Paraneoplastic Acrokerartosis

Abstract

The history is presented of a patient who developed an acral keratotic rash diagnosed as paraneoplastic acrokeratosis, associated with cervical metastases from a carcinoma of the tongue. The skin changes settled with surgical removal of the tumour and metastases. During treatment localized bullous pemphigoid also developed.The relationship of these problems is discussed.

Paraneoplastic acrokeratosis of Bazex (Bazex's syndrome): Report of a female case associated with cholangiocarcinoma and review of the published work

“…5,7,17,26 If the cutaneous lesions resolve with the treatment of the tumor and then subsequently return, [6][7][8]20,21,25,27,32,35 one can presume that metastatic disease has occurred. 3,4,6,7,14,22 Despite the classical skin findings, our case is noteworthy because our patient was female. To our knowledge this is the first case report with adenocarcinoma of the choledoch.…”

“…Nail changes, however, frequently persist 5,7,17,26 . If the cutaneous lesions resolve with the treatment of the tumor and then subsequently return, one can presume that metastatic disease has occurred 3,4,6,7,14,22 …”

Bazex syndrome (acrokeratosis paraneoplastica)

“…Infiltrations may also contain neutrophils or eosinophils [6]. Direct and indirect immunofluorescence of lesional skin has either shown negative findings or non-specific reactions such as the deposition of immunoglobulins, C3 or fibrin at the basement membrane zone [45,47,73,79,80,87,90]. In one case, the amino acid composition of affected nails was found to be abnormal in comparison to normal and psoriatic nails [54].…”

“…Several authors have described the occurrence of synchronic or metachronic carcinomas (as a second primary) in a few patients [15,32]. Other cutaneous signs that may reflect an underlying malignancy include acquired ichthyosis [14,35,68], bullous pemphigoid [80], or Leser-Trèlat syndrome [82]. to be unresponsive to topical treatment with steroids, antimycotics or keratolytics.…”

Bazex paraneoplastic acrokeratosis: a case report and response to Tigason

“…Its typical features and development have been well reviewed by Bazex and Griffiths (1980) and Pecora et al (1983)Ê rythematous scaly involvement of the extremities, ears, and the bridge of the nose is associated with a malignancy, most frequently in the laryngo-pharyngeal region. It is usually, but not exclusively, seen in Caucasian males of 40 years of age and over and there is only rare association with other cutaneous signs of malignancy-ichthyosis (Moulin, Valignot & Boucher 1975;Dupre et al 1976) and bullous pemphigoid (Rosner, Nurse & Dowling, 1984). It has been found associated with squamous cell carcinoma involving lymph nodes of the neck without an identifiable primary site in 10 of 50 cases (Pecora et al, 1983).…”