There are several types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, Intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus.

- Pemphigus vulgaris (PV - ICD-10 L10.0) is the most common form of the disorder and occurs when antibodies attack "Desmoglein 3". Sores often originate in the mouth, making eating difficult and uncomfortable. Although pemphigus vulgaris may occur at any age, it is most common among people between the ages of 40 and 60. It is more frequent among Ashkenazi Jews. Rarely, it is associated with myasthenia gravis. Nail disease may be the only finding and has prognostic value in management.

- Pemphigus foliaceus (PF) is the least severe of the three varieties. "Desmoglein 1", the protein that is destroyed by the autoantibody, is found in only the top dry layer of the skin. PF is characterized by crusty sores that often begin on the scalp, and may move to the chest, back, and face. Mouth sores do not occur. This form is also frequent among Ashkenazi Jews. It is not as painful as pemphigus vulgaris, and is often mis-diagnosed as dermatitis or eczema

- Intraepidermal neutrophilic IgA dermatosis is characterized histologically by intraepidermal bullae with neutrophils, some eosinophils, and acantholysis.

- The least common and most severe type of pemphigus is paraneoplastic pemphigus (PNP). This disorder is a complication of cancer, usually lymphoma and Castleman's disease. It may precede the diagnosis of the tumor. Painful sores appear on the mouth, lips, and the esophagus. In this variety of pemphigus, the disease process often involves the lungs, causing bronchiolitis obliterans (constrictive bronchiolitis). Though much less frequent, it is still found the most in the Ashkenazi Jewish population. Complete removal of and/or cure of the tumor may improve the skin disease, but lung damage is generally irreversible.

- Endemic pemphigus foliaceus, including the Fogo Selvagem, the new variant of endemic pemphigus folaiceus in El Bagre, Colombia, South America, and the Tunisian endemic pemphigus in North Africa.

Note that Hailey-Hailey disease, also called familial benign pemphigus, is an inherited (genetic) skin disease, not an autoimmune disease. It is therefore not considered part of the Pemphigus group of diseases.

Clinically, the earliest lesions may appear urticarial (like hives), but could also appear dermatitic, targetoid, lichenoid, nodular, or even without visible rash (essential pruritus). Tense bullae eventually erupt, most commonly at the inner thighs and upper arms, but the trunk and extremities are frequently both involved. Any part of the skin surface can be involved. Oral lesions are present in a minority of cases. The disease may be acute, but typically will wax and wane. Several other skin diseases may have similar symptoms. However, milia are more common with epidermolysis bullosa acquisita, because of the deeper antigenic targets. A more ring-like configuration, with a central depression or centrally collapsed bullae may indicate linear IgA disease. Nikolsky's sign is negative unlike pemphigus vulgaris where it is positive.

The forms of pemphigoid are considered to be connective tissue autoimmune skin diseases. There are several types:

- Gestational pemphigoid or Pemphigoid gestationis (PG) (formerly called Herpes gestationis)

- Bullous pemphigoid (BP) Rarely affect the mouth

- Mucous membrane pemphigoid, also known as Cicatricial pemphigoid (CP) (No skin involvement)

Bullous and Cicatricial pemphigoids usually affect persons who are over age 60. Gestational pemphigoid occurs during pregnancy, typically in the second or third trimester, and/or immediately following pregnancy.

Pigmented purpuric dermatosis (also known as "progressive pigmentary dermatosis," "purpura pigmentosa chronica," "pigmentary purpuric eruptions," or "progressive pigmenting purpura" or "Schamberg's disease") refers to one of the three major classes of skin conditions characterized by purpuric skin eruptions.

Pigmented purpuric dermatosis are distinguished from other purpura by size (0.3–1 cm) and are most often seen in the lower extremities. Pigmentary purpuric eruptions may present with one of several clinical patterns. There may be overlapping characteristics among pigmented purpuric dermatosis and between their signs and those of other purpuric eruptions. Examples of the pigmented purpuric dermatosis group include:

Although vascular damage may be present, it is insufficient for these conditions to be considered forms of vasculitis.

Pemphigus ( or ) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. The name is derived from the Greek root "pemphix" meaning "pustule".

In pemphigus, autoantibodies form against desmoglein. Desmoglein forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes "unglued", a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a significant area of the skin.

Originally, the cause of this disease was unknown, and "pemphigus" was used to refer to any blistering disease of the skin and mucosa. In 1964, researchers found that the blood of patients with pemphigus contained antibodies to the layers of skin that separate to form the blisters. In 1971, an article investigating the autoimmune nature of this disease was published.

Pemphigoid is a group of rare autoimmune blistering skin diseases. As its name indicates, pemphigoid is similar in general appearance to pemphigus, but, unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells.

