Pemphigus erythematosus, also called Senear-Usher syndrome, is a rare superficial autoimmune blistering disorder with clinical, histopathologic, and serologic overlap between pemphigus foliaceus and lupus erythematosus. It is modeled here as a localized pemphigus spectrum disorder with seborrheic and photo-distributed cutaneous lesions, not as generic pemphigus or mucosal- dominant pemphigus vulgaris.
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Conditions with similar clinical presentations that must be differentiated from Pemphigus Erythematosus:
name: Pemphigus Erythematosus
creation_date: "2026-05-07T02:02:04Z"
updated_date: "2026-05-07T02:16:00Z"
category: Autoimmune
parents:
- Autoimmune Disease
- Skin Disease
disease_term:
preferred_term: Pemphigus Erythematosus
term:
id: MONDO:0019323
label: pemphigus erythematosus
description: >-
Pemphigus erythematosus, also called Senear-Usher syndrome, is a rare
superficial autoimmune blistering disorder with clinical, histopathologic, and
serologic overlap between pemphigus foliaceus and lupus erythematosus. It is
modeled here as a localized pemphigus spectrum disorder with seborrheic and
photo-distributed cutaneous lesions, not as generic pemphigus or mucosal-
dominant pemphigus vulgaris.
references:
- reference: DOI:10.5114/ada.2024.139233
title: Senear-Usher syndrome in a 5-year-old girl
found_in:
- Pemphigus_Erythematosus-deep-research-falcon.md
- reference: DOI:10.7759/cureus.59389
title: "Seborrheic Pemphigus: A Misunderstood Variant of Pemphigus Foliaceus"
found_in:
- Pemphigus_Erythematosus-deep-research-falcon.md
- reference: DOI:10.1111/cup.13992
title: >-
Pemphigus erythematosus: A case series from a tertiary academic center and
literature review
found_in:
- Pemphigus_Erythematosus-deep-research-falcon.md
- reference: DOI:10.1016/j.cger.2023.09.002
title: Diagnosis and Management of Bullous Disease
found_in:
- Pemphigus_Erythematosus-deep-research-falcon.md
- reference: DOI:10.5070/d3241037928
title: Unusually extensive scalp ulcerations manifested in pemphigus erythematosus
found_in:
- Pemphigus_Erythematosus-deep-research-falcon.md
- reference: DOI:10.7759/cureus.49268
title: A Case Report on Senear-Usher Syndrome
found_in:
- Pemphigus_Erythematosus-deep-research-falcon.md
pathophysiology:
- name: Desmosomal Autoantibody-Mediated Acantholysis
description: >-
Autoantibodies against desmosomal adhesion proteins in keratinocytes,
especially desmoglein 1 and sometimes desmoglein 3, disrupt epidermal
cell-cell adhesion. The resulting acantholysis produces superficial
intraepidermal fluid collections and fragile bullae or erosions.
cell_types:
- preferred_term: Keratinocyte
term:
id: CL:0000312
label: keratinocyte
biological_processes:
- preferred_term: Cell Adhesion
term:
id: GO:0007155
label: cell adhesion
modifier: DECREASED
- preferred_term: Immunoglobulin Production
term:
id: GO:0002377
label: immunoglobulin production
modifier: INCREASED
evidence:
- reference: PMID:38957191
reference_title: A Case Report on Senear-Usher Syndrome.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The autoantigens are desmoglein 3, desmoglein 1, and desmosomal adhesion
proteins in keratinocytes. When these bonds are disrupted, it causes
acantholysis of keratinocytes, leading to the fluid collection between
layers.
explanation: >-
This case report abstract directly links desmoglein/desmosomal
autoantigens to keratinocyte acantholysis and intraepidermal fluid
collection in Senear-Usher syndrome.
downstream:
- target: Dermal-Epidermal Junction Immune Complex and Complement Deposition
description: >-
Desmosomal autoimmunity co-occurs with lupus-like immunopathology at the
dermal-epidermal junction in pemphigus erythematosus.
causal_link_type: UNKNOWN
- name: Dermal-Epidermal Junction Immune Complex and Complement Deposition
description: >-
Pemphigus erythematosus includes lupus-band-like immunopathology, modeled as
immunoglobulin-associated immune complex and complement deposition at the
dermal-epidermal junction. This event distinguishes the disorder from plain
superficial pemphigus foliaceus and helps explain the lupus erythematosus
overlap.
biological_processes:
- preferred_term: Humoral immune response mediated by circulating immunoglobulin
term:
id: GO:0002455
label: humoral immune response mediated by circulating immunoglobulin
- preferred_term: Classical complement activation
term:
id: GO:0006958
label: complement activation, classical pathway
evidence:
- reference: PMID:33609053
reference_title: >-
Pemphigus erythematosus: A case series from a tertiary academic center and
literature review.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Pemphigus erythematosus (PE) is a rare autoimmune skin condition with
clinical, histopathological, and serological features that show overlap
between lupus erythematosus and pemphigus foliaceus.
