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. [13]
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. [12] The interval between acrokeratosis paraneoplastica skin manifestations and tumor diagnosis has been reported to be as long as 3 years in some cases. [14] 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. [15]
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. [12, 13]
Cutaneous manifestations of acrokeratosis paraneoplastica include the following:
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Symmetrical, acral, scaly, red-to-violaceous plaques or patches
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Acrally distributed, hyperpigmented patches possible in persons with darker skin types
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Involvement of the fingers, distal hands, feet, nose, and helices of the ears: Isolated involvement of the helices is particularly suggestive of acrokeratosis paraneoplastica. [16]
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Involvement of the cheeks, trunk, elbows, knees, palms, and soles in advanced disease
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Nail dystrophy, horizontal and vertical ridging (75%), subungual hyperkeratosis, nail plate atrophy, tender periungual erythema, and cuticle loss [17]
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Swelling of the digits with a blue-to-violet discoloration
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Bullae of the hands and feet
There have been case reports of more unusual presentations, including oral ulcerations [18] and generalized erythroderma. [19]
Three stages of lesion evolution have been described for acrokeratosis paraneoplastica. After performing the physical examination, the patient can be staged as follows [20] :
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Stage 1: Characterized by poorly defined psoriasiform plaques involving the ears, nose, nails, fingers, and toes; nail folds become dystrophic at this stage
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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
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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. [21] Other physical examination findings suggesting the possibility of neoplasia are as follows:
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Acrokeratosis paraneoplastica. A 67-year-old woman presented with scaly plaques of the hands, feet, ears, and nose associated with esophageal squamous cell carcinoma. The eruption resolved with resection of the cancer. Image courtesy of Ronald Grimwood, MD.
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Acrokeratosis paraneoplastica. A 67-year-old woman presented with scaly plaques of the hands, feet, ears, and nose associated with esophageal squamous cell carcinoma. The eruption resolved with resection of the cancer. Image courtesy of Ronald Grimwood, MD.
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Bazex syndrome. Acquired palmar keratoderma in a woman with a history of breast cancer and recent primary lung cancer. Courtesy of Jeffrey J. Meffert, MD.
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Bazex syndrome. Violaceous psoriasiform dermatitis on the ankles. Lung cancer appeared to be in remission; both keratoderma and psoriasiform plaques resolved quickly with clobetasol ointment. Courtesy of Jeffrey J. Meffert, MD.
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At this power, a patchy lichenoid infiltrate of predominantly lymphocytes can be seen underneath an epidermis with psoriasiform hyperplasia and serum crust in the parakeratotic cornified layer (hematoxylin and eosin, 100X).
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Focal vacuolar interface change is seen with associated pigment incontinence and exocytosis of lymphocytes (hematoxylin and eosin, 200X).
