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Table 1 Main findings of patients affected with PNP associated with CD reported in literature

From: Paraneoplastic pemphigus and Castleman’s disease: a case report and a revision of the literature

Reference

Number of patients

Case number

Age (years)

/sex

Personal history

Initial symptoms

Time to diagnosis

Mucosal manifestation

Skin manifestation

Systemic symptoms

Bronchiolitis obliterans

Index case

1

Index case

13/F

No

Mucosal (oral)

2 years

Oral

Conjunctival

Blistering eruption

No

No

Shu‐Ping Han et al. [7]

1

1

13/F

No

Mucosal (oral)

6 months

Oral

Lichen planus

No

Yes

Mimouni et al. [8]

1

2

9/M

No

Mucosal (Oral,

Conjunctival)

3 weeks

Oral

Conjunctival

Blistering eruption

Papular lichenoid rash

Dystrophic nails

Dyspnea

Yes

Choh NA et al. [9]

1

3

16/M

No

Mucosal (oral)

Skin

Low grade fever

1 month

Oral

Lichenoid rash

Fever

Yes

Daneshpazhooh M et al. [10]

1

4

10/F

Resection of cervical UCD (HV) 5 years before

Mucosal (oral, conjunctival)

Skin

2 months

Oral

Conjunctival

Blistering eruption

Papular lichenoid rash

Dystrophic nails

Fever

Dyspnea

Cough

Yes

Jindal T et al. [11]

1

5

14/F

No

Mucosal (oral)

5 months

Oral

No

No

Yes

Koch LH et al. [12]

1

6

14/F

No

Mucosal (Oral, Conjunctival, Genital)

3 weeks

Oral

Conjunctival

Genital

Blistering eruption

No

Yes

Jing L et al. [13]

1

7

16/F

No

Mucosal (oral)

3 months

Oral

Conjunctival

Genital

Blistering eruption

No

No

Hung IJ et al. [14]

1

8

14/M

No

Mucosal (oral)

Skin

Cough

1 month

Oral

Conjunctival

Genital

Papular rash

Fever

Cough

Yes

Lane JE et al. [15]

1

9

10/M

No

Mucosal (oral, conjunctival)

Skin

2 months

Oral

Conjunctival

Genital

Papular rash

Dystrophic nails

Fever

Cough Fatigue Constipation

Weight loss (no food intake)

Yes

Chin AC et al. [16]

1

10

14/M

No

Mucosal (Oral, Conjunctival, Genital)

3 months

Oral

Conjunctival

Genital

No

Dyspnea

Yes

Nousari HC et al. [17]

1

11

13/M

No

Mucosal (oral)

Not available

Oral

Genital

Blistering eruption

No

Yes

Lemon MA et al. [18]

1

12

13/M

No

Epistaxis

Mucosal (oral, conjunctival)

1.5 months

Oral

Conjunctival

Genital

Maculopapular eruption

Swollen nail folds

Nails shedding

Weight loss (no food intake)

No

Wang J et al. [19]

2

13

14

17/M

17/F

Not available

Not available

Mucosal (oral)

Mucosal (oral)

6 months

4 months

Oral

Conjunctival

Genital

Oral

Conjunctival

Genital

Erythema multiform

Lichen planus

Not available

Not available

Yes

Yes

Kumar S et al. [20]

1

15

15/M

No

Mucosal (oral)

Abdominal pain

3 months

Oral

No

Abdominal pain

Weight loss (no food intake)

No

Coulson IH et al. [21]

1

16

17/F

No

Mucosal (oral)

2 years

Oral

Conjunctival

Genital

Acral lichenoid eruption

Aseptic meningitis

Not available

Velasco et al. [22]

1

17

13/F

Not available

Mucosal (oral and conjunctival)

3 months

Oral

Conjunctival

No

Thyroid carcinoma

Yes

Sathishkumar et al. [23]

