What is Pemphigus herpetiformis?
What is Pemphigus herpetiformis?
Pemphigus herpetiformis is a rare subtype of intraepidermal autoimmune bullous diseases that shares clinical characteristics with dermatitis herpetiformis and immunologic features with pemphigus. It usually has an indolent course and is less life-threatening than many forms of pemphigus.
What is pemphigus erythematosus?
Pemphigus erythematosus, or Senear-Usher syndrome, is an autoimmune skin blistering disorder with an overlapping clinical presentation of pemphigus foliaceus and systemic lupus erythematosus (SLE). It is generally regarded as a limited form of pemphigus foliaceus.
Is paraneoplastic pemphigus painful?
Paraneoplastic conditions occur in association with malignancies (cancer). Paraneoplastic pemphigus is characterised by painful blisters and denuded areas of the mouth, lips, oesophagus and skin. It is the least common but most serious form of pemphigus, particularly considering the presence of the underlying cancer.
What is a paraneoplastic phenomenon?
Paraneoplastic syndromes are a group of rare disorders that are triggered by an abnormal immune system response to a cancerous tumor known as a “neoplasm.” Paraneoplastic syndromes are thought to happen when cancer-fighting antibodies or white blood cells (known as T cells) mistakenly attack normal cells in the nervous …
What is Pregnancy PG?
Pemphigoid gestationis (PG) is a skin condition that usually presents in the second trimester of pregnancy; however, it may occur at any time during the pregnancy, as well as in the days post-partum. PG was formerly called herpes gestationis, but it not related to the herpes virus.
Is Pemphigus an autoimmune disease?
Pemphigus is a rare group of autoimmune diseases. It causes blisters on the skin and mucous membranes throughout the body. It can affect the mouth, nose, throat, eyes, and genitals. Pemphigus vulgaris is the most common type of pemphigus.
What is the Hallopeau type of pemphigus vegetans?
There are two clinically recognized forms of pemphigus vegetans, the Hallopeau type, and Neumann type. The Hallopeau type has an indolent course and characteristically demonstrates pustules that heal as vegetative plaques. The oral mucosa is frequently uninvolved.
What is the rarest clinical variant of pemphigus?
Among the many types of pemphigus, the rarest clinical variant is pemphigus vegetans. It distinguishes itself from pemphigus vulgaris by the formation of vegetative plaques in intertriginous areas and oral mucosa. Thought to be a subtype of pemphigus vulgaris, pemphigus vegetans represents 1-2% of all pemphigus .
What is the difference between Hallopeau and Neumann pustules?
The Hallopeau type has an indolent course and characteristically demonstrates pustules that heal as vegetative plaques. The oral mucosa is frequently uninvolved. The Neumann type is more severe and refractory to treatment, with vegetations that develop during an eruption of vesiculobullous lesions.
What are the treatment options for Pemphigus vegetans?
The mainstay of treatment for pemphigus vegetans is similar to that of pemphigus vulgaris, consisting of systemic corticosteroids and immunosuppressants. Copyright © 2021, StatPearls Publishing LLC.