Pemphigoid is more common than pemphigus, and is slightly more common in women than in men. It is also more common in people over 60 years of age than it is in younger people.

Bullous pemphigoid is an acute or chronic autoimmune skin disease, involving the formation of blisters, more appropriately known as bullae, at the space between the epidermis and dermis skin layers. It is classified as a type II hypersensitivity reaction, with the formation of anti-hemidesmosome antibodies.

Dermatitis herpetiformis is characterized by intensely itchy, chronic papulovesicular eruptions, usually distributed symmetrically on extensor surfaces (buttocks, back of neck, scalp, elbows, knees, back, hairline, groin, or face). The blisters vary in size from very small up to 1 cm across.

The condition is extremely itchy, and the desire to scratch can be overwhelming. This sometimes causes the sufferer to scratch the blisters off before they are examined by a physician. Intense itching or burning sensations are sometimes felt before the blisters appear in a particular area.

Untreated, the severity of DH can vary significantly over time, in response to the amount of gluten ingested.

Dermatitis herpetiformis symptoms typically first appear in the early years of adulthood between 20 and 30 years of age.

Although the first signs and symptoms of dermatitis herpetiformis are intense itching and burning, the first visible signs are the small papules or vesicles that usually look like red bumps or blisters. The rash rarely occurs on other mucous membranes, excepting the mouth or lips. The symptoms range in severity from mild to serious, but they are likely to disappear if gluten ingestion is avoided and appropriate treatment is administered.

Dermatitis herpetiformis symptoms are chronic, and they tend to come and go, mostly in short periods of time. Sometimes, these symptoms may be accompanied by symptoms of coeliac disease, commonly including abdominal pain, bloating or loose stool, and fatigue.

The rash caused by dermatitis herpetiformis forms and disappears in three stages. In the first stage, the patient may notice a slight discoloration of the skin at the site where the lesions appear. In the next stage, the skin lesions transform into obvious vesicles and papules that are likely to occur in groups. Healing of the lesions is the last stage of the development of the symptoms, usually characterized by a change in the skin color. This can result in areas of the skin turning darker or lighter than the color of the skin on the rest of the body. Because of the intense itching, patients usually scratch, which can lead to the formation of crusts.

Maternal autoimmune bullous disease is a blistering skin condition that presents at birth.

Urticarial dermatoses are distinct from urticaria, which examples being drug-induced urticaria, eosinophilic cellulitis and bullous pemphigoid. It is important to distinguish urticaria from urticarial dermatoses. The individual wheals of urticaria are ‘here today and gone tomorrow’ (i.e. they last less than 24 hours), whereas with urticarial dermatoses, the individual lesions last for days or longer.

The disease presents with the widespread formation of fluid-filled blisters that are thin walled and easily ruptured and the patient can be positive for Nikolsky's sign. Ritter's Disease of the Newborn is the most severe form of SSSS with similar signs and symptoms. SSSS often includes a widespread painful erythroderma, often involving the face, diaper, and other intertriginous areas. Extensive areas of desquamation might be present. Perioral crusting and fissuring are seen early in the course. Unlike toxic epidermal necrolysis, SSSS spares the mucous membranes. It is most common in children under 6 years, but can be seen in adults who are immunosuppressed or have renal failure.

Autoimmune estrogen dermatitis presents as a cyclic skin disorder, that may appear eczematous, papular, bullous, or urticarial. with pruritus typically present, skin eruptions that may be chronic but which are exacerbated premenstrually or occur immediately following menses.

Although lichen planus can present with a variety of lesions, the most common presentation is as a well defined area of purple-coloured, itchy, flat-topped papules with interspersed lacy white lines (Wickham's striae). This description is known as the characteristic "6 Ps" of lichen planus: planar (flat-topped), purple, polygonal, pruritic, papules, and plaques. This rash, after regressing, is likely to leave an area of hyperpigmentation that slowly fades. That said, a variety of other lesions can also occur.

Vesicular pemphigoid is a cutaneous condition, a clinical variant of bullous pemphigoid, characterized by a dermatitis herpetiformis-like presentation with grouped small tense blisters.

Lichen planus may be categorized as affecting mucosal or cutaneous surfaces.

- "Cutaneous" forms are those affecting the skin, scalp, and nails.

- "Mucosal" forms are those affecting the lining of the gastrointestinal tract (mouth, pharynx, esophagus, stomach, anus), larynx, and other mucosal surfaces including the genitals, peritoneum, ears, nose, bladder and conjunctiva of the eyes.

Although other forms of the disease occur at younger ages, some individuals start having long term symptoms at thirty years of age. This disease also affects adolescents and young adults. This also affects the immune system which therefore results in rashes. The symptoms rarely affect the face or scalp, but occurs at other sites of the body. The duration may last for months or even several years. For instance, new crops of lesions appear every few weeks.

