Histologic characterization of cellular infiltration in autoimmune subepidermal bullous diseases in a tertiary hospital in Saudi Arabia

BinJadeed, Hessah; Alyousef, Alanoud M; Alsaif, Fahad; Alhumidi, Ahmed; Alotaibi, Homaid

doi:10.2147/ccid.s158388

Cited by 10 publications

(11 citation statements)

References 54 publications

(52 reference statements)

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“…In a comparative study about histopathology of subepidermal bullous diseases, dermal eosinophilic infiltration was observed in 80.0% of BP patients 12 . In our study, dermal eosinophil infiltration was detected in 98.3% of the patients.…”

Section: Discussionsupporting

confidence: 56%

Tissue eosinophil levels as a marker of disease severity in bullous pemphigoid

Karaali¹,

Aksu²,

Cin³

et al. 2021

Aust J Dermatology

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Background: Eosinophils play an important role in bullous pemphigoid (BP) pathogenesis. Although tissue infiltration with eosinophils has been known for a long time, there is a lack of knowledge about the relationship between tissue eosinophil levels and disease severity and clinical characteristics of the patients.Methods: Fifty-nine patients diagnosed with BP between January 2008 and December 2018 were reviewed. Haematoxylin-Eosin (H&E)-stained preparations were re-evaluated in terms of tissue eosinophil levels. For disease severity, Bullous Pemphigoid Disease Area Index (BPDAI) was used. The relationship between tissue eosinophil levels and disease severity and clinical features were evaluated.Results: Erosion/blister and urticaria/erythema BPDAI scores were higher in the group with high tissue eosinophil level than the group with low tissue eosinophil level. Tissue and peripheral blood eosinophil count were correlated with total urticaria/erythema BPDAI scores. There was no correlation between blood and tissue eosinophil count. The mortality rate was 64.7% vs 44.0% in the high vs low tissue eosinophil groups. Tissue eosinophil levels were high in patients with BP accompanying neurological disease.Conclusions: Tissue eosinophil count and peripheral blood eosinophil count were correlated with disease severity in BP. Tissue eosinophil levels were also high in patients with BP accompanying neurological disease.

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Section: Discussionsupporting

confidence: 56%

Tissue eosinophil levels as a marker of disease severity in bullous pemphigoid

Karaali¹,

Aksu²,

Cin³

et al. 2021

Aust J Dermatology

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“…Our study indicates that lymphocytes infiltrated lesional skin specimens of all study participants, thus outreaching the observation of a recent smallscale histological study that reported dermal perivascular lymphocytic infiltrate in 32.0% of patients with BP. 8 The same study, though, disclosed that all patients with pemphigoid gestations showed lymphocytic infiltration. 8 Our study revealed that patients with lymphocyte-predominant inflammatory infiltrate were typified by a more severe phenotype, both on the erosive and erythematous level.…”

Section: Discussionmentioning

confidence: 90%

“…8 The same study, though, disclosed that all patients with pemphigoid gestations showed lymphocytic infiltration. 8 Our study revealed that patients with lymphocyte-predominant inflammatory infiltrate were typified by a more severe phenotype, both on the erosive and erythematous level. Whilst this association has not been reported as yet, it can be conceived in light that BP is an antibody-mediated autoimmune disease that relies on autoreactive T-helper (Th) cells which, in turn, promote the activation of autoreactive B cells.…”

Section: Discussionmentioning

confidence: 90%

“…7 Histologic sections of BP classically demonstrate a subepidermal blister with a variable degree of inflammatory infiltrate comprised of lymphocytes, neutrophils and characteristically eosinophils. 8 In the prodromal, non-bullous phase of the disease, urticarial lesions may present with eosinophilic spongiosis, without a frank subepidermal clefting. 9 Whilst direct immunofluorescence (DIF) and immunoserological assays embody an indispensable role in establishing the diagnosis of BP, revealing the histological features and determining the types of inflammatory cells infiltrating lesional skin are still of value to differentiate between different SAIBDs.…”

Section: Introductionmentioning

confidence: 99%

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The impact of lesional inflammatory cellular infiltrate on the phenotype of bullous pemphigoid

