Acanthosis nigricans maligna L83

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.

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

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.

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

No sex preference.

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

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.

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).

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