Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

Porro, Adriana María; Caetano, Livia de Vasconcelos Nasser; Maehara, Laura de Sena Nogueira; Enokihara, Milvia Maria dos Santos

doi:10.1590/abd1806-4841.20142459

Cited by 75 publications

(128 citation statements)

References 120 publications

(153 reference statements)

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“…disease, but a syndrome characterized by the presence of mucocutaneous and non-cutaneous pathology associated with neoplasia [3]. More than 260 case reports of PNP have been documented since that time [1,4,5] and about 20 of the reported patients were children or adolescents.…”

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confidence: 99%

Paraneoplastic Pemphigus

Zhong-Shuai¹,

Li²

2017

J Dermatol Res Ther

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Paraneoplastic Pemphigus (PNP) is an autoimmune-related acquired bullous disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. Suprabasal acantholysis and clefts with scattered necrotic keratinocytes are the unique histopathological features. PNP patient sera recognize multiple antigens, which have been identified as the plakin protein family. Non-Hodgkin's lymphoma, Castleman's disease, thymoma, follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasms in PNP. The treatment of PNP to date has been rather disappointing. For patients with Castleman's disease, the early detection and removal of the tumor are critical for the treatment of PNP.

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confidence: 99%

Paraneoplastic Pemphigus

Zhong-Shuai¹,

Li²

2017

J Dermatol Res Ther

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“…Patients with the SPD type sometimes show irregular erythematous skin lesions with vesiculopustules, erosions and desquamation resembling the features of subcorneal pusular dermatosis (Sneddon-Wilkinson disease) (15). The IEN type demonstrates a characteristic clinical feature, the so-called "sunflower-like" configuration (16). A herpetiform appearance has also been reported (17).…”

Section: Discussionmentioning

confidence: 99%

“…Acantholysis in IgA pemphigus is much milder than in classic pemphigus. Characteristically, the clefts and pustules localize in the subcorneal region in SPD type IGAP, whereas they are present in the entire or mid epidermis in IEN type IGAP (3,16). In the upper dermis, a superficial perivascular and interstitial infiltrates of lymphocytes, neutrophils and sometimes eosinophils are present (19) Desmocollin 1 (Dsc1) has recently been identified as the target antigen of the SPD type (15).…”

Section: Discussionmentioning

confidence: 99%

“…In SPD type, IgA deposition is seen predominantly in the upper epidermal layers, whereas in IEN type, it is seen throughout the epidermis or restricted to the lower epidermis. IgG or complement component C3 is also sometimes deposited, but is weaker than IgA (3,5,16,23).…”

Section: Discussionmentioning

confidence: 99%

“…Enzyme-linked immunosorbent assay (ELISA) is used for the diagnosis of IGAP and for detection of autoantibodies in individual patients (16,27). The specificity and sensitivity of Dsc and ELISA is not very high compared with immunofluorescence study using Dsc-transfected COS-7 cells (4,28).…”

Section: Discussionmentioning

confidence: 99%

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IgA Pemphigus in a Child – a Case Report

Lekić

Gajić-Veljić

Popadić

et al. 2017

Serbian Journal of Dermatology and Venereology

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IgA pemphigus (IGAP) is a rare autoimmune bullous disease characterized by IgA deposits on keratinocyte cell surfaces. The IGAP is classified into: 1) subcorneal pustular dermatosis (SPD) type, and 2) intraepidermal neutrophilic (IEN) IgA dermatosis type. So far, only 9 children with IGAP have been described in the literature, of whom only 3 with SPD type. We report a 3-year-old boy with SPD type of IGAP. Clinically, he presented with pruritic vesicles, pustules and erosions on the face, trunk, groin area, and extremities. Histopathology showed subcorneal pustules containing a few acantholytic cells. Direct immunofluorescence (DIF) test of Tzanck smear showed intercellular IgA deposits on the surface of the groups of epidermal cells. Oral dapsone and prednisone induced remission after two weeks; the treatment was discontinued 11 months later, and complete remission was achieved during 19 months without any treatment. Direct immunofluorescence of Tzanck smear is a simple, sensitive, rapid and non-aggressive test, very suitable for the diagnosis of IGAP in children.

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A case of IgA pemphigus, with a poor response to dapsone, successfully treated with adalimumab

Al‐Zahawi,

Kamyab,

Balighi

2024

Clinical Case Reports

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Key Clinical MessageIgA pemphigus is usually treated by Dapsone. Recalcitrant cases may be treated by Colchicine, Sulfapyridine, or Acitretin. Some patients with recurrent severe disease may not respond to the aforementioned medications. Our study highlights the role of TNFa inhibitor as an alternative modality in the treatment of recalcitrant IgA pemphigus.AbstractIgA pemphigus is a rare autoimmune blistering disease characterized by a pruritic, annular, vesiculopustular eruption. In IgA pemphigus, there are IgA autoantibodies targeting the keratinocyte cell surface adhesion molecules, causing cell‐to‐cell dehiscence and a flaccid vesiculopustular eruption, mainly in the axilla and groin. Dapsone, despite being the drug of choice for treating IgA pemphigus, is not effective in clearing lesions in a minority of patients and such rare cases of recalcitrant IgA pemphigus need alternative modalities of treatment. Here, we report the successful treatment of a 50‐year‐old male patient with an adalimumab injection who had a poor response to dapsone.

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Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

Cited by 75 publications

References 120 publications

Paraneoplastic Pemphigus

Paraneoplastic Pemphigus

IgA Pemphigus in a Child – a Case Report

A case of IgA pemphigus, with a poor response to dapsone, successfully treated with adalimumab

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