IgA Pemphigus

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IgA pemphigus

IgA Pemphigus

IgA pemphigus also called immunoglobulin A pemphigus or intercellular IgA dermatosis is a very rare autoimmune blistering skin disease presenting as painful, itchy blisters that fill with neutrophils and progressing into pus-filled blisters (pustules) 1, 2, 3, 4, 5. IgA pemphigus skin blisters and skin pustules occur due to circulating immunoglobulin-A (IgA) autoantibodies targeting keratinocyte cell surface components involved in cell-to-cell adherence.

Normally your immune system makes antibodies to protect you against bacteria, viruses, fungi, parasites and cancer cells and these antibodies do not attack your own body. However, in IgA pemphigus, your immune system makes antibodies (autoantibodies) that attack the “glue” holding the outer layer of the skin (epidermis) together, making it fragile and prone to blistering. It is not clear why some people develop these autoantibodies. The immunoglobulin A (IgA) autoantibodies in IgA pemphigus attack proteins called desmogleins (Dsg). These proteins form the “glue” that holds the cells of the skin together. When the immunoglobulin A (IgA) autoantibodies formed in IgA pemphigus attack the desmoglein proteins, the cells in the skin no longer hold together and separate. This causes blisters that are typical of IgA pemphigus.

IgA pemphigus has 2 major subtypes 6, 7:

  • Subcorneal pustular dermatosis (SPD) type IgA pemphigus
    • Deposition of immunoglobulin A (IgA) autoantibodies against the desmocollin-1 glycoprotein, one of the desmosomal cadherins, that maintain the shape of a cell. The result is the surface keratinocytes separate from each other, and are replaced by fluid or blister. Because the blister is very close to the surface of the skin, the blisters rupture easily.
    • Subcorneal pustular dermatosis (SPD)-type IgA pemphigus shows subcorneal pustules.
  • Intraepidermal neutrophilic (IEN) type IgA pemphigus
    • Deposition of immunoglobulin A (IgA) autoantibodies against desmoglein (Dsg) members of the cadherin superfamily seen predominantly in lower epidermis.
    • Intraepidermal neutrophilic (IEN)-type IgA pemphigus is characterized by pustule formation throughout the entire epidermis 8, 9, 10. In at least some cases of intraepidermal neutrophilic (IEN)-type IgA pemphigus, the autoantigens are desmoglein-1 (Dsg1), desmoglein-3 (Dsg3) and an unspecified transmembrane protein 9, 10.
  • Both subtypes exhibit immunologic mechanisms targeting keratinocyte cell surface components.

A recent classification that classified IgA pemphigus cases from clinical, histopathological, and immunological perspectives describes 6 new subtypes of IgA pemphigus 11:

  • Subcorneal pustular dermatosis (SPD) type IgA pemphigus
  • Intraepidermal neutrophilic (IEN) type IgA pemphigus
  • IgA‐pemphigus foliaceus
  • IgA-pemphigus vulgaris
  • IgA‐pemphigus vegetans
  • Unclassified IgA pemphigus.

A autoimmune blistering skin disease may be suspected often after the skin condition has failed to clear with antibiotics.

When obtaining a patient’s medical history, clinicians should ascertain the presence of mucosal involvement, as the presence of mucosal lesions can help distinguish between different subtypes of pemphigus disease. IgA pemphigus usually lacks mucosal involvement 1. The initial manifestation of IgA pemphigus involves a subacute onset of flaccid blisters on an erythematous base filled with clear fluid. These evolve into pustules that rapidly rupture, forming annular crusts. Patients often describe these plaques as both painful and itchy. Although commonly observed in skin folds areas such as the armpit and groin, the trunk and extremities are the most commonly affected areas 12. Patients typically experience symptoms limited to the skin with no systemic symptoms such as fever, malaise, headache, or weight loss 13, 14.

IgA pemphigus diagnosis requires a skin biopsy for histology and direct immunofluorescent (DIF) testing. Clinicians should obtain a 4-mm lesional biopsy from the edge of an early lesion or erosion for hematoxylin and eosin staining and routine histopathological examination. In IgA pemphigus, a skin biopsy reveals intraepidermal neutrophil infiltration 15, 16. Blistering skin specimens will show mixed inflammatory infiltrate in upper, mid or lower epidermis, depending on the subtype with little or no acantholysis (the separation of keratinocytes).

For direct immunofluorescence (DIF), an additional perilesional skin biopsy should be taken from unaffected skin, situated 4 mm away from a vesicle or erosion. Lesional skin biopsies for direct immunofluorescence are more likely to give a false negative result due to the inflammatory response potentially destroying the immunoreactants.

