September 2004
Diagnosis: Acrokeratosis paraneoplastica (Bazex syndrome)
Acrokeratosis paraneoplastica or Bazex syndrome is a psoriasiform dermatosis that is associated with internal malignancy. Majority of the patients are males over the age of 40. Patients usually present with acute onset of dystrophic changes involving all fingernails and toenails. Next, the skin of fingers, toes, hands, and feet develops violaceous scaly patches and plaques. Eventually, the scaly violaceous dermatosis progresses to involve helices of the ears, nose, cheeks. In the most severe cases, patients can have more generalized disease affecting the scalp, trunk, and extremities.
Internal malignancies most commonly associated with acrokeratosis paraneoplastica are from the upper 1/3 of the aerodigestive tract including oral cavity, pharynx, larynx, and esophagus. The skin eruption usually precedes the development of internal malignancies. Other rarely associated cancers include small cell carcinoma of the lung, adenocarcinoma of the uterus, Hodgkin's lymphoma, and colon adenocarcinoma.
Topical steroids may improve the scaly lesions and cholecalciferol
has been reported to be beneficial in some cases. However, the
best results occur when the underlying neoplasm is treated. If
the underlying cancer is cured, the skin signs abate. Nail dystrophy
may persist after effective cancer treatment and resolution of
other cutaneous changes.
The term Bazex syndrome describes 2 different entities: 1. Acrokeratosis
paraneoplastica and 2. The genetic syndrome of basal cell carcinomas,
follicular atrophoderma, hypotrichosis, and hypohidrosis or hyperhidrosis.
References:
1. Atilla MK, Yllmaz Y, Bekerecioglu M, Akpolut N, Kosem M,
Aydin S. Bazex syndrome: acrokeratosis paraneoplastica in association
with simultaneous multiple genitourinary tumors Urology. 2000
Jul;56(1):153.
2. Hsu YS, Lien GS, Lai HH, Cheng YS, Hu CH, Hsieh MC, Fang CL,
Pan S. Acrokeratosis paraneoplastica (Bazex syndrome) with adenocarcinoma
of the colon: report of a case and review of the literature. J
Gastroenterol. 2000;35(6):460-4. Review.
3. Lucker GP, Steijlen PM. Acrokeratosis paraneoplastica (Bazex
syndrome) occurring with acquired ichthyosis in Hodgkin's disease.
Br J Dermatol. 1995 Aug;133(2):322-5.
4. Estrela F, Pinto GM, Pinto LM, Afonso A. Acrokeratosis paraneoplastica
(Bazex syndrome) with oropharyngeal squamous cell carcinoma. Cutis,
1995 Apr;55(4):233-6.
5. Bolognia JL, Brewer YP, Cooper DL. Bazex syndrome (acrokeratosis
paraneoplastica). An analytic review. Medicine (Baltimore). 1991
Jul;70(4):269-80. Review.
My thanks to Drs. Tony Hsu and Susan Mallory for presenting this case.