Acanthosis nigricans
| Acanthosis nigricans | |
|---|---|
| Classification and external resources | |
Acanthosis nigricans on axilla
| |
| ICD-10 | L83 |
| ICD-9 | 701.2 |
| OMIM | 100600 |
| DiseasesDB | 58 |
| MedlinePlus | 000852 |
| eMedicine | derm/1 |
| MeSH | D000052 |
Acanthosis nigricans is a brown to black, poorly defined, velvety hyperpigmentation of theskin. It is usually found in body folds,[1] such as the posterior and lateral folds of the neck, the armpits, groin, navel, forehead, and other areas.
Classification
Acanthosis nigricans is conventionally divided into benign and malignant forms.,[2][3]although may be divided into syndromes according to cause.[4]:506:
- Benign This may include obesity-related, hereditary, and endocrine forms of acanthosis nigricans.[2]
- Malignant. This may include forms that are associated with tumour products and insulin-like activity, or tumour necrosis factor.[2]
An alternate classification system still used to describe acanthosis nigricans was proposed in 1994 by dermatologist Schwartz. This classification system delineates acanthosis nigricans syndromes according to their associated syndromes, including benign and malignant forms, forms associated with obesity and drugs, acral acanthosis nigricans, unilateral acanthosis nigricans, and mixed and syndromic forms.[5][6]
Signs and symptoms
Acanthosis nigricans may present with thickened, relatively darker areas of skin on the neck, armpit and in skin folds.[2]
Causes
It typically occurs in individuals younger than age 40, may be genetically inherited, and is associated with obesity or endocrinopathies, such as hypothyroidism, acromegaly, polycystic ovary disease, insulin-resistant diabetes, orCushing's disease.
Endocrine
Endocrine syndromes associated with acanthosis nigricans can develop in many conditions, particularly:[2]:978 [7]:86
- states with insulin resistance, such as diabetes mellitus
- excess circulating androgens, particularly Cushing's disease, acromegaly, polycystic ovarian disease
- Addison's disease and hypothyroidism
- Rare diseases, including pinealoma, leprechaunism, lipotrophic diabetes, pineal hyperplasia syndrome, pituitary basophilism, ovarian hyperthecosis, stromal luteoma, ovarian dermoid cysts, Prader-Willi syndrome, and Alstrom syndrome.
Acanthosis nigricans associated with endocrine dysfunction is more insidious in its onset, is less widespread, and the patients are often concurrently obese.[8]:676 This is also known as "Acanthosis nigricans type III",[4]:506–7 and insulin resistance syndromes may be divided into Type A (HAIR-AN) and type B syndromes.[2]:978
The majority of cases of acanthosis nigricans are associated with obesity and otherwise idiopathic. This is likely because of insulin resistance, and more likely to occur in darker-skinned persons.[2]:968 This is also known as "Pseudo-Acanthosis Nigricans", or "Type 3 Acanthosis Nigricans".[7]:86
Acanthosis nigricans has been linked to the use of nicotinic acid,[2] glucocorticoid use, COCP, growth hormone therapy, and stilbesterol.<.[7] This is also known as Type 4 Acanthosis nigricans.[7]:86
Familial
Familial acanthosis may arise as a result of an autosomal dominant trait, presenting at birth or developing during childhood.[4]:506[8]:676. This is also known as Acanthosis nigricans type 1.[7]:86
Acral Acanthotic Anomaly
Acral Acanthotic Anomaly refers to a variant of acanthosis nigricans limited to the elbows, knees, knuckles and dorsal surfaces of the feet, in the absence of any other findings, in otherwise healthy individuals.[9][10][11][12] While the etiology remains unknown,[12] its presence does not suggest a likelihood of malignancy.[12]
Malignant
Malignant acanthosis nigricans refers to acanthosis nigricans occurring as a paraneoplastic syndrome associated with a cancer. Malignant acanthosis nigricans is most-commonly associated with gastrointestinal adenocarcinomas, as well as genitourinary cancers such as those of the prostate, breast, and ovary. Other cancers, such as those of the lung, stomach, and lymphoma, are occasionally associated with acanthosis nigricans.[7]:86[13]
This form of acanthosis nigricans is more likely to involve mucous membranes (25-50% of cases)[3][14] This variant is also known as Acanthosis nigricans type I[8]:676 or 5[7]:86. Malignant acanthosis nigricans that may either precede (18%), accompany (60%), or follow (22%) the onset of an internal cancer.[4]:506 Malignancy-associated acanthosis nigricans is usually rapid in onset and may be accompanied by skin tags, multiple seborrheic keratoses, or tripe palms.[8]:676
Pathophysiology
The pathogenesis of acanthosis nigricans is poorly understood, and likely to relate to an interplay of factors,[15] including insulin-mediated activation of ILGF receptors on keratinocytes, and increased growth factor levels.[15]
Factors involved in the development of acanthosis nigricans include:
- Increased circulating insulin. This activates keratinocyte ILGF receptors, particularly IGF-1. At high concentrations, insulin may also displace IGF-1 from IGFBP. Increased circulating IGF may lead to keratinocyte and dermal fibroblast proliferation.[15]
- Fibroblast growth factor. Hereditary variants are associated with FGFR defects.[15]
- Increased TGF, which appears to be the mechanism for malignancy-associated acanthosis nigricans. TGF acts on epidermal tissue via the EGFR.[7]:86
In conjunction with increased end levels of ILGF, it is likely that perspiration and friction may be necessary predeterminants for lesions.[15]
Diagnosis
Acanthosis nigricans is typically diagnosed clinically.[2] A skin biopsy may be needed in unusual cases. If no clear cause of acanthosis nigricans is obvious, it may be necessary to search for one. Blood tests, an endoscopy, or x-rays may be required to eliminate the possibility of diabetes or cancer as the cause.[7]:87
On biopsy, hyperkaratosis, epidermal folding, leukocyte infltration, and melanocyte proliferation may be seen.[2]:979[7]:87
Differential Diagnosis
Acanthosis nigricans should be distinguished from the Casal collar appearing in pellegra.[citation needed]
Prognosis
Acanthosis nigricans is likely to improve in circumstances where a known cause is removed. For example, obesity-related acanthosis nigricans will improve with weight loss, and drug-induced acanthosis nigricans is likely to resolve when the drug is ceased. Hereditary variants may or may not fade with age, and malignancy-associated variants may, after a malignancy is removed, fade.[7] :87
Treatment
People with acanthosis nigricans should be screened for diabetes and, although rare, cancer. Controlling blood glucose levels through exercise and diet often improves symptoms. Acanthosis nigricans maligna may resolve if the causative tumor is successfully removed.[16]
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