Direct immunofluorescence (DIF) of a skin biopsy of healthy appearing skin close to the area of blistering reveals IgA deposition in epidermal keratinocyte cell membranes.

Indirect immunofluorescence (IIF) detects the presence of IgA circulating autoantibodies in patient serum using patient serum on monkey esophagus or other epithelial substrates. Indirect immunofluorescence (IIF) testing of serum can show IgA anti-keratinocyte cell-surface autoantibodies in approximately 50% of affected patients 6. Immunoglobulin G (IgG) anti-keratinocyte cell-surface antibodies have also been reported.

Immunoblotting allows clinicians to document the specific skin antigen recognized by the patient’s IgA autoantibodies 1. The sensitivity of immunoblotting is approximately 40% in revealing IgA reactivity to any autoantigen 1. Enzyme-linked immunosorbent assay (ELISA) can detect specific desmosomal antigens in patients with IgA pemphigus but is associated with a low sensitivity of approximately 55% 1.

Wallach et al. 17 observed the association of IgA monoclonal gammopathy in patients with IgA pemphigus. Hiroshi et al 18 raised concern about the association of subcorneal pustular dermatosis (SPD) type IgA pemphigus with IgA‐type multiple myeloma. The introduction of COVID‐19 vaccination in 2021 was accompanied by different dermatological side effects; one of the reported skin disease after COVID‐19 vaccination was IgA pemphigus 19. IgA pemphigus also has been reported in patients with ulcerative colitis and it seems that patients with ulcerative colitis respond less to the usual conventional treatment 20, 21. Three cases of IgA pemphigus have been reported in patients with human immunodeficiency virus (HIV) in which they responded very well to topical clobetasol and dapsone 22. Other reported associations with IgA pemphigus include rheumatoid arthritis and Sjogren syndrome 1. Given the potential association of IgA pemphigus with underlying cancers such as multiple myeloma and chronic conditions such as monoclonal IgA gammopathy, human immunodeficiency virus (HIV) infection, Sjögren’s syndrome, rheumatoid arthritis, and Crohn’s disease, a thorough physical examination and additional testing are essential to rule out these potentially serious conditions.

Since few cases of IgA pemphigus have been described, the most effective treatment is unknown. IgA pemphigus is usually treated with a combination of systemic steroids (anti-inflammatory and reduce overactive immune system) and dapsone which is thought to reduce neutrophil infiltration. This combination treatment is more effective than the use of steroids alone.

Other medications and treatment options worth considering include 23:

  • Colchicine
  • Mycophenolate mofetil 24
  • Isotretinoin 25
  • Apremilast 26
  • Acitretin
  • Alitretinoin
  • Adalimumab 27
  • UVB phototherapy 28

Although IgA pemphigus typically heals without scarring, patients must understand the medications used for treatment, including any potential adverse effects. Abruptly stopping medications, especially oral steroids, can lead to a recurrence of skin lesions and adrenal insufficiency. Patients must understand the need for a gradual reduction in medication dosage. In addition, if concurrent medical conditions are present, such as cancers or gastrointestinal diseases, effectively diagnosing and managing these conditions are essential for overall health and may impact the prognosis of IgA pemphigus. Clinicians should stress the importance of continuing treatment and regularly attending scheduled follow-up appointments.

Figure 1. IgA pemphigus

IgA pemphigus

Footnote: IgA pemphigus with small vesciles and pustules mostly on well-circumscribed red patches on the armpit.

[Source 7 ]

Figure 2. IgA pemphigus

IgA Pemphigus

Footnotes: IgA pemphigus with multiple pustules on a reddish base with areas of hyperpigmentation in armpit (A), groin (B), behind left ear (C), and the neck (D).

[Source 29 ]

Figure 3. IgA pemphigus with oral mucosal lesions

IgA pemphigus with oral mucosal lesions

Footnote: IgA pemphigus with multiple erosions of the oral mucosa.

[Source 30 ]

Figure 4. IgA pemphigus direct immunofluorescence

IgA pemphigus direct immunofluorescence

Footnote: IgA pemphigus direct immunofluorescence (DIF) of perilesional skin showing intercellular deposits of IgA throughout the epidermis.

[Source 7 ]

Who gets IgA pemphigus?

IgA pemphigus can occur in any age group, the reported range is 1 month to 94 years, but is more commonly seen between the fourth and sixth decades with no distinct gender predilection in reported cases 31. , 32, 33.