explanation: >-
The case series and literature review defines the disorder by overlap
across clinical, histopathologic, and serologic dimensions.
downstream:
- target: Erythematous Scaly Plaques
description: >-
Lupus-like cutaneous immune deposition contributes to the erythematous,
scaly plaque phenotype in seborrheic or photo-distributed skin.
causal_link_type: INDIRECT_UNKNOWN_INTERMEDIATES
histopathology:
- name: Superficial Acantholysis
diagnostic: true
description: >-
Superficial or subcorneal acantholysis is the pemphigus foliaceus-like
microscopic component of pemphigus erythematosus and explains the shallow
blistering pattern.
evidence:
- reference: PMID:38957191
reference_title: A Case Report on Senear-Usher Syndrome.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
When these bonds are disrupted, it causes acantholysis of keratinocytes,
leading to the fluid collection between layers.
explanation: >-
The abstract supports acantholysis as a histopathologic correlate of
desmosomal disruption and intraepidermal fluid collection.
- reference: PMID:33609053
reference_title: >-
Pemphigus erythematosus: A case series from a tertiary academic center and
literature review.
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
Pemphigus erythematosus (PE) is a rare autoimmune skin condition with
clinical, histopathological, and serological features that show overlap
between lupus erythematosus and pemphigus foliaceus.
explanation: >-
The Hobbs abstract supports histopathology as a defining axis of the
pemphigus foliaceus overlap, although the abstract does not spell out each
microscopic criterion.
- name: Direct Immunofluorescence IgG/C3 Deposition Pattern
diagnostic: true
description: >-
Direct immunofluorescence is expected to show pemphigus-type intercellular
epidermal IgG/C3 staining together with lupus-band-like IgG and/or C3 along
the dermal-epidermal junction.
evidence:
- reference: PMID:33609053
reference_title: >-
Pemphigus erythematosus: A case series from a tertiary academic center and
literature review.
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
Clinical, histopathological, and serological features were consistent with
what has been reported in the literature
explanation: >-
The cached abstract supports the combined histopathologic and serologic
diagnostic axis; the detailed DIF pattern comes from the Falcon-summarized
full-text review and is modeled conservatively here.
phenotypes:
- name: Erythematous Scaly Plaques
category: Dermatologic
notes: >-
Well-demarcated erythematous, scaly, crusted, or hyperkeratotic plaques are
typical and often occur in seborrheic or photo-exposed areas such as the
malar face, scalp, chest, upper trunk, and elbows.
phenotype_term:
preferred_term: Erythematous scaly plaques
term:
id: HP:0010783
label: Erythema
evidence:
- reference: PMID:33609053
reference_title: >-
Pemphigus erythematosus: A case series from a tertiary academic center and
literature review.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
It typically presents with erythematous, scaly plaques and has a female
predominance.
explanation: >-
The literature review identifies erythematous scaly plaques as the typical
clinical presentation.
- reference: PMID:38957191
reference_title: A Case Report on Senear-Usher Syndrome.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
We report the case of a 21-year-old female presenting to us with multiple
hyperkeratotic plaques, mainly on the seborrheic areas, including the
face, chest, and elbows.
explanation: >-
This case report supports seborrheic-area plaques involving the face,
chest, and elbows.
- name: Superficial Cutaneous Blistering
category: Dermatologic
notes: >-
Fragile superficial bullae may rupture quickly, leaving crusting and scaling.
phenotype_term:
preferred_term: Superficial cutaneous blistering
term:
id: HP:0008066
label: Abnormal blistering of the skin
evidence:
- reference: PMID:38957191
reference_title: A Case Report on Senear-Usher Syndrome.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Hence, the patient will present clinically with small flaccid bullae with
crusting and scaling, mainly on the seborrheic areas.
explanation: >-
The abstract explicitly describes flaccid bullae with crusting and scaling
in seborrheic areas.
- name: Cutaneous Photosensitivity
category: Dermatologic
notes: >-
Photosensitivity and photo-distributed lesions are repeatedly discussed in
pemphigus erythematosus, but the exact frequency is not well established in
cache-backed abstracts.
phenotype_term:
preferred_term: Cutaneous photosensitivity
term:
id: HP:0000992
label: Cutaneous photosensitivity
- name: Uncommon Mucosal Involvement
category: Oral
notes: >-
Pemphigus erythematosus is primarily cutaneous; Falcon research summarized
low pooled oral involvement, so mucosal disease is noted here as uncommon
rather than modeled as a core high-frequency feature.
biochemical:
- name: ANA and anti-dsDNA positivity
presence: PRESENT
context: Lupus-overlap serologic finding reported in Senear-Usher syndrome.