Erythema multiforme major (also known as "erythema multiforme majus") is a form of rash with skin loss or epidermal detachment.

The term "erythema multiforme majus" is sometimes used to imply a bullous (blistering) presentation.

According to some sources, there are two conditions included on a spectrum of this same disease process:

- Stevens–Johnson syndrome (SJS)

- Toxic epidermal necrolysis (TEN) which described by Alan Lyell and previously called Lyell syndrome[5].

In this view, EM major, SJS and TEN are considered a single condition, distinguished by degree of epidermal detachment.

However, a consensus classification separates erythema multiforme minor, erythema multiforme major, and SJS/TEN as three separate entities.

Erosive pustular dermatitis of the scalp (also known as "Erosive pustular dermatosis of the scalp") presents with pustules, erosions, and crusts on the scalp of primarily older Caucasean females, and on biopsy, has a lymphoplasmacytic infiltrate with or without foreign body giant cells and pilosebaceous atrophy.

Grover's disease often starts quite suddenly. There are intensely itchy spots on the central back, mid chest and occasionally elsewhere. Frequently, it follows sweating or some unexpected heat stress.

The itchy eruption lasts an average of 10–12 months. It is characterized by papules and papulovesicles with excoriations occurring on the chest, back, lower sternum, arms, and thighs. The papules are most commonly found on the mid chest.

Sometimes the features of Grover's are found in people who do not itch or have a conspicuous rash. Most of the people with Grover's who visit a dermatologist, however, itch a lot.

Pityriasis lichenoides chronica is an uncommon, idiopathic, acquired dermatosis, characterized by evolving groups of erythematous, scaly papules that may persist for months.

Linear IgA bullous dermatosis is frequently associated with medication exposure, especially vancomycin, with men and women being equally affected. It was first described by Tadeusz Chorzelski in 1979. Linear IgA dermatosis is a rare immune-mediated blistering skin disease that may be divided into two types:

- "Adult linear IgA disease" is an acquired, autoimmune blistering disease that may present with a clinical pattern of vesicles indistinguishable from dermatitis herpetiformis, or with vesicles and bullae in a bullous pemphigoid-like appearance. This disease can often be difficult to treat even with usually effective medications such as rituximab.

- "Childhood linear IgA disease" (also known as "Chronic bullous disease of childhood") is an acquired, self-limited bullous disease that may begin by the time the patient is age 2 to 3 and usually remits by age 13.

IgA pemphigus is a subtype of pemphigus with two distinct forms:

- "Subcorneal pustular dermatosis" (also known as Sneddon–Wilkinson disease and pustulosis subcornealis) is skin condition that is a rare, chronic, recurrent, pustular eruption characterized histopathologically by subcorneal pustules that contain abundant neutrophils. This is distinct from and not to be confused with subcorneal pustular dermatosis type of IgA pemphigus. Sneddon's syndrome, also known as Ehrmann-Sneddon syndrome, is also a different syndrome.

- "Intraepidermal neutrophilic IgA dermatosis" is characterized histologically by intraepidermal bullae with neutrophils, some eosinophils, and acantholysis.

Bullous drug reaction (also known as a "bullous drug eruption", "generalized bullous fixed drug eruption", and "multilocular bullous fixed drug eruption") most commonly refers to a drug reaction in the erythema multiforme group. These are uncommon reactions to medications, with an incidence of 0.4 to 1.2 per million person-years for toxic epidermal necrolysis and 1.2 to 6.0 per million person-years for Stevens–Johnson syndrome. The primary skin lesions are large erythemas (faintly discernible even after confluence), most often irregularly distributed and of a characteristic purplish-livid color, at times with flaccid blisters.

Dermatitis herpetiformis (DH), or Duhring's disease, is a chronic blistering skin condition, characterised by blisters filled with a watery fluid. Despite its name, DH is neither related to nor caused by herpes virus: the name means that it is a skin inflammation having an appearance similar to herpes.

DH was first described by Louis Adolphus Duhring in 1884. A connection between DH and celiac disease was recognised in 1967, although the exact causal mechanism is not known. DH is a specific manifestation of celiac disease.

The age of onset is usually about 15-40, but DH can also affect children and the elderly. Men and women are equally affected. Estimates of DH prevalence vary from 1 in 400 to 1 in 10,000. It is most common in patients of northern European/northern Indian ancestry, and is associated with the human leukocyte antigen (HLA) haplotype HLA-DQ2 along with coeliac disease and gluten sensitivity.

Neutrophilic dermatosis of the dorsal hands (also known as "Pustular vasculitis of the dorsal hands") is a skin condition that presents with edematous pustular or ulcerative nodules or plaques localized to the dorsal hands.