Paraneoplastic syndromes (overview) L83.x

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 18.12.2020

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Synonym(s)

Cutaneous paraneoplasia; Paraneoplasia; Paraneoplastic erythema; Paraneoplastic syndrome

History
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Denny-Brown, 1948

Definition
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Non-metastatic symptoms based on humoral remote action of visceral malignancies, which may subside after tumour removal. See Table 1: Previous and now obsolete definitions suggested specificity of skin symptoms for a particular tumour. This thesis is not correct. There is a parallelism between skin symptoms and tumour in that skin symptoms may improve or disappear completely after treatment or removal of the tumour.

Classification
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Known are 5 obligate paraneoplasias and numerous facultative paraneoplasias. The latter play a much greater role in clinical routine than the rare obligate paraneoplasias. A further classification can be made according to:can be made.

Etiopathogenesis
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The possible causes include:
  • Ectopically formed hormones and hormone-like polypeptides.
  • Tumour-specific humoral or cell-mediated immunological reactions.
  • Tumor-induced non-specific immune reactions.
  • Production of growth factors by the tumor.
  • Prostaglandins and others.

Tables
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Skin diseases and monitorial indices of the skin in malignant tumors. Paraneoplastic dermatoses

Tumor frequency

Tumor type

Obligate paraneoplastic dermatoses

Acanthosis nigricans maligna

80-100%

85% tumours of the gastrointestinal tract (apudomas?)

Birt-Hogg-Dubé syndrome

unexplained

renal cell carcinoma, colon carcinoma

Keratosis palmoplantaris with esophageal carcinoma (Howell-Evans syndrome)

Esophageal carcinoma

Cowden syndrome

unexplained

Thyroid carcinoma, colon carcinoma

Hereditary leiomyomatosis renal cell carcinoma syndrome

100%

Renal cell carcinoma

Erythema gyratum repens (Gammel)

~ 100%

all tumors

Erythema necroticans migrans

100%

Glucagonoma

Acrokeratosis, paraneoplastic(Bazex)

100%

Carcinoma of the nasopharynx and upper trachea

Hypertrichosis lanuginosa acquisita

60-80%

all tumors

Optional paraneoplastic dermatoses

Dermatomyositis

10-50%

Ovarian, breast, bronchial and other carcinomas

Erythroderma (overview)

10-20%

Malignant lymphomas and others

Thrombophlebitis migrans

2-10%

Carcinoma of the eccrine pancreas, other malignancies

Pachydermoperiostosis, symptomatic(Marie-Bamberger syndrome)

5-10%

Lung carcinoma

Panniculitis, pancreatic

5%

eccrine pancreatic carcinoma

Pyoderma gangraenosum

leukaemias, plasmocytoma

Pyoderma gangraenosum (paraneoplastic)

Thymoma, lymphoma, other tumors

Bullous pemphigoid

1-2%

various tumors

Lichen planus pemphigoides

Pituitary, adrenal tumors, sympathicoblastoma

Hereditary leiomyomatosis

Renal cell carcinomas

Papular dissipated (disseminated) palmoplantar keratoses

~ 20-40%

Lung, bladder carcinoma

Filiform disseminated palmoplantar keratosis

unexplained

Lung, rectum, kidney carcinoma; malignant melanoma

Skin manifestations due to known tumor products

Substance

inducing tumor type

Porphyria, paraneoplastic

Porphyrins

Liver carcinomas

Carcinoid syndrome

prostaglandins, kinins (?), serotonin (?)

Carcinoid

Cushing's syndrome with hyperpigmentation, oral mucosal pigmentation

ACTH, MSH (?)

small cell bronchial carcinoma, other tumors

(pseudo-)pubertas praecox, gynecomastia

Gonadotropins

bronchial, liver, other carcinomas, NNR tumours

Amyloidosis, systemic

Paraprotein

plasmocytoma, reticulosis, mycosis fungoides

Scleromyxedema

Paraprotein

Plasmocytoma

Skin symptoms in which internal malignancies are frequently observed

Skin disease

Tumour type

Figurated erythema(erythema gyratum perstans, erythema anulare centrifugum)

all tumors

Epidermolysis bullosa acquisita

Carcinomas

panniculitis with polyarthritis and eosinophilia

eccrine pancreatic carcinoma

Diffuse plane xanthomatosis

plasmocytoma, mycosis fungoides

Ichthyosis acquisita (pseudoichthyosis)

Lymphoma, carcinoma

Keratosis palmoplantaris acquisita

Carcinomas

Pseudoscleroderma (see below Pseudoscleroderma)

Plasmocytoma

Calciphylaxis

Breast carcinoma

Pruritus

Lymphoma, other tumours

Atypical pyoderma, herpes zoster generalisatus, tinea generalisata, candidosis generalisata

Lymphoma, leukemia,
metastatic carcinoma

Hematomas

Leukemias

Purpura

Hypernephroma, other tumors

Vasculitides

all tumors

Fibrinogen deficiency syndrome

Prostate carcinoma

periarteritis nodosa

Carcinomas

Vitiligo

Carcinomas, melanomas

Scleromyxedema

Monoclonal gammopathies

Toxic epidermal necrolysis (TEN)

Lymphomas

Urticaria pigmentosa

Genital carcinomas in women

Acute febrile neutrophilic dermatosis (Sweet syndrome)

Acute leukemia

Multicentric reticulohistiocytosis

Carcinomas

Multiple seborrheic warts(Leser-Trélat syndrome)

Carcinomas

Multiple sebaceous gland neoplasms, keratoacanthomas and carcinomas of internal organs(Muir-Torre syndrome)

Carcinomas

Hypereosinophilic dermatitis

Hypereosinophilia syndrome

Literature
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  1. Blum A, Grossmann S, Rohm S, Rocken M (2003) Facultative paraneoplasia of the skin. German Med Weekly 128: 2257-2260
  2. Blum A, Rohm S, Grossmann S, Rocken M (2003) Obligate paraneoplasias of the skin. German Med Weekly 128: 2195-2199
  3. Cohen PR (2006) Granuloma annulare, relapsing polychondritis, sarcoidosis, and systemic lupus erythematosus: conditions whose dermatologic manifestations may occur as hematologic malignancy-associated mucocutaneous paraneoplastic syndromes. Int J Dermatol 45: 70-80
  4. Niebauer G (1984) Malignoma and cutaneous paraneoplastic syndromes. dermatologist 35: 602-608
  5. Zappasodi P, Forno C, Corso A, Lazzarino M (2006) Mucocutaneous paraneoplastic syndromes in hematologic malignancies. Int J Dermatol 45: 14-22

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 18.12.2020