evidence:
- reference: PMID:38957191
reference_title: A Case Report on Senear-Usher Syndrome.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
the diagnosis of pemphigus erythematosus associated with anti-double-stranded
deoxyribonucleic acid (anti-dSDNA) and anti-nuclear antibody (ANA)
positivity was made.
explanation: >-
The case report directly supports ANA and anti-dsDNA positivity as a
lupus-overlap serologic finding in this patient.
diagnosis:
- name: Integrated Clinicopathologic Diagnosis
description: >-
Diagnosis relies on correlating clinical distribution with histopathology,
direct immunofluorescence, and serology to distinguish pemphigus
erythematosus from pemphigus foliaceus, seborrheic dermatitis, and cutaneous
lupus erythematosus.
evidence:
- reference: PMID:33609053
reference_title: >-
Pemphigus erythematosus: A case series from a tertiary academic center and
literature review.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Clinical, histopathological, and serological features were consistent with
what has been reported in the literature
explanation: >-
The case series and literature review demonstrates that diagnosis is
grounded in concordant clinical, histopathologic, and serologic features.
- name: Desmoglein Autoantibody Serology
description: >-
Desmoglein autoantibody testing can support the pemphigus component of the
diagnosis, with desmoglein 1 expected to dominate in superficial pemphigus
patterns while some cases also show desmoglein 3 reactivity.
markers: anti-desmoglein 1, anti-desmoglein 3
evidence:
- reference: PMID:38957191
reference_title: A Case Report on Senear-Usher Syndrome.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The autoantigens are desmoglein 3, desmoglein 1, and desmosomal adhesion
proteins in keratinocytes.
explanation: >-
The abstract identifies desmoglein 1 and desmoglein 3 as relevant
autoantigens for Senear-Usher syndrome.
environmental:
- name: Ultraviolet Exposure
presence: PRESENT
description: >-
Ultraviolet exposure is treated as a plausible exacerbating factor based on
the photo-distributed pattern emphasized in Falcon research and the
MONDO/Orphanet definition, but this page does not assign a stronger causal
claim without an abstract-backed quote.
effect: Exacerbating factor for photo-distributed cutaneous lesions.
treatments:
- name: Topical Anti-Inflammatory Therapy and Photoprotection
description: >-
Localized disease may be approached with strict photoprotection and topical
anti-inflammatory therapy. This entry keeps the treatment general because
the cache-backed abstracts do not provide detailed PE-specific regimen
outcomes for topical corticosteroids.
treatment_term:
preferred_term: supportive care
term:
id: MAXO:0000950
label: supportive care
- name: Systemic Immunosuppression
description: >-
More extensive or refractory disease may require systemic immunosuppression,
including corticosteroids and steroid-sparing agents, selected according to
severity and overlap features.
treatment_term:
preferred_term: immunosuppressive therapy
term:
id: NCIT:C15261
label: Immunosuppressive Therapy
evidence:
- reference: PMID:38957191
reference_title: A Case Report on Senear-Usher Syndrome.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The patient was then managed using immunosuppressant therapy, and the
entire course has been detailed in this case report.
explanation: >-
This case report documents management of Senear-Usher syndrome using
immunosuppressant therapy.
- reference: PMID:38817480
reference_title: >-
Seborrheic Pemphigus: A Misunderstood Variant of Pemphigus Foliaceus.
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
we highlight the effectiveness of topical treatment in managing SP,
contrary to the systemic therapy often required for PE.
explanation: >-
This seborrheic pemphigus report contrasts SP with pemphigus
erythematosus, noting that systemic therapy is often required for PE.
differential_diagnoses:
- name: Pemphigus foliaceus
distinguishing_features:
- Pemphigus foliaceus lacks the defining lupus-like clinical or serologic overlap
expected in pemphigus erythematosus.
evidence:
- reference: PMID:33609053
reference_title: >-
Pemphigus erythematosus: A case series from a tertiary academic center and
literature review.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Pemphigus erythematosus (PE) is a rare autoimmune skin condition with
clinical, histopathological, and serological features that show overlap
between lupus erythematosus and pemphigus foliaceus.
explanation: >-
The overlap definition supports pemphigus foliaceus as a close diagnostic
comparator rather than an identical concept.
- name: Seborrheic pemphigus
distinguishing_features:
- Seborrheic pemphigus may resemble pemphigus erythematosus clinically but is
framed as a localized superficial pemphigus foliaceus variant without the
same lupus-overlap framing.
evidence:
- reference: PMID:38817480
reference_title: >-
Seborrheic Pemphigus: A Misunderstood Variant of Pemphigus Foliaceus.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Additionally, SP may be conceptually confused with pemphigus
erythematosus (PE) due to historical terminology and overlapping clinical
features.
explanation: >-
The abstract directly identifies confusion between seborrheic pemphigus
and pemphigus erythematosus.
