Paraneoplastic Disorders

ICD-11

EL10

Synonyms

Cutaneous markers of internal malignancy.

Epidemiology

No clear data in general, however, prevalence of association of skin signs with the individual neoplasm exist.

Definition

Cutaneous markers for underlying malignancies (not including metastases). Parallel course for tumour or blood diseases and skin findings, with improvement when neoplastic proliferation is removed or treated and worsening with recurrence or progression.

Aetiology & Pathogenesis

Genetically determined syndromes with a cutaneous component (genodermatoses) that predispose at-risk individuals to develop cancer
Paraneoplastic syndromes which occur as a result of circulating factor(s) or presumed factors produced by the underlying cancer
Factors related to neoplastic disorders are: immune reaction and production of tumor-associated antibodies from tumor or lymphoma cells or T cells or certain cytokines, enzymes, embryonic or fetal or other growth factors / proteins, hormones or hormone precursors incl. altered pathways of several metabolisms.

Signs & Symptoms

Very different and depending on the underlying disease producing several clinical patterns involving all skin structures incl. adnexes, musculoskeletal system and nerves.

Localisation

The localization may be localized or generalized, mostly symmetrical because of circulating factors.

Classification

Obligate paraneoplastic disorders /syndromes (almost 100% tumour-associated): Necrolytic migratory erythema (glucagonoma syndrome), acrokeratosis paraneoplastica of Bazex, acanthosis nigricans maligna, paraneoplastic pemphigus, erythema gyratum repens, hypertrichosis lanuginosa acquisita.
Obligate paraneoplastic genodermatoses/syndromes: Birt–Hogg–Dubé syndrome, Cowden disease, Gardner syndrome, basal cell naevus syndrome, Peutz-Jeghers syndrome, Torre-Muir syndrome.
Facultative paraneoplastic disorders (less reliable markers, but still deserve investigation): Ichthyosis acquisita, thrombophlebitis migrans, dermatomyositis in adults, bullous pemphigoid, Sweet’s syndrome, paraneoplastic pruritus, endocrine flushing ( carcinoid).

Laboratory & other workups

The laboratory work up is depending of the suspected tumor which includes skin biopsy, immunofluorescence, immunoblotting and tumor markers. Blood marker of hematological diseases.

Dermatopathology

Depends on skin signs, often similar microscopic picture as the disease the paraneoplastic one is mimicking. Some are specific: dermatomyositis or in correlation with the clinic such as genodermatoses related neoplasias.

Course

Depending on type of paraneoplastic disorder. Removal of tumor can lead to complete resolution of symptoms.

Complications

Depending on underlying neoplasm.

Diagnosis

Often to be made by clinical picture and case history and laboratory and imaging techniques/ procedures.

Differential diagnosis

The clinical picture resembles the picture of a skin disorder, therefore, a great variety has to be excluded.

Prevention & Therapy

No prevention possible despite early detection by regular tumor prevention programs. Family members of some syndromes with genetical background need to be regularly checked.

Therapy depends on underlying tumor / hematological disease.

Special

Because of orphan status of paraneoplastic skin disorders always dermatologic examination necessary and interdisciplinary work up.

3.3.15 Paraneoplastic Disorders