Chapter 6 Malignancy in Relation to Autoimmune Blistering Dermatoses: Molecular and Clinical Aspects

1. Introduction

2. Autoimmune blistering dermatoses

2.1. ABD with autoimmunity to enzymes

2.2. ABD with autoimmunity to structural proteins

2.2.1. Anti-desmosomal autoimmunity circle / pemphigus group

2.2.2. Subepidermal autoimmune blistering dermatoses

2.2.3. Other, vaguely characterized, autoimmune blistering diseases

2.3. Malignancies associated with ABD

3. Endogenous factors leading to breakage of self-tolerance in malignancy

3.1. Development of paraneoplastic immunity

3.2. Genetic predisposition

3.3. The role of cytokines

3.4. Sex hormones

4. Exogenous factors: Epitope spreading and bystander effect

5. Molecular mechanisms: The possible connection between malignancies and ABD

5.1. Malignancy in relation to the autoimmunity in pemphigus group: The role of desmosomes components

5.2. Malignancy in relation to the autoimmunity in subepidermal blistering dermatoses: The role of the DEJ proteins

6. Case reports of MAABD

6.1. Anti-desmosomal autoimmunity circle / pemphigus group cases

6.1.1. Case 1 — Mucocutaneous pemphigus vulgaris / lung cancer

6.1.2. Case 2 — Mucocutaneous pemphigus vulgaris / oropharyngeal cancer

6.1.3. Case 3 — Pemphigus foliaceus / pseudomyxoma peritonei

6.1.4. Case 4 — Pemphigus foliaceus / squamous cell carcinoma of the thorax

6.1.5. Case 5 — Mucocutaneous pemphigus vulgaris shifting from mucosal dominant form / lower lip squamous cell carcinoma

6.1.6. Case 6 — Mucosal dominant pemphigus vulgaris / breast cancer

6.1.7. Case 7 — Pemphigus foliaceus / squamous cell carcinoma of external nose and upper lip

6.2. Subepidermal autoimmune blistering diseases

6.2.1. Case 8 — Bullous pemphigoid / colon cancer

6.2.2. Case 9 — Bullous pemphigoid / family history of renal cancer

6.2.3. Case 10 — Mucous membrane pemphigoid / hodgkin lymphoma

6.2.4. Case 11 — Bullous pemphigoid / renal cancer / prostate cancer

6.2.5. Case 12 — Bullous pemphigoid / lung cancer

6.2.6. Case 13 — Bullous pemphigoid / prostate cancer

6.2.7. Case 14 — Ocular mucous membrane pemphigoid / endometrial cancer

6.2.8. Case 15 — Bullous pemphigoid / breast cancer

6.3. Discussion on MAABD cases

7. Conclusion

Author details

References