2

18

19

8/F

8/M

Not available

Not available

Mucosal

Mucosal

Not available

Not available

Oral

Conjunctival

Genital

Oral

Conjunctival

Pemphigus vulgaris

Lichen planus

Pemphigus vulgaris

Lichen planus

Papular rash

Not available

Not available

No

No

Reference

Case number

Form and Histological findings

Tumor localization

Tumor resection

Medication

outcome

Survival time

Index case

Index case

MCD (HV)

Retroperitoneal

No

Steroids

Rituximab

Siltuximab

Survive

Not available

Shu‐Ping Han et al. [7]

1

UCD (HV-SR)

Retroperitoneal (Pararenal)

Yes, complete

oral methylprednisolone

rituximab

tocilizumab

plasmapheresis

Immunoglobulin (2 g/kg)

Survive

12 months

Mimouni et al. [8]

2

UCD (MX)

Retroperitoneal

Yes, complete

Prednisone

Steroid

Radiotherapy

Rituximab

Daclizumab

Survive

Not available

Choh NA et al. [9]

3

UCD (HV)

Retroperitoneal

Yes, complete

Systemic and local steroids

Death (respiratory failure)

1 month

Daneshpazhooh M et al. [10]

4

UCD (HV)

Cervical

Yes, complete

Prednisone

Azathioprine

Death (respiratory failure)

2 months

Jindal T et al. [11]

5

UCD (HV)

Chest (paratracheal area)

Yes, complete

Prednisone

Death (respiratory failure)

4 months

Koch LH et al. [12]

6

UCD (HV)

Retroperitoneal

Yes, complete

Prednisone

Cyclophosphamide

Thalidomide

Tocilizumab

Survive

Not available

Jing L et al. [13]

7

UCD (HV)

Retroperitoneal

Yes, complete

Prednisone

Plasmapheresis

Survive

Not available

Hung IJ et al. [14]

8

UCD (HV)

Chest (paratracheal region)

Yes, complete

Steroids

Death (respiratory failure)

7 months

Lane JE et al. [15]

9

UCD (HV)

Retroperitoneal

Yes, partial

Prednisone

Immunoglobulin

Cyclosporine

Rituximab

Daclizumab

Radiotherapy

Death (respiratory failure)

15 months

Chin AC et al. [16]

10

UCD (HV)

Chest (Mediastinum)

Yes, complete

Prednisone

Azathioprine

Colchicine

Thalidomide

Embrel

Cyclosporine

Rapamycin

Plasmapheresis

Pirfenidone

Living-related bilateral lung-lobe transplantation

Survive

Not available

Nousari HC et al. [17]

11

UCD (HV)

Chest (Mediastinum)

Yes, complete

Prednisone

Cyclosporine

Plasmapheresis

Death (respiratory failure)

30 months

Lemon MA et al. [18]

12

UCD (HV)

Chest (Mediastinum)

Yes, complete

Prednisone

Cyclophosphamide

Plasmapheresis

Survive

Not available

Wang J et al. [19]

13

14

17/M UCD (HV)

17/F UCD (HV)

Retroperitoneal

Chest (Mediastinum)

Yes, complete

Yes, complete

Not available

Immunoglobulin

Not available

Not available

Not available

Not available

Kumar S et al. [20]

15

UCD

Extra peritoneal region of right hemipelvis

Yes, complete

Steroids

Survive

Not available

Coulson IH et al. [21]

16

UCD (MX)

Retroperitoneal

Yes, complete

Prednisone

Survive

Not available

Velasco et al. [22]

17

UCD (HV)

Chest (Mediastinum)

Yes, complete

Corticosteroids

Rituximab

Immunoglobulin

Lung transplantation

Death

34 months

Sathishkumar et al. [23]

18

19

8/F UCD (HV)

8/M UCD (HV)

Retroperitoneal

Cervical

Yes, complete

Yes, complete

Prednisone

Methotrexate

Prednisone

Survive

Survive

Not available

Not available

  1. F Female, M Male, UCD (HV-SR) Unicentric Castleman’s Disease (Hyaline-Vascular, Stroma Rich variant), UCD (MX) Unicentric Castleman’s Disease (Mixed variant)