IgA Pemphigus causes

IgA pemphigus is an autoimmune blistering skin disease caused by anti‐keratinocyte cell surface IgA autoantibodies, but the triggering factors is unknown 34, 1. In IgA pemphigus, immunoglobulin A (IgA) autoantibodies specifically target desmosomal and nondesmosomal keratinocyte cell surface components, notably desmoglein-1 (Dsg1), desmoglein-3 (Dsg3) and desmocollin-1 glycoproteins belong to the cadherin superfamily and are responsible for adhesion between skin cells. Despite identifying IgA autoantibody targets, the precise mechanistic cascade underlying the clinical presentation of IgA pemphigus remains elusive and necessitates further research 6. Research suggests the potential involvement of interleukin 5 (IL-5), a T-helper type 2 cytokine associated with stimulating IgA class antibody and γδ T-cell receptor–containing T cells, crucial for mucosal IgA production 1. In addition, the IgA autoantibodies possess specific binding sites for the monocyte/granulocyte IgA-Fc receptor (CD89), which may allow neutrophils to accumulate intraepidermally, causing the blistering process to occur 1.

IgA pemphigus has 2 major subtypes 6, 7:

  • Subcorneal pustular dermatosis (SPD) type IgA pemphigus
    • Deposition of immunoglobulin A (IgA) autoantibodies against the desmocollin-1 glycoprotein, one of the desmosomal cadherins, that maintain adhesion between skin cells. The result is the surface keratinocytes separate from each other, and are replaced by fluid or blister. Because the blister is very close to the surface of the skin, the blisters rupture easily.
    • Subcorneal pustular dermatosis (SPD)-type IgA pemphigus shows subcorneal pustules.
  • Intraepidermal neutrophilic (IEN) type IgA pemphigus
    • Deposition of immunoglobulin A (IgA) autoantibodies against desmoglein (Dsg) members of the cadherin superfamily seen predominantly in lower epidermis. In at least some cases of intraepidermal neutrophilic (IEN)-type IgA pemphigus, the autoantigens are desmoglein-1 (Dsg1), desmoglein-3 (Dsg3) and an unspecified transmembrane protein 9, 10.
    • The binding of IgA autoantibodies to the IgA-Fc receptor (CD89) triggers an intense inflammatory response, leading to epidermal neutrophilic infiltration followed by the formation of blisters and pustules. Intraepidermal neutrophilic (IEN)-type IgA pemphigus is characterized by pustule formation throughout the entire epidermis 8, 9, 10.
  • Both subtypes exhibit immunologic mechanisms targeting keratinocyte cell surface components.

In direct immunofluorescence (DIF), subcorneal pustular dermatosis (SPD)-type IgA pemphigus involves cell surface IgA binding only in the upper epidermis, where as intraepidermal neutrophilic (IEN)-type IgA pemphigus shows binding throughout the epidermis 8.

IgA pemphigus is also associated with monoclonal IgA gammopathy and multiple myeloma 17. Experts are uncertain if monoclonal gammopathy precedes or follows IgA pemphigus, but most cases are present at the time of diagnosis 1. Other associated diseases include HIV infection, Sjögren syndrome, rheumatoid arthritis, lung cancer, ulcerative colitis, peripheral T-cell lymphoma, chronic myeloid leukemia, and diffuse large B-cell lymphoma 1. Although the direct relationship between the various diseases and IgA pemphigus is still unclear, healthcare professionals should thoroughly diagnose patients presenting with IgA pemphigus for blood, gastrointestinal, rheumatological, and infectious disorders 35, 36.

IgA Pemphigus symptoms

IgA pemphigus signs and symptoms include skin blisters, skin pustules (pus-filled blister), skin redness, skin erosions and vegetating lesions. The first signs are flaccid vesicles (thin-walled sacs filled with a fluid) and pustules (pus-filled blisters). The skin vesicles and pustules rupture to form erosions and crusted plaques. The eruption tends to follow a circular pattern. A herpetiform appearance (tendency for blisters to appear in clusters, resembling herpes simplex infection) has also been reported 9. The whole body can be involved appears to favor the trunk, upper and lower extremities, armpits, groin and submammary (under the breast) area 8. Mucosa is usually free of lesions 9, 37. However, a recent systematic review found oral mucosal involvement in 16 of 121 (13.2%) patients with IgA pemphigus 34.

IgA Pemphigus complications

Many potential complications associated with IgA pemphigus are due to the effects of long-term treatment. Secondary infection of the lesions and scarring are the primary complications associated with IgA pemphigus.