- name: Cutaneous lupus erythematosus
distinguishing_features:
- Cutaneous lupus erythematosus can share malar or photo-distributed plaques,
but pemphigus erythematosus also has pemphigus-type acantholysis and
desmosomal autoantigens.
evidence:
- reference: PMID:33609053
reference_title: >-
Pemphigus erythematosus: A case series from a tertiary academic center and
literature review.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Pemphigus erythematosus (PE) is a rare autoimmune skin condition with
clinical, histopathological, and serological features that show overlap
between lupus erythematosus and pemphigus foliaceus.
explanation: >-
The overlap definition supports lupus erythematosus as a differential
diagnosis that must be separated from the pemphigus component.
Pemphigus erythematosus is a rare autoimmune blistering disorder with overlapping clinical, histopathologic, and immunologic features of pemphigus foliaceus and lupus erythematosus, often presenting as erythematous, scaly/crusted plaques and/or superficial flaccid blisters on seborrheic and photoexposed sites (especially malar face/scalp/upper trunk). (hobbs2021pemphiguserythematosusa pages 1-2, onalajaunderwood2024diagnosisandmanagement pages 1-3)
A central clinicopathologic concept is that PE demonstrates pemphigus-type intraepidermal acantholysis plus a lupus-band–like direct immunofluorescence pattern along the dermal–epidermal junction (DEJ). (chandan2018unusuallyextensivescalp pages 4-5, hobbs2021pemphiguserythematosusa pages 1-2)
The disease-specific information in this report is derived mainly from aggregated disease-level synthesis (a tertiary-center case series + literature review) and recent case reports/reviews rather than EHR-scale datasets. (hobbs2021pemphiguserythematosusa pages 1-2, arasiewicz2024senearushersyndromein pages 1-3, jangid2023acasereport pages 1-4)
PE is caused by an autoimmune response against desmosomal adhesion molecules (most prominently desmoglein-1), leading to impaired keratinocyte–keratinocyte adhesion (acantholysis) and superficial intraepidermal blistering. (onalajaunderwood2024diagnosisandmanagement pages 1-3, chandan2018unusuallyextensivescalp pages 1-4)
Genetic (susceptibility): A genetic predisposition via HLA haplotypes has been suggested; a pediatric PE report mentions HLA haplotypes including A10/A26 and DRW6 in the context of susceptibility. (arasiewicz2024senearushersyndromein pages 1-3)
Environmental (exacerbating/triggering): - Ultraviolet (UV) exposure/sunlight is repeatedly described as an exacerbating factor; a PE case report review notes UV can contribute mechanistically (including desmoglein-1 ectodomain cleavage and immune deposition at the DEJ in UV-exposed skin). (chandan2018unusuallyextensivescalp pages 4-5, onalajaunderwood2024diagnosisandmanagement pages 1-3)
Medication triggers (class effect for pemphigus; PE-specific evidence limited): A 2024 bullous disease review lists multiple medications associated with pemphigus (e.g., penicillamine, captopril, propranolol) while discussing pemphigus foliaceus and variants including PE, supporting consideration of drug triggers in susceptible individuals, though PE-specific causal attribution is not established in the retrieved PE-focused data. (onalajaunderwood2024diagnosisandmanagement pages 3-7)
No protective genetic or environmental factors were identified in the retrieved evidence set. (hobbs2021pemphiguserythematosusa pages 1-2)
A plausible gene–environment interaction is HLA-associated susceptibility combined with UV exposure as a clinical and mechanistic aggravator, but direct studies in PE were not available in the retrieved evidence set. (arasiewicz2024senearushersyndromein pages 1-3, chandan2018unusuallyextensivescalp pages 4-5)
Across a tertiary-center case series and literature review (87 literature cases summarized), common anatomic distributions included trunk (62%), face (51.7%), extremities (39%), scalp (35.6%). (hobbs2021pemphiguserythematosusa pages 2-5)
Mucosal involvement is uncommon; pooled oral involvement was ~9.2%. (hobbs2021pemphiguserythematosusa pages 2-5)
Common morphologies include erythematous scaly/crusted plaques, superficial erosions, and fragile/flaccid blisters that rupture with crusting. (jangid2023acasereport pages 1-4, hobbs2021pemphiguserythematosusa pages 10-11)
Course: A 2024 review characterizes PE as generally chronic and localized to malar and other seborrheic areas. (onalajaunderwood2024diagnosisandmanagement pages 3-7)
