Acrokeratosis paraneoplastica of Bazex and Dupre

Acrokeratosis paraneoplastica occurs in association with malignant epithelial tumours of the upper respiratory or digestive tracts, in particular the pharyngolaryngeal area piriform fossa, tonsillar area, epiglottis, hard and soft palate, vocal cords, tongue, lower lip, oesophagus and the upper third of the lungs. It also occurs with metastases to the cervical and upper mediastinal lymph nodes. This 'paraneoplasia' may precede the signs of the associated malignancy, disappear when the tumour is removed and reappear with its recurrence; however, the nail involvement does not always benefit from total recovery, in contrast to the other lesions. This condition almost exclusively occurs in men over 40 years of age. The lesions are erythematous, violaceous and keratotic with ill-defined borders. They are symmetrically distributed, affecting hands, feet, ears and occasionally the nose. The toe nails suffer more severely than the finger nails. Roughened, irregular, keratotic, fissured and warty excrescences are found equally on the terminal phalanges of both fingers and toes (Figure 5.46).

The nails are invariably involved and are typically the earliest manifestation of the disease. In mild forms, the nail involvement is discrete; the affected nails are thin and soft, and may become fragile and crumble. In more established disease, the nails are flaky, irregular and whitened, and the free edge is raised by subungual hyperkeratosis. In severe forms, the

Paraneoplasia Bazex

Figure 5.46

Psoriasis-like Acrokeratosis paraneoplastica.

Figure 5.46

Psoriasis-like Acrokeratosis paraneoplastica.

lesions resemble advanced psoriatic nail dystrophy and may progress to complete loss of the diseased nails. The nail bed is eventually replaced by a smooth epidermis to which the irregular, horny vestiges of the nail still adhere. The periungual skin shows an erythemato-squamous eruption, predominantly on the dorsum of the terminal phalanges;

there may be associated chronic paronychia with occasional acute suppurative exacerbations.

The two extremes of the disease may coexist. In these cases, the proximal third of the nail is atrophic and the distal two-thirds exhibits hypertrophic changes. The histopathological changes are non-specific, although they do enable the exclusion of a diagnosis of psoriasis, lupus erythematosus or other similar eruptions.

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