Acanthosis nigricans maligna L83

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

All authors of this article

Last updated on: 16.11.2022

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Definition
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Form of acanthosis nigricans belonging to the obligate cutaneous paraneoplasia, predominantly occurring in abdominal adenocarcinomas (in 56% of cases it is a gastric carcinoma). Mostly occurring in already advanced, aggressive or metastatic tumor 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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Unresolved. A polypeptide produced by the tumor cells as a promoter of epidermal proliferation is discussed as a trigger. See also Leser-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.

Localization
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Preferably affected are the intertrigines; furthermore, palmae and plantae as well as the flexor sides of the fingers and toes may be affected.

Clinical features
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The paraneoplastic form of acanthosis nigricans is characterized by the rapid appearance of blurred, symmetrical, initially yellow-brown, later brown-black velvety lichenified plaques. Furthermore by a verrucous enhancement of the skin texture. The changes are blurred against the surroundings. Possibly development of intertriginous black-brown warty papules and plaques.

On the oral mucosa, verrucous growths are possible but rarely pronounced; here, planar, leukoplakic and rarely hyperpigmented plaques.

Rarer is the development of a cutis verticis gyrata.

Possibly moderate to marked severe pruritus.

In addition to the intertriginous manifestations, hyperkeratosis 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 (referred to as pachydermodactyly or"tripe palms" when isolated in Anglo-American).

In addition, there may be other associated paraneoplastic syndromes, such as Leser-Trélat syndrome, hypertrichosis lanuginosa acquisita, or pemphigus.

Comment: The various forms of acanthosis nigricans do not differ significantly clinically. Thus, no specificity can be postulated for "acanthosis nigricans maligna". However, the involvement in the paraneoplastic form of acanthosis nigricans is often more extensive and 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

Gastric carcinoma

56%

Liver carcinoma (cholangiocarcinoma)

7%

Uterine carcinoma

6%

Ovarian carcinoma

2%

Pancreatic, gallbladder or bile duct carcinoma

Small intestine, colon or rectum carcinoma

Hypernephroma or Wilm's tumor of the kidney

Extra-abdominal tumors

Lung/bronchus carcinoma

7%

Breast carcinoma

5%

Lymphoma

3%

Mycosis fungoides

Malignancies of:

Esophagus

Bone

Prostate

Melanoma

Thyroid gland

Testes

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 review of the literature. 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. Stevens J et al (2021) anthosis nigricans as a Paraneoplastic Precursor of Metastatic Cholangiocarcinoma. Indian Dermatol Online J 12:939-940.

  7. Thiers BH et al (2009) Cutaneous manifestations of internal malignancy. Cancer J Clin 59: 73-98

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