(These are ontology suggestions; the retrieved articles do not provide HPO codes.) - Erythematous scaly plaques / erythema: HP:0020116 (Erythema) (suggested) (hobbs2021pemphiguserythematosusa pages 2-5) - Blistering: HP:0030057 (Blistering of the skin) (suggested) (jangid2023acasereport pages 1-4) - Skin erosions/crusting: HP:0100717 (Skin erosion); HP:0200042 (Crusting of skin lesions) (suggested) (arasiewicz2024senearushersyndromein pages 1-3, jangid2023acasereport pages 1-4) - Photosensitivity: HP:0000992 (Photosensitivity) (suggested) (hobbs2021pemphiguserythematosusa pages 10-11, onalajaunderwood2024diagnosisandmanagement pages 3-7) - Positive Nikolsky sign (clinical sign; HPO mapping may vary): (suggested) (onalajaunderwood2024diagnosisandmanagement pages 1-3)
Disease-specific QoL instrument data were not identified for PE in the retrieved evidence set; however, PE is described as causing symptomatic, visible lesions and is discussed as requiring integrated assessment in case-report literature. (jangid2023acasereport pages 6-7)
PE is not a monogenic disorder in the retrieved evidence set; no single causal gene/variant is established. Evidence supports HLA-associated susceptibility (immunogenetic predisposition) rather than Mendelian inheritance. (arasiewicz2024senearushersyndromein pages 1-3)
Not applicable based on retrieved evidence; PE evidence focuses on autoantibodies and HLA associations rather than pathogenic variants. (hobbs2021pemphiguserythematosusa pages 1-2)
No specific infectious trigger was identified in the retrieved evidence set for PE. (hobbs2021pemphiguserythematosusa pages 1-2)
1) Autoantibody production against desmosomal proteins (especially DSG1, sometimes DSG3) occurs in susceptible individuals. (onalajaunderwood2024diagnosisandmanagement pages 1-3, jangid2023acasereport pages 1-4) 2) Binding of autoantibodies to keratinocyte adhesion molecules leads to loss of intercellular adhesion (acantholysis), producing superficial intraepidermal blistering and erosions. (chandan2018unusuallyextensivescalp pages 1-4, hobbs2021pemphiguserythematosusa pages 2-5) 3) PE uniquely shows lupus-like immunopathology, commonly featuring DEJ immune deposits (IgG/C3) on direct immunofluorescence (“lupus-band”-like), especially in photoexposed skin. (chandan2018unusuallyextensivescalp pages 4-5, hobbs2021pemphiguserythematosusa pages 10-11) 4) Clinically this yields erythematous scaly/crusted plaques and superficial erosions in seborrheic/photoexposed distributions with relatively infrequent mucosal involvement. (hobbs2021pemphiguserythematosusa pages 2-5, onalajaunderwood2024diagnosisandmanagement pages 3-7)
(These are ontology suggestions; the retrieved articles do not provide GO/CL codes.) - GO biological processes: keratinocyte cell–cell adhesion; complement activation; humoral immune response; inflammatory response in skin. (suggested) (hobbs2021pemphiguserythematosusa pages 10-11, onalajaunderwood2024diagnosisandmanagement pages 1-3) - CL cell types: keratinocyte; B cell/plasma cell (autoantibody production). (suggested) (onalajaunderwood2024diagnosisandmanagement pages 1-3)
No transcriptomic/proteomic/metabolomic PE-specific profiling was identified in the retrieved evidence set. (hobbs2021pemphiguserythematosusa pages 1-2)
In the Hobbs literature review, age ranged from 5–84 years (mean ~51.77 years, median 57), supporting predominantly adult onset but possible pediatric presentation. (hobbs2021pemphiguserythematosusa pages 2-5)
PE is described as generally chronic; disease duration in reviewed literature ranged 1–60 months (mean 18.2 months; median 11 months). (onalajaunderwood2024diagnosisandmanagement pages 3-7, hobbs2021pemphiguserythematosusa pages 5-5)
Epidemiology is not well established; a recent pediatric report states epidemiology is unknown and cites a prevalence estimate of 4.4 cases/million. (arasiewicz2024senearushersyndromein pages 1-3)
No Mendelian inheritance is supported; risk appears multifactorial with HLA-linked susceptibility reported. (arasiewicz2024senearushersyndromein pages 1-3)
In Hobbs et al., diagnostic criteria required (i) pemphigus foliaceus-like histopathology plus (ii) DIF evidence of epidermal intercellular IgG and (iii) IgG and/or C3 along the DEJ. (hobbs2021pemphiguserythematosusa pages 1-2)
A 2024 review emphasizes that when a blistering disorder is suspected, clinicians should obtain a lesional biopsy for H&E and a perilesional biopsy for DIF, with the DIF specimen taken from immediately adjacent uninvolved skin and sent in Michel medium. (onalajaunderwood2024diagnosisandmanagement pages 1-3)
