The importance of direct immunofluorescence in pemphigus herpetiformis diagnosis

Faria, Paula Carolina Pessanha de; Cruz, Camila Caberlon; Abulafia, Luna Azulay; Maceira, Juan Piñeiro; Cassia, Flávia de Freire; Medeiros, Paula Mota

doi:10.1590/abd1806-4841.20174510

Cited by 7 publications

(6 citation statements)

References 9 publications

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“…The initial search yielded 371 publications: 284 from the PubMed database and 87 from the Ovid Medline database. We identified 96 publications (88 from PubMed and 63 from Ovid Medline [55 duplicates between the two databases]) reporting 158 individual cases that fulfilled the inclusion criteria. We also report an additional three unpublished cases diagnosed at our institution.…”

Section: Resultsmentioning

confidence: 99%

Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria

2019

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Pemphigus herpetiformis (PH), a rare type of pemphigus, is characterized by immunologic findings consistent with pemphigus but with a unique clinical and pathologic presentation. PH was first described as resembling dermatitis herpetiformis clinically, but because of its variable presentation, it can also resemble linear immunoglobulin A bullous dermatosis and bullous pemphigoid. We reviewed reported cases to analyze the most frequent clinical, pathologic, and immunologic characteristics and to propose corresponding diagnostic criteria. Through a comprehensive review of Medline and PubMed databases, 96 publications and 158 cases were identified. After reviewing the reported characteristics of PH, we suggest the following diagnostic criteria: Clinical: 1) pruritic herpetiform intact blisters with/without erosions; and/or 2) pruritic annular or urticarial erythematous plaques with/without erosions; Pathologic: 1) intraepidermal eosinophils or neutrophils, or both; and/or 2) intraepidermal split with/without acantholysis; Immunologic: 1) direct immunofluorescence showing immunoglobulin G with/without C3 intercellular deposits; and/or 2) indirect immunofluorescence showing immunoglobulin G to epithelial cell surface; and/or 3) detection of serum autoantibodies against desmogleins (1,3) or desmocollins (1,2,3), or both. Diagnosis requires one clinical, one pathologic, and one immunologic feature. We also report three new cases diagnosed at our institution to demonstrate the applicability of the suggested criteria.

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Section: Resultsmentioning

confidence: 99%

Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria

2019

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“…and therefore repeat biopsies are often required. 6 The aetiology of PH remains unclear. Disruption of keratinocyte intercellular adhesion has been suggested because of autoreactivity to desmogleins/desmocollins on indirect immunofluorescence.…”

Section: Discussionmentioning

confidence: 99%

A herpetiform eruption with unexpected immunofluorescence findings

Earp¹,

Mitchell²,

Biswas

2020

Clin Experimental Derm

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A 63-year-old man presented with an 8-week history of a progressive, pruritic eruption affecting his trunk and limbs. He had a history of ischaemic heart disease. There had been no recent change in medication, and the patient did not report any history of personal or family history of dermatological disease. Physical examination revealed clusters of erythematous papulovesicles, small de-roofed bullae and excoriated lesions distributed in a symmetrical herpetiform pattern over the trunk and extensor surfaces of the limbs (Fig. 1). There was no mucosal involvement. Routine laboratory tests including haematinics, thyroid function and an autoimmune screen were unremarkable. The patient underwent a diagnostic skin biopsy sampled from his lower back.

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“…They may show eosinophilic and neutrophilic spongiosis as well as intraepidermal pustules with or without acantholysis. Usually, more than one biopsy is needed to demonstrate acantholysis . Hence, the finding of epidermal intercellular IgG and C3 on direct immunofluorescence is extremely important for establishing an accurate diagnosis.…”

Section: Discussionmentioning

confidence: 99%

An annular pruritic eruption

et al. 2018

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A 36-year-old female patient presented with a 3-month history of a pruritic eruption on the trunk and extremities. She had no past medical history. Clinical examination revealed erythematous gyrate annular and edematous lesions with clusters of small vesicles arranged in a herpetiform pattern affecting the anterior and posterior skin of the trunk and the upper and lower limbs. There were multiple papules, vesicles, and crusted excoriations on an erythematous background on the trunk (Fig. 1a, b). Besides, there was no mucosal involvement. Histopathological findingsSkin biopsy revealed suprabasal bullae containing some acantholytic cells, neutrophils, and eosinophils. There was also eosinophilic spongiosis with intraepidermal vesicles filled with neutrophils and eosinophils ( Fig. 2a,b). Direct immunofluorescence of a perilesional skin biopsy revealed epidermal IgG in an intercellular pattern. Antibodies to desmoglein 1 but not desmoglein 3 were demonstrated by enzyme-linked immunosorbent assay. (a) (b) Figure 2 (a) Suprabasal bullae containing some acantholytic cells, neutrophils and eosinophils (Hematoxylin and eosin, 940); (b) Eosinophilic spongiosis with intraepidermal vesicles filled with neutrophils and eosinophils (Hematoxylin and eosin, 9100) (a) (b) Figure 1 (a) Erythematous gyrate annular and edematous lesions with clusters of small vesicles arranged in a herpetiform pattern affecting the lower limbs; (b) Multiple papules, vesicles, and crusted excoriations on an erythematous background on the trunk ª

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The importance of direct immunofluorescence in pemphigus herpetiformis diagnosis

Cited by 7 publications

References 9 publications

Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria

Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria

A herpetiform eruption with unexpected immunofluorescence findings

An annular pruritic eruption

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