Acanthosis nigricans maligna L83

Authors: Prof. Dr. med. Peter Altmeyer, Prof. Dr. med. Martina Bacharach-Buhles

All authors of this article

Last updated on: 29.10.2020

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Definition
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Obligate cutaneous paraneoplasia, predominantly occurring in abdominal adenocarcinomas (in 60% of cases it is a stomach carcinoma) . Mostly in cases of already advanced, aggressive or metastatic tumour growth of internal organs, see table 1.

Occurrence/Epidemiology
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Mostly middle to high age (only exceptionally also with children).

Etiopathogenesis
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Unsolved. A polypeptide produced by the tumour cells as a promoter of epidermal proliferation is being discussed as a trigger. See also reader-Trélat syndrome; see also Acanthosis nigricans.

Manifestation
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Usually occurring after the age of 50; rapid development of symptoms. Children may also become ill in exceptional cases.

No sex preference.

Clinical features
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  • Rapid appearance of blurred, planar, symmetrical, initially yellow-brown, later brown-black velvety lichenified plaques. Gradual increase of the skin texture, also blurred against the surroundings. Possible development of intertriginous black-brown warty papules and plaques.
  • At the oral mucosa, sour growths are possible but rarely pronounced; here areal, leukoplakic and rarely hyperpigmented plaques.
  • More rarely is the development of a cutis verticis gyrata.
  • Possibly moderate to clearly pronounced pruritus.
  • In addition to the intertriginous manifestations, hyperkeratoses may occur on palmae and plantae as well as on the flexor sides of the fingers and toes with a velvety texture of the palms (in isolated occurrence in Anglo-American called pachydermodactyly or "tripe palms").
  • In addition, there may be other associated paraneoplastic syndromes, such as reader-Trélat syndrome, hypertrichosis lanuginosa acquisita or pemphigus.
  • Note: The different forms of acanthosis nigricans are not clinically significantly different. However, in acanthosis nigricans maligna the infestation is often more extensive and more prominent than in the benign forms.

Diagnosis
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Subtle tumour search: sonography of the abdomen, CT-thorax, X-ray thorax, gastroscopy with removal of biopsy material for histological examination of tumour-specific areas, colonoscopy, possibly upper abdomen CT, gynaecological consultation (Krugenberg draining metastases).

Therapy
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Tumour search and sanitation (e.g. stomach carcinoma), symptomatic therapy, see also Acanthosis nigricans.

Progression/forecast
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Depending on the underlying tumor disease unfavorable. After tumour removal, occasionally improvement or healing of acanthosis nigricans. Recurrence in case of tumour recurrence or metastasis.

Tables
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Disease

Frequency

Abdominal tumors

Stomach cancer

56%

Liver Carcinoma

7%

Uterus carcinoma

6%

Ovarian cancer

2%

Carcinoma of the pancreas, gallbladder or bile duct

carcinoma of the small intestine, colon or rectum

Hypernephroma or Wilm's tumor of the kidney

Extra-abdominal tumors

Lung / bronchial carcinoma

7%

Breast carcinoma

5%

Lymphomas

3%

mycosis fungoides

Malignancies of:

esophagus

Bones

Prostate

Melanoma

Thyroid gland

Tests

Larynx

Hypopharynx

Urinary bladder

Chorion

Mediastinal tumor

Pheochromocytoma

unknown tumor

Note(s)
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Basically, the clinical (descriptive) term "Acanthosis nigricans" describes only a symptomatic phenomenon (see below Acanthosis nigricans), which can be triggered by different promoters. The additional term "maligna" does not indicate a special morphology but only a special etiology. An acanthosis nigricans "maligna" is therefore an "acanthosis nigricans" with a special syndromal clinical constellation, the production of growth factors by an underlying malignant tumor.

Literature
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  1. Gheeraet P et al (1991) Florid cutaneous papillomatosis, malignant acanthosis nigricans, and pulmonary squamous cell carcinoma. Int J Derm 30: 193-197
  2. Lenzner U et al (1998) Acanthosis nigricans maligna. Case description and literature review. Dermatologist 49: 41-47
  3. Longshore SJ et al (2003) Malignant acanthosis nigricans and endometrioid adenocarcinoma of the parametrium: the search for malignancy. J Am Acad Dermatol 49: 541-543
  4. Muehe B et al(2016) Verrucous cutaneous and esophageal papules, hyperpigmented flexural skin, and velvety palmar hyperkeratosis. J Dtsch Dermatol Ges 14:952-955.
  5. Schwartz RA (1994) Acanthosis nigricans. J Am Acad Dermatol 31: 1-22
  6. Thiers BH et al (2009) Cutaneous manifestations of internal malignancy. Cancer J Clin 59: 73-98

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