Typical histology includes superficial/subcorneal or intraepidermal acantholysis, often with dyskeratosis and lymphocytic dermal inflammation. (hobbs2021pemphiguserythematosusa pages 2-5, hobbs2021pemphiguserythematosusa pages 10-11)
DIF typically demonstrates intercellular space IgG/C3 deposition plus a linear/granular DEJ (lupus-band–like) IgG/C3 pattern. (chandan2018unusuallyextensivescalp pages 4-5, hobbs2021pemphiguserythematosusa pages 10-11)
PE is repeatedly discussed in diagnostic context as overlapping lupus erythematosus and pemphigus foliaceus and may be confused with seborrheic dermatitis and other seborrheic eruptions; accurate diagnosis hinges on histology and immunopathology. (espadas2024seborrheicpemphigusa pages 1-3, hobbs2021pemphiguserythematosusa pages 1-2)
PE is often described as more benign/easier to manage than pemphigus vulgaris in case literature, but robust survival or long-term morbidity statistics were not available from the retrieved evidence set. (chandan2018unusuallyextensivescalp pages 4-5)
Complications reported/mentioned include secondary infection and severe erythroderma in individual cases. (arasiewicz2024senearushersyndromein pages 1-3)
A 2024 review provides practical management framing: “Localized disease can typically be managed by potent topical steroids”, while more extensive disease may require systemic therapy including prednisone and steroid-sparing agents such as azathioprine, mycophenolate mofetil, rituximab, or dapsone. (onalajaunderwood2024diagnosisandmanagement pages 3-7)
Case-based evidence includes use of hydroxychloroquine + systemic prednisone + dapsone with clinical response in pediatric PE, supporting real-world use of antimalarial and anti-neutrophil/anti-inflammatory adjunct approaches in overlap-like presentations. (arasiewicz2024senearushersyndromein pages 1-3)
Evidence-based PE-specific prevention strategies were not identified beyond avoidance of known aggravating factors, especially sunlight/UV exposure given repeated associations with photosensitivity and worsening. (onalajaunderwood2024diagnosisandmanagement pages 3-7, chandan2018unusuallyextensivescalp pages 4-5)
No natural disease reports in non-human species specific to pemphigus erythematosus were identified in the retrieved evidence set. (hobbs2021pemphiguserythematosusa pages 1-2)
No PE-specific model organism systems were identified in the retrieved evidence set. Mechanistic work in pemphigus broadly often uses antibody transfer/ex vivo skin systems, but such details were not retrieved here for PE specifically. (hobbs2021pemphiguserythematosusa pages 1-2)
1) Clinical recognition and pediatric presentations: Recent case reports highlight that PE can occur in children (e.g., a 2024 report in a 5-year-old), reinforcing that the age range includes pediatric-onset disease even if adult-onset is more typical. (arasiewicz2024senearushersyndromein pages 1-3)
2) Refined diagnostic workflows in bullous disease practice: A 2024 expert review reinforces practical details for DIF sampling (perilesional biopsy, Michel medium) and emphasizes ELISA availability for desmoglein autoantibodies, reflecting continuing standardization and accessibility of serologic testing in clinical practice. (onalajaunderwood2024diagnosisandmanagement pages 1-3)
3) Therapeutic positioning: A 2024 review frames PE management within modern pemphigus care, including topical steroids for localized disease and systemic immunosuppression and biologics (including rituximab) for more extensive disease, consistent with current real-world practice patterns. (onalajaunderwood2024diagnosisandmanagement pages 3-7)
| Domain | Key finding | Supporting citations |
|---|---|---|
| Definition / disease concept | Pemphigus erythematosus (PE), also called Senear–Usher syndrome, is a rare autoimmune blistering disorder generally regarded as a localized variant of pemphigus foliaceus with clinical, histologic, and immunologic overlap with cutaneous lupus erythematosus. | (arasiewicz2024senearushersyndromein pages 1-3, jangid2023acasereport pages 1-4, hobbs2021pemphiguserythematosusa pages 1-2, onalajaunderwood2024diagnosisandmanagement pages 3-7) |
| Typical clinical distribution | Lesions are usually erythematous, scaly or crusted plaques / superficial flaccid bullae in seborrheic and photoexposed areas, especially the malar face, scalp, upper trunk, and back; face involvement was 80% in one center series and 51.7% in the pooled literature review. | (arasiewicz2024senearushersyndromein pages 1-3, hobbs2021pemphiguserythematosusa pages 1-2, hobbs2021pemphiguserythematosusa pages 2-5, onalajaunderwood2024diagnosisandmanagement pages 3-7, onalajaunderwood2024diagnosisandmanagement pages 1-3) |