Potential complications related to corticosteroid use 6:

  • Osteoporosis
  • Peptic ulcer disease
  • Adrenal insufficiency
  • Infection
  • Growth restriction in children
  • Weight gain
  • Anemia
  • Hypertension
  • Diabetes

Potential complications related to dapsone use include 1:

  • Methemoglobinemia
  • Hemolytic anemia
  • Neutropenia
  • Agranulocytosis
  • Hepatic failure
  • Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome

IgA Pemphigus diagnosis

When obtaining a patient’s medical history, clinicians should ascertain the presence of mucosal involvement, as the presence of mucosal lesions can help distinguish between different subtypes of pemphigus disease. IgA pemphigus usually lacks mucosal involvement 1. The initial manifestation of IgA pemphigus involves a subacute onset of flaccid blisters on an erythematous base filled with clear fluid. These evolve into pustules that rapidly rupture, forming annular crusts. Patients often describe these plaques as both painful and itchy. Although commonly observed in skin folds areas such as the armpit and groin, the trunk and extremities are the most commonly affected areas 12. Patients typically experience symptoms limited to the skin with no systemic symptoms such as fever, malaise, headache, or weight loss 13, 14.

IgA pemphigus diagnosis requires a skin biopsy for histology and direct immunofluorescent (DIF) testing. Clinicians should obtain a 4-mm lesional biopsy from the edge of an early lesion or erosion for hematoxylin and eosin staining and routine histopathological examination. In IgA pemphigus, a skin biopsy reveals intraepidermal neutrophil infiltration 15, 16. Blistering skin specimens will show mixed inflammatory infiltrate in upper, mid or lower epidermis, depending on the subtype with little or no acantholysis.

For direct immunofluorescence (DIF), an additional perilesional skin biopsy should be taken from unaffected skin, situated 4 mm away from a vesicle or erosion. Lesional skin biopsies for direct immunofluorescence are more likely to give a false negative result due to the inflammatory response potentially destroying the immunoreactants.

Direct immunofluorescence (DIF) of a skin biopsy of healthy appearing skin close to the area of blistering reveals IgA deposition in epidermal keratinocyte cell membranes.

Indirect immunofluorescence (IIF) detects the presence of IgA circulating autoantibodies in patient serum using patient serum on monkey esophagus or other epithelial substrates. Indirect immunofluorescence (IIF) testing of serum can show IgA anti-keratinocyte cell-surface autoantibodies in approximately 50% of affected patients 6. Immunoglobulin G (IgG) anti-keratinocyte cell-surface antibodies have also been reported.

Immunoblotting allows clinicians to document the specific skin antigen recognized by the patient’s IgA autoantibodies 1. The sensitivity of immunoblotting is approximately 40% in revealing IgA reactivity to any autoantigen 1. Enzyme-linked immunosorbent assay (ELISA) can detect specific desmosomal antigens in patients with IgA pemphigus but is associated with a low sensitivity of approximately 55% 1.

Histopathology

Histological examination of IgA pemphigus reveals subcorneal blisters with massive neutrophilic infiltration and a mild loss of cohesion between keratinocytes 1. This histopathological analysis aids in distinguishing the 2 major subtypes of IgA pemphigus 1. The subcorneal pustular dermatosis (SPD) subtype IgA pemphigus may reveal minimal subcorneal acantholysis and pustules with increased IgA autoantibody intensity on the epidermis’s upper surface. In contrast, the characteristic features of the intraepidermal neutrophilic (IEN) dermatosis subtype IgA pemphigus are pustules deeper in the epidermis and inflammatory infiltrates, primarily in the entire or lower part of the dermis 38, 39. Although immunoglobulin A (IgA) is predominately detected on direct immunofluorescence (DIF), occasionally, immunoglobulin G (IgG) and complement component C3 may be detected to a much lesser extent 1. Unlike IgG pemphigus, the separation of keratinocytes (acantholysis) may be minimal or absent in IgA pemphigus 1.

IgA pemphigus differential diagnosis

Due to the rarity of IgA pemphigus, clinicians should consider various differential diagnoses when managing and treating the condition.