Ständer

Hammers

Vorobyev

et al. 2021

Acad Dermatol Venereol

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Background The influence of cutaneous cellular infiltration on the phenotype of bullous pemphigoid (BP) remains to be established.Objectives To evaluate the main histopathological characteristics of patients with BP and to assess the association between the composition of lesional inflammatory infiltrate and the various clinical, immunological and immunopathological aspects of the disease. MethodsRetrospective study encompassing patients diagnosed with BP throughout the years 2009-2020 in a specialized tertiary referral centre. ResultsThe study encompassed 136 patients with BP, of whom 27 (19.9%) demonstrated a cell-poor inflammatory infiltrate in lesional skin specimens. Overall, 78 (57.4%), 71 (52.2%) and 5 (3.7%) specimens were found to include eosinophil-predominant, lymphocyte-predominant and neutrophil-predominant inflammatory infiltrates, respectively.Relative to the remaining patients with BP, those with an eosinophil-predominant inflammatory infiltrate had higher (90.8% vs. 77.2%; P = 0.030) whilst those with a cell-poor inflammatory infiltrate lower (70.3% vs. 88.7%; P = 0.017) seropositivity of anti-BP180 NC16A IgG. The latter subgroup presented with higher prevalence of mucosal involvement (25.9% vs. 8.3%; P = 0.011) and a non-inflammatory clinical phenotype (50.0% vs. 17.1%; P = 0.041). Patients with lymphocyte-predominant inflammatory infiltrate manifested with higher severity BPDAI scores and a lower frequency of the non-inflammatory subtype (11.1% vs. 36.4%; P = 0.035), whilst those with a neutrophilic infiltrate presented with lower mean (SD) levels of anti-BP180 NC16A IgG [269.3 (227.6) vs. 722.7 (1499.6) U/mL; P = 0.003].Conclusions Eosinophil-predominance and high cellularity in the lesional inflammatory infiltrate of BP skin are associated with increased seropositivity of anti-BP180 NC16A IgG. Lymphocyte-predominant infiltrates predict a more severe phenotype, pointing towards a pathogenic role of autoreactive lymphocytes.

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“…Histology of blistered skin in pemphigoid diseases normally shows superficial and mid-dermis perivascular inflammation infiltrated by lymphocytes, neutrophils, eosinophils, mast cells, and other immune cells, with the relative abundance and contribution depending on each disease (1, 12). The hallmark of BP lesions is eosinophil infiltration, whilst MMPh and classical variant EBA lesions exhibit minimal inflammation compared to the other pemphigoid diseases (12–14). Direct immunofluorescent microscopy of the patient skin is used diagnostically to visualize the deposition of immunoglobulins and/or complements along the basement membrane zone (1, 9, 11).…”

Section: Characteristics Of Pemphigoid Diseasesmentioning

confidence: 99%

Proteases in Pemphigoid Diseases

et al. 2019

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Pemphigoid diseases are a subgroup of autoimmune skin diseases characterized by widespread tense blisters. Standard of care typically involves immunosuppressive treatments, which may be insufficient and are often associated with significant adverse events. As such, a deeper understanding of the pathomechanism(s) of pemphigoid diseases is necessary in order to identify improved therapeutic approaches. A major initiator of pemphigoid diseases is the accumulation of autoantibodies against proteins at the dermal-epidermal junction (DEJ), followed by protease activation at the lesion. The contribution of proteases to pemphigoid disease pathogenesis has been investigated using a combination of in vitro and in vivo models. These studies suggest proteolytic degradation of anchoring proteins proximal to the DEJ is crucial for dermal-epidermal separation and blister formation. In addition, proteases can also augment inflammation, expose autoantigenic cryptic epitopes, and/or provoke autoantigen spreading, which are all important in pemphigoid disease pathology. The present review summarizes and critically evaluates the current understanding with respect to the role of proteases in pemphigoid diseases.

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Histologic characterization of cellular infiltration in autoimmune subepidermal bullous diseases in a tertiary hospital in Saudi Arabia

Cited by 10 publications

References 54 publications

Tissue eosinophil levels as a marker of disease severity in bullous pemphigoid

Tissue eosinophil levels as a marker of disease severity in bullous pemphigoid

The impact of lesional inflammatory cellular infiltrate on the phenotype of bullous pemphigoid

Proteases in Pemphigoid Diseases

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