| Mucosal involvement | Mucosal disease is uncommon; oral/pharyngeal/vulvar mucosa are often spared, and pooled oral involvement was ~9.2% in Hobbs et al. | (arasiewicz2024senearushersyndromein pages 1-3, hobbs2021pemphiguserythematosusa pages 2-5, onalajaunderwood2024diagnosisandmanagement pages 1-3) |
| Key autoantigens | Main autoantigen is desmoglein 1 (Dsg1); some reports also describe Dsg3 and other desmosomal adhesion proteins / plakins. PF-variant pathogenesis is linked to IgG4 against Dsg1. | (arasiewicz2024senearushersyndromein pages 1-3, jangid2023acasereport pages 1-4, chandan2018unusuallyextensivescalp pages 1-4, onalajaunderwood2024diagnosisandmanagement pages 1-3) |
| Histology | Characteristic pathology is superficial/subcorneal or intraepidermal acantholysis, often with dyskeratosis; inflammatory infiltrates may be lymphocytic or mixed, with papillary dermal edema/perivascular infiltrates in some cases. | (arasiewicz2024senearushersyndromein pages 1-3, chandan2018unusuallyextensivescalp pages 1-4, hobbs2021pemphiguserythematosusa pages 2-5, hobbs2021pemphiguserythematosusa pages 10-11) |
| Direct immunofluorescence (DIF) | Classic DIF shows intercellular epidermal IgG/C3 deposition plus IgG and/or C3 along the dermal–epidermal junction ("lupus-band"-like pattern). PE diagnostic criteria in Hobbs et al. required PF histology plus these DIF findings. | (chandan2018unusuallyextensivescalp pages 4-5, hobbs2021pemphiguserythematosusa pages 1-2, hobbs2021pemphiguserythematosusa pages 10-11, jangid2023acasereport pages 4-6) |
| ANA / lupus serology | ANA positivity is variably reported, commonly cited as ~30%–80% of cases; in the Hobbs center series ANA was elevated in 1/5 patients (20%). Additional lupus-associated serologies reported in case literature include anti-dsDNA, anti-Ro/SSA, anti-Sm, and anti-RNP. | (jangid2023acasereport pages 1-4, hobbs2021pemphiguserythematosusa pages 1-2, hobbs2021pemphiguserythematosusa pages 2-5) |
| Epidemiology | Epidemiology is poorly defined. One cited prevalence estimate is 4.4 cases/million. In Hobbs et al., the literature review found mean age 51.77 years (median 57; range 5–84) and female:male ratio 45:36; the center series had mean age 43.8 years and 3/5 female. Oral involvement in the pooled review was ~9.2%. | (arasiewicz2024senearushersyndromein pages 1-3, hobbs2021pemphiguserythematosusa pages 1-2, hobbs2021pemphiguserythematosusa pages 2-5) |
| Triggers / exacerbating factors | Ultraviolet exposure / sunlight is a recognized exacerbating factor; photosensitivity is a recurring clinical feature and UV may contribute to DEJ immune deposition in PE. Medication-associated pemphigus triggers are also discussed in broader reviews, though PE-specific evidence is limited. | (chandan2018unusuallyextensivescalp pages 4-5, hobbs2021pemphiguserythematosusa pages 10-11, onalajaunderwood2024diagnosisandmanagement pages 3-7, onalajaunderwood2024diagnosisandmanagement pages 1-3) |
| Common treatments | Localized disease can often be treated with potent topical corticosteroids. More extensive disease is treated with systemic corticosteroids plus steroid-sparing agents such as azathioprine, mycophenolate mofetil, dapsone, methotrexate, cyclophosphamide, or rituximab; hydroxychloroquine has also been used in overlap-style cases. | (arasiewicz2024senearushersyndromein pages 1-3, chandan2018unusuallyextensivescalp pages 4-5, hobbs2021pemphiguserythematosusa pages 10-11, onalajaunderwood2024diagnosisandmanagement pages 3-7, jangid2023acasereport pages 1-4) |
Table: This table condenses the main disease-characteristics evidence for pemphigus erythematosus (Senear–Usher syndrome), including defining overlap features, phenotype, diagnostics, epidemiology, triggers, and treatment. It is useful as a quick-reference summary mapped directly to the gathered citation IDs.
References
(arasiewicz2024senearushersyndromein pages 1-3): Hubert Arasiewicz, Joanna Czuwara, Michał Dec, and Lilianna Leśniak-Jakubiec. Senear-usher syndrome in a 5-year-old girl. Advances in Dermatology and Allergology/Postȩpy Dermatologii i Alergologii, 41:242-244, Apr 2024. URL: https://doi.org/10.5114/ada.2024.139233, doi:10.5114/ada.2024.139233. This article has 1 citations.
(espadas2024seborrheicpemphigusa pages 1-3): Diana Gallegos Espadas, Arely Gissell Ramirez Cibrian, and Jesús Iván Martínez-Ortega. Seborrheic pemphigus: a misunderstood variant of pemphigus foliaceus. Cureus, Apr 2024. URL: https://doi.org/10.7759/cureus.59389, doi:10.7759/cureus.59389. This article has 2 citations.
