is yet to be defined.