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

The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present some clinical, histological and immunopathological characteristics that are different from the classical types. These are reviewed in this article. Future research may help definitively to locate the position of these forms in the pemphigus group, especially with regard to pemphigus herpetiformis and the IgG/ IgA pemphigus.

Pathology; Pemphigus; Skin; Diseases, Vesiculobullous

Authorship

Adriana Maria Porro MAILING ADDRESS: Adriana Maria Porro. Rua Borges Lagoa 508 - Vila Clementino. São Paulo - SP Brazil. E-mail: adriana.porro@uol.com.br

Dermatologist. Masters Degree and PhD . Adjunct Professor and Coordinator of Bullous Dermatosis at the Dermatology Department, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP) - São Paulo (SP), Brazil.Federal University of São PauloBrazilSão Paulo, SP, BrazilDermatologist. Masters Degree and PhD . Adjunct Professor and Coordinator of Bullous Dermatosis at the Dermatology Department, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP) - São Paulo (SP), Brazil.

Livia de Vasconcelos Nasser Caetano

Dermatologist with specialization in Bullous Dermatosis at the Dermatology Department, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP) - São Paulo (SP), Brazil.Federal University of São PauloBrazilSão Paulo, SP, Brazil Dermatologist with specialization in Bullous Dermatosis at the Dermatology Department, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP) - São Paulo (SP), Brazil.

Laura de Sena Nogueira Maehara

Dermatologist with specialization in Bullous Dermatosis and Pediatric Dermatology at the Dermatology Department, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP). PhD-candidate at UNIFESP (Translational Medicine) and the University of Groningen (Center for Blistering Diseases, Groningen University Medical Center, Netherlands).Federal University of São PauloDermatologist with specialization in Bullous Dermatosis and Pediatric Dermatology at the Dermatology Department, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP). PhD-candidate at UNIFESP (Translational Medicine) and the University of Groningen (Center for Blistering Diseases, Groningen University Medical Center, Netherlands).

Milvia Maria dos Santos Enokihara

Pathologist. Masters Degree and PhD. Dermatopathologist at the Dermatology and Pathology Departments, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP) - São Paulo (SP), Brazil.Federal University of São PauloBrazilSão Paulo, SP, Brazil Pathologist. Masters Degree and PhD. Dermatopathologist at the Dermatology and Pathology Departments, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP) - São Paulo (SP), Brazil.

MAILING ADDRESS: Adriana Maria Porro. Rua Borges Lagoa 508 - Vila Clementino. São Paulo - SP Brazil. E-mail: adriana.porro@uol.com.br

Conflict of interest: None

SCIMAGO INSTITUTIONS RANKINGS

Figures

Figures (4)

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FIGURE 1
Pemphigus herpetiformis: (A) patient presenting grouped vesicles, blisters, erosions and crusts onto an erythematous skin in a herpetiform pattern on her forearms; (B) similar lesions on her buttocks and back; (C) the same patient 10 days after pulse therapy with methylprednisolone (1 g/day for 3 days), showing a good clinical response; (D) histopathological exam of a forearm lesion showing suprabasal blister containing some acantholytic cels, neutrophils and eosinophils, besides focal eosinophilic spongiosis (HE 400x); (E) DIF of perilesional skin showing intercellular distribution of IgG and C3 throughout the entire epidermis

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FIGURE 2
IgA Pemphigus (IEN type): (A) and (B) vesicles, blisters, pustules and crusts confluent, occupying almost the entire trunk, neck and part of the upper limbs; (C) DIF: IgA deposits intercellular;( D) IIF showing presence of IgA in the patient´s sera (1:640)

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FIGURE 3
Paraneoplastic pemphigus: (A); ulcer in the side of the tongue, organ typically affected in paraneoplastic pemphigus. This patient also had erosions in the jugal mucosa and gingival enanthema. The diagnosis of an abdominal myofibroblastic tumor led to the suspicion of PNP, which was confirmed by indirect immunofluorescence in rat bladder and immunoblotting. The patient was initially treated with prednisone and azathioprine, and later, rituximab, with improvement; (B) DIF of perilesional patient's skin showing intercellular and basement membrane zone staining (IgG, 10x); (C) IIF in transitional epithelium: positive test for a patient with PNP (rat bladder, 10x); (D) Immunoblotting (left) and immunoprecipitation (right): detection of antibodies directed against periplakin (190 kd) and envoplakin (210 kd) is a criterium for diagnosis

Thumbnail

FIGURE 4
Paraneoplastic Pemphigus in patient presenting non-Hodgkin B-cell linfoma: (A) lesions affecting the lips and oral mucosa; (B) erosions on the back; (C) blisters on the hands; (D)histopathology showing suprabasal blister containing acantholytic cells (HE 40x); (E) closer view of the acantholytic cells and loss of intercellular cohesiveness (HE 400x); (F) DIF showing intercellular deposits of IgG and C3, and also linear deposits in the BMZ (DIF, 400x); (G) IIF (rat bladder) showing intercellular distribution of anti-IgG (1:320)

FIGURE 1 Pemphigus herpetiformis: (A) patient presenting grouped vesicles, blisters, erosions and crusts onto an erythematous skin in a herpetiform pattern on her forearms; (B) similar lesions on her buttocks and back; (C) the same patient 10 days after pulse therapy with methylprednisolone (1 g/day for 3 days), showing a good clinical response; (D) histopathological exam of a forearm lesion showing suprabasal blister containing some acantholytic cels, neutrophils and eosinophils, besides focal eosinophilic spongiosis (HE 400x); (E) DIF of perilesional skin showing intercellular distribution of IgG and C3 throughout the entire epidermis

FIGURE 2 IgA Pemphigus (IEN type): (A) and (B) vesicles, blisters, pustules and crusts confluent, occupying almost the entire trunk, neck and part of the upper limbs; (C) DIF: IgA deposits intercellular;( D) IIF showing presence of IgA in the patient´s sera (1:640)

FIGURE 3 Paraneoplastic pemphigus: (A); ulcer in the side of the tongue, organ typically affected in paraneoplastic pemphigus. This patient also had erosions in the jugal mucosa and gingival enanthema. The diagnosis of an abdominal myofibroblastic tumor led to the suspicion of PNP, which was confirmed by indirect immunofluorescence in rat bladder and immunoblotting. The patient was initially treated with prednisone and azathioprine, and later, rituximab, with improvement; (B) DIF of perilesional patient's skin showing intercellular and basement membrane zone staining (IgG, 10x); (C) IIF in transitional epithelium: positive test for a patient with PNP (rat bladder, 10x); (D) Immunoblotting (left) and immunoprecipitation (right): detection of antibodies directed against periplakin (190 kd) and envoplakin (210 kd) is a criterium for diagnosis

FIGURE 4 Paraneoplastic Pemphigus in patient presenting non-Hodgkin B-cell linfoma: (A) lesions affecting the lips and oral mucosa; (B) erosions on the back; (C) blisters on the hands; (D)histopathology showing suprabasal blister containing acantholytic cells (HE 40x); (E) closer view of the acantholytic cells and loss of intercellular cohesiveness (HE 400x); (F) DIF showing intercellular deposits of IgG and C3, and also linear deposits in the BMZ (DIF, 400x); (G) IIF (rat bladder) showing intercellular distribution of anti-IgG (1:320)

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