(hobbs2021pemphiguserythematosusa pages 1-2): Landon K. Hobbs, Mary‐Margaret B. Noland, Shyam S. Raghavan, and Alejandro A. Gru. Pemphigus erythematosus: a case series from a tertiary academic center and literature review. Journal of Cutaneous Pathology, 48:1038-1050, Mar 2021. URL: https://doi.org/10.1111/cup.13992, doi:10.1111/cup.13992. This article has 12 citations and is from a peer-reviewed journal.
(onalajaunderwood2024diagnosisandmanagement pages 3-7): Amanda A. Onalaja-Underwood, Maria Yadira Hurley, and Olayemi Sokumbi. Diagnosis and management of bullous disease. Clinics in Geriatric Medicine, 40:37-74, Feb 2024. URL: https://doi.org/10.1016/j.cger.2023.09.002, doi:10.1016/j.cger.2023.09.002. This article has 4 citations and is from a peer-reviewed journal.
(onalajaunderwood2024diagnosisandmanagement pages 1-3): Amanda A. Onalaja-Underwood, Maria Yadira Hurley, and Olayemi Sokumbi. Diagnosis and management of bullous disease. Clinics in Geriatric Medicine, 40:37-74, Feb 2024. URL: https://doi.org/10.1016/j.cger.2023.09.002, doi:10.1016/j.cger.2023.09.002. This article has 4 citations and is from a peer-reviewed journal.
(chandan2018unusuallyextensivescalp pages 4-5): Neha Chandan, Eden P Lake, and Lawrence S Chan. Unusually extensive scalp ulcerations manifested in pemphigus erythematosus. Dermatology Online Journal, Feb 2018. URL: https://doi.org/10.5070/d3241037928, doi:10.5070/d3241037928. This article has 7 citations and is from a peer-reviewed journal.
(jangid2023acasereport pages 1-4): Shivani D Jangid, Bhushan Madke, Adarshlata Singh, Drishti M Bhatt, and Arshiya Khan. A case report on senear-usher syndrome. Cureus, Nov 2023. URL: https://doi.org/10.7759/cureus.49268, doi:10.7759/cureus.49268. This article has 1 citations.
(chandan2018unusuallyextensivescalp pages 1-4): Neha Chandan, Eden P Lake, and Lawrence S Chan. Unusually extensive scalp ulcerations manifested in pemphigus erythematosus. Dermatology Online Journal, Feb 2018. URL: https://doi.org/10.5070/d3241037928, doi:10.5070/d3241037928. This article has 7 citations and is from a peer-reviewed journal.
(hobbs2021pemphiguserythematosusa pages 2-5): Landon K. Hobbs, Mary‐Margaret B. Noland, Shyam S. Raghavan, and Alejandro A. Gru. Pemphigus erythematosus: a case series from a tertiary academic center and literature review. Journal of Cutaneous Pathology, 48:1038-1050, Mar 2021. URL: https://doi.org/10.1111/cup.13992, doi:10.1111/cup.13992. This article has 12 citations and is from a peer-reviewed journal.
(hobbs2021pemphiguserythematosusa pages 10-11): Landon K. Hobbs, Mary‐Margaret B. Noland, Shyam S. Raghavan, and Alejandro A. Gru. Pemphigus erythematosus: a case series from a tertiary academic center and literature review. Journal of Cutaneous Pathology, 48:1038-1050, Mar 2021. URL: https://doi.org/10.1111/cup.13992, doi:10.1111/cup.13992. This article has 12 citations and is from a peer-reviewed journal.
(jangid2023acasereport pages 6-7): Shivani D Jangid, Bhushan Madke, Adarshlata Singh, Drishti M Bhatt, and Arshiya Khan. A case report on senear-usher syndrome. Cureus, Nov 2023. URL: https://doi.org/10.7759/cureus.49268, doi:10.7759/cureus.49268. This article has 1 citations.
(hobbs2021pemphiguserythematosusa pages 5-5): Landon K. Hobbs, Mary‐Margaret B. Noland, Shyam S. Raghavan, and Alejandro A. Gru. Pemphigus erythematosus: a case series from a tertiary academic center and literature review. Journal of Cutaneous Pathology, 48:1038-1050, Mar 2021. URL: https://doi.org/10.1111/cup.13992, doi:10.1111/cup.13992. This article has 12 citations and is from a peer-reviewed journal.
(jangid2023acasereport pages 4-6): Shivani D Jangid, Bhushan Madke, Adarshlata Singh, Drishti M Bhatt, and Arshiya Khan. A case report on senear-usher syndrome. Cureus, Nov 2023. URL: https://doi.org/10.7759/cureus.49268, doi:10.7759/cureus.49268. This article has 1 citations.