Acrokeratosis Neoplastica Clinical Presentation

A diagnostic algorithm for acrokeratosis neoplastica has been proposed by Valdivielso, et al. At first suspicion of acrokeratosis paraneoplastica, a detailed history should be taken.^[4]

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

In one review of acrokeratosis neoplastica, when skin findings preceded the diagnosis of malignancy, they were present for an average of 1 year.^[3] The interval between acrokeratosis paraneoplastica skin manifestations and tumor diagnosis has been reported to be as long as 3 years in some cases.^[5] Signs of acrokeratosis paraneoplastica precede the detection of the primary malignancy in 65-70% of instances. Of acrokeratosis neoplastica patients, 10-15% have lesion appearance after the diagnosis of malignancy. In the remaining 15-25% of acrokeratosis neoplastica cases, both occur simultaneously.^[6]

A complete physical examination should be performed in all patients suspected of having acrokeratosis neoplastica. This should include a thorough head and neck examination, including endoscopic examination, and pelvic examination in women.^{[3, 4]}

Cutaneous manifestations of acrokeratosis neoplastica are as follows:

• Symmetrical, acral, scaly, red-to-violaceous plaques or patches
• Involvement of the fingers, distal hands, feet, nose, and helices of the ears: Isolated involvement of the helices is particularly suggestive of acrokeratosis neoplastica.
• Involvement of the cheeks, trunk, elbows, knees, palms, and soles in advanced disease
• Nail dystrophy
• Swelling of the digits with a blue-to-violet discoloration
• Acrally distributed, hyperpigmented patches possible in persons with darker skin types
• Bullae of the hands and feet

Three stages of lesion evolution have been described for acrokeratosis neoplastica. After performing the physical examination, the patient can be staged as follows^[7] :

• Stage 1: These acrokeratosis neoplastica patients have poorly defined psoriasiform plaques involving the ears, nose, nails, fingers, and toes. Nail folds become dystrophic at this stage.
• Stage 2: These acrokeratosis neoplastica patients have involvement of larger and more proximal regions. Violaceous keratoderma with central clearing is seen over the cheeks, palms, and/or soles.
• Stage 3: This acrokeratosis neoplastica stage is reached when lesions have spread over more distal sites such as 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.^[8] Other physical examination findings suggesting the possibility of neoplasia are as follows:

• Ichthyosis
• Pruritus
• Sign of Leser-Trélat
• Clubbing
• Dermatomyositis

• Most acrokeratosis neoplastica 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, and skin and of 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,^[2] liposarcoma,^[9] well-differentiated thymic carcinoma,^[10] and adenocarcinoma of multiple primary sites including tonsils.^[11]