IgA pemphigus differential diagnosis include 1:

  • Bacterial skin infection
  • Bullous impetigo
  • Pustular psoriasis
  • Dermatitis herpetiformis
  • Linear IgA bullous dermatosis
  • Pemphigus foliaceus. Pemphigus foliaceus is characterized by flaccid bullae (large skin blisters filled with clear fluid) typically found on the trunk that eventually crust over, much like the lesions in IgA pemphigus. Generally considered a benign disease, pemphigus foliaceus responds well to topical and oral corticosteroids. A clinical differentiation between IgA pemphigus and pemphigus foliaceus is nearly impossible 1. Therefore, immunofluorescence is critical in diagnosis. Direct immunofluorescence (DIF) of pemphigus foliaceus demonstrates IgG autoantibodies against desmoglein-1 in contrast to the IgA deposits against desmocollin-1 found in IgA pemphigus 1. Therefore, proper histology and immunofluorescence diagnosis are essential in differentiating the 2 conditions 40.
  • Eosinophilic pustular folliculitis
  • Subcorneal pustular dermatosis or Sneddon-Wilkinson disease. Subcorneal pustular dermatosis or Sneddon-Wilkinson disease is a chronic skin disease characterized by sterile pustular lesions that erupt in cyclical patterns 1. Similar to the subcorneal pustular dermatosis (SPD) subtype of IgA pemphigus, the pustular lesions observed in Sneddon-Wilkinson disease coalesce in an circular pattern and eventually burst to form crusted plaques. The 2 conditions have the same distribution area, favoring the groin, trunk, and armpits while avoiding mucosal surfaces. Histological examination of Sneddon-Wilkinson disease demonstrates perivascular infiltration of neutrophils and mild spongiosis. However, in contrast to IgA pemphigus, direct immunofluorescence of Sneddon-Wilkinson disease reveals a negative result for IgA deposits against adhesion molecules such as desmocollin-1 41.

The clinical presentation of these conditions is remarkably similar, and careful investigation using histology and immunofluorescence may be necessary to differentiate these conditions 13.

IgA Pemphigus treatment

Due to the inflammatory nature of IgA pemphigus, the primary treatment approach for IgA pemphigus involves the use of oral and topical corticosteroids with a suggested daily dose of 0.5 to 1 mg/kg 42. However, patients should be aware of the potential side effects associated with the long-term use of steroids, including osteoporosis, diabetes, cataracts, adrenal suppression, and infection. In contrast to IgG pemphigus, IgA pemphigus typically does not respond adequately to steroid therapy alone 43. Numerous studies demonstrate that combining dapsone with corticosteroids yields significantly better outcomes 1. Dapsone primarily works by suppressing neutrophilic infiltration 1. Healthcare professionals should carefully monitor patients receiving dapsone for potential adverse effects, such as hemolysis and methemoglobinemia.

Other medications reported to effectively treat IgA pemphigus include colchicine, retinoids, mycophenolate mofetil, and adalimumab 44, 24. Many of these medications were tested based on their successful treatment of other forms of neutrophilic skin diseases and classic pemphigus. For example, adalimumab, a recombinant human immunoglobulin antibody, is believed to be therapeutic due to its tumor necrosis factor-α (TNF-α) inhibition. TNF-α activates neutrophilic infiltration in the epidermis; therefore, its inhibition may hinder the further progression of IgA pemphigus 45. Rituximab (monoclonal anti-CD20), a monoclonal antibody targeting the B-lymphocyte antigen CD20 protein on cells, has also been safely used to treat patients with IgA pemphigus 46.

Clinicians should also consider providing proton-pump inhibitors (PPIs) and bisphosphonate therapy to prevent peptic ulceration and osteoporosis 1. Patients should receive counseling regarding weight-bearing exercise and adequate calcium and vitamin D intake 1. Patients should undergo a thorough physical examination and, when clinically indicated, appropriate testing to exclude cancers, inflammatory bowel disease, rheumatological diseases, and infectious diseases 1.

IgA Pemphigus prognosis

In comparison to pemphigus vulgaris and pemphigus foliaceus, IgA pemphigus presents with a milder disease and more localized condition and carries a better prognosis. When managed appropriately with regular monitoring, IgA pemphigus mostly heals with no scarring but it is common to have recurrent eruptions at the sites of previous lesions, for which prolonged treatment with dapsone and steroids may be required to prevent such recurrences 1. Studies indicate that abruptly discontinuing oral steroids may lead to lesion recurrence; therefore, clinicians should gradually reduce and taper the steroid dosage 1. People with IgA pemphigus should be counseled about potential side effects of steroids, dapsone and other alternative medications. Prognosis in cases of IgA pemphigus associated with other conditions such as cancers, gastrointestinal diseases, or monoclonal gammopathy depends on the progression of the underlying conditions 6, 18. IgA pemphigus associated with other medical conditions should be managed by multidisciplinary team.

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