Image from: Squires, B., S. D. Daveluy, M. C. Joiner, N. Hurst, M. Bishop and S. R. Miller (2016). "Acrokeratosis Paraneoplastica Associated with Cervical Squamous Cell Carcinoma." Case Reports in Dermatological Medicine 2016. Under the Creative Commons Licence.
Case reports are frequently published in dermatological literature as short pieces and for me act as a form of mini-CPD. The articles are in quiz form or just start as a clinical presentation with a case history and a picture. By reading through it is often a self-test to try and reach a diagnosis before it is given in the article. I was recently reading a paper [1] in a journal which caught my eye as it is one of those conditions which may be very rare to the podiatrist but the condition itself can be a cutaneous marker of a more sinister internal disorder.
Bazex disease is a disorder which typically presents in the acral regions first – particularly on the foot and in the toe nails. What makes this more challenging is that the condition is describe as “psoriaform” in that it can mimic the typical pedal presentation of psoriasis namely scaly plaques, hyperkeratosis and associated nail changes. Typically, according to the available case reports, the condition may develop over a short period (often a few months) and advances very much in way similar to psoriasis on the foot with the development of hyperkeratotic areas of the soles and around the digits which may enlarge and gradually coalesce. Some reports suggest they may be of a bluish or violet discolouration [2]. Hyperkeratosis maybe more pronounced over the weight bearing areas. The distribution, again, like psoriasis is generally symmetrical. Examination of the nails is also important here as changes typically occur such as onycholysis, sub-ungual debris, longitudinal or transverse ridges, loss of cuticles [3] and nail plate discolouration. The condition is rarely painful or itchy.
From this initial presentation, without treatment, the lesions may then progress typically to the legs and arms. Reports also suggest that fine, hyperkeratotic lesions are frequently observed in other acral areas including the hands, ears and nose [4]. Diagnosis of the condition can only be achieved through a careful history, examination and biopsy. On the skin, it is important to rule out disorders such as psoriasis, eczema, palmo-plantar keratoderma and fungal infection.
However, what sets Bazex’s disease apart from these conditions is that it is a paraneoplastic disorder. A neoplastic disorder is defined as a clinical disorder (i.e. of the skin, neurological, haematological or hormonal system etc.,) which is associated with an internal neoplasm. Basex’s disease (also termed “Acrokeratosis Neoplastica of Bazex”) was first described by Bazex in 1965 in which he reported it that it was most frequently is associated with squamous cell carcinoma of the aero-digestive tract. The condition is see in men much more than in women, in the forty plus age group. Only around 10% of the 140 or so reported cases have been in women.
The appearance of the skin symptoms can be key to an earlier diagnosis as lesions may precede the diagnosis of an internal malignancy by a year or two in some cases. As the condition is associated with an underlying internal neoplasm, direct treatment of the skin condition is frequently unsuccessful until the underlying neoplasm has been removed or effectively treated. The reasons for the development of the skin lesions remains unclear but it is proposed that there is a cross reactivity between antibodies directed against the tumour within the basement membrane of the skin [5].
From a podiatric standpoint, it is important to know that the initial skin problem can arise many months before any internal symptoms develop and so practitioners should be alert to this fact and be suspicious with any patient presenting with a sudden onset, symmetrical plantar hyperkeratosis. In such cases a medical assessment would be advisable, paying particular attention to aspects of the relevant history such as weight loss, smoking, drinking and a family history of carcinoma.
Article References
1. Zhao, J., et al., Case Report: Bazex Syndrome Associated With Pulmonary Adenocarcinoma. Medicine (Baltimore), 2016. 95(2): p. e2415.
2. Rodrigues, I.A., Jr., et al., Bazex syndrome. An Bras Dermatol, 2013. 88(6 Suppl 1): p. 209-11.
3. Fleming, J.D., C.M. Stefanato, and N.R. Attard, Bazex syndrome (acrokeratosis paraneoplastica). Clinical and Experimental Dermatology, 2014. 39(8): p. 955-956.
4. Humphrey, S.R., et al., Acute onset of acrokeratosis paraneoplastica (bazex syndrome). JAMA Dermatology, 2015. 151(6): p. 677-678.
5. Vatandoust, S., et al., Acrokeratosis paraneoplastica (Bazex syndrome) associated with metastatic cutaneous squamous cell carcinoma. Intern Med J, 2016. 46(1): p. 119-20.
Links to recent case reports (full and free access):