Acrokeratosis Paraneoplastica Clinical Presentation

Updated: Jan 23, 2017
  • Author: Katherine R Garrity, MD; Chief Editor: William D James, MD  more...
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Presentation

History

A diagnostic algorithm for acrokeratosis paraneoplastica has been proposed by Valdivielso, et al. At first suspicion of acrokeratosis paraneoplastica, a detailed history should be taken. [5]

The physician should inquire regarding risk factors for malignancy, including smoking habit, alcohol consumption, and family history. Upon further questioning, patients with suspected acrokeratosis paraneoplastica may admit to mild constitutional symptoms, weight loss, and other nonspecific findings of internal malignancy.

In one review of acrokeratosis paraneoplastica, when skin findings preceded the diagnosis of malignancy, they were present for an average of 1 year. [4] The interval between acrokeratosis paraneoplastica skin manifestations and tumor diagnosis has been reported to be as long as 3 years in some cases. [6] Signs of acrokeratosis paraneoplastica precede the detection of the primary malignancy in 65-70% of instances. Of acrokeratosis paraneoplastica patients, 10-15% have lesion appearance after the diagnosis of malignancy. In the remaining 15-25% of acrokeratosis paraneoplastica cases, both occur simultaneously. [7]

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Physical Examination

A complete physical examination should be performed in all patients suspected of having acrokeratosis paraneoplastica. This should include a thorough head and neck examination, including endoscopic examination, and pelvic examination in women. [4, 5]

Cutaneous manifestations of acrokeratosis paraneoplastica include the following:

  • Symmetrical, acral, scaly, red-to-violaceous plaques or patches

  • Acrally distributed, hyperpigmented patches possible in persons with darker skin types

  • Involvement of the fingers, distal hands, feet, nose, and helices of the ears: Isolated involvement of the helices is particularly suggestive of acrokeratosis paraneoplastica. [14]

  • Involvement of the cheeks, trunk, elbows, knees, palms, and soles in advanced disease

  • Nail dystrophy, horizontal and vertical ridging (75%), subungual hyperkeratosis, nail plate atrophy, tender periungual erythema, and cuticle loss

  • Swelling of the digits with a blue-to-violet discoloration

  • Bullae of the hands and feet

There have been case reports of more unusual presentations, including oral ulcerations [15] and generalized erythroderma. [16]

Three stages of lesion evolution have been described for acrokeratosis paraneoplastica. After performing the physical examination, the patient can be staged as follows [17] :

  • Stage 1: Characterized by poorly defined psoriasiform plaques involving the ears, nose, nails, fingers, and toes; nail folds become dystrophic at this stage

  • Stage 2: Characterized by involvement of larger and more proximal regions; violaceous keratoderma with central clearing is seen over the cheeks, palms, and/or soles

  • Stage 3: Characterized by involvement of the legs, knees, thighs, and arms

Attention should also be paid to signs of other paraneoplastic syndromes, because patients may present with more than one. [18] Other physical examination findings suggesting the possibility of neoplasia are as follows:

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Causes

Most acrokeratosis paraneoplastica cases are associated with squamous cell carcinoma (SCC) of the upper one third of the respiratory or GI tracts (ie, oropharynx/larynx, lungs, esophagus).

Case reports also describe SCC of the thymus, vulva, cervix, [8] and skin [9] (including SCC in situ). [10] Reports have also described acrokeratosis paraneoplastica associated with SCC with an unknown primary tumor.

Other reported associated tumors include poorly differentiated carcinoma (not otherwise specified), small cell carcinoma of the lung, lymphoma, ductal carcinoma of the breast, carcinoid, multiple myeloma, transitional cell carcinoma of the bladder, cholangiocarcinoma, [3] liposarcoma, [11] well-differentiated thymic carcinoma, [12] and adenocarcinoma of multiple primary sites including tonsils. [13]

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