Bazex syndrome

Classification according to ICD-10
L85.1	Acquired keratosis palmoplantaris [Acquired keratoma palmoplantare]
ICD-10 online (WHO version 2019)

The Bazex syndrome is a rare paraneoplastic skin disease with Akrokeratosen (cornification) in carcinomas of the upper air and digestive tract or cervical lymph node metastasis .

Synonyms are: acrokeratosis type Bazex; Paraneopastic acrokeratosis ; Latin Akrokeratosis paraneopastica

The name refers to the first author of the first description from 1965 by the French dermatologist André Bazex (1911-1944) and colleagues.

The syndrome should not be confused with the Bazex-Dupre-Christol syndrome, sometimes also referred to as Bazex syndrome for short .

distribution

The frequency is given as less than 1 in 1,000,000. So far, about 150 patients have been reported, mostly men over 40 years of age, the mean age of onset is 61 years.

Clinical manifestations

Clinical criteria are:

symmetrically on the extremities (usually ears, nails, nose, fingers, hands and feet) locally scaly psoriasis-like erythema , focusing on arms , legs and torso can spread
typical beginning isolated on the auricles
Erythema with pityriasiform changes on the bridge of the nose
18% have itching
subungual hyperkeratosis , onychodystrophy (nail malformations)
palmar plantar hyperkeratosis
obligatory association with carcinoma or LN metastases
possibly initial symptom of the carcinoma
Improvement of the skin changes in the treatment of the carcinoma

diagnosis

The diagnosis requires a thorough examination of the upper respiratory and digestive tract with a search for the malignancy.

Differential diagnosis

The following are to be distinguished:

therapy

The skin changes respond to cancer treatment and also indicate a relapse.

literature

KE Douwes, T. Burgdorff, RM Szeimies, H. Messmann, T. Vogt, M. Landthaler: Paraneoplastic acrokeratosis Bazex in adenocarcinoma of the stomach. In: German Medical Weekly (1946). Vol. 126, No. 8, February 2001, pp. 203-206, doi: 10.1055 / s-2001-11311 , PMID 11256024 .
JG Zarzour, S. Singh, A. Andea, JA Cafardi: Acrokeratosis paraneoplastica (Bazex syndrome): report of a case associated with small cell lung carcinoma and review of the literature. In: Journal of radiology case reports. Vol. 5, No. 7, 2011, pp. 1-6, doi: 10.3941 / jrcr.v5i7.663 , PMID 22470801 , PMC 3303451 (free full text) (review).
V. Sharma, NL Sharma, N. Ranjan, GR Tegta, S. Sarin: Acrokeratosis paraneoplastica (Bazex syndrome): case report and review of literature. In: Dermatology online journal. Vol. 12, No. 1, 2006, p. 11, PMID 16638379 (Review).

Individual evidence

↑ ^a ^b Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
↑ ^a ^b ^c ^d Bazex syndrome. In: Orphanet (Rare Disease Database).
↑ Who named it
↑ Syndrome paranéoplastique à type d'hyperkératose des extrémités. Guérison après le traitement de l'épithélioma laryngé. In: Bulletin de la Société française de dermatologie et de syphiligraphie , Vol. 72, 1965, p. 182

[Leiber-1] Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .

[Orpha-2] Bazex syndrome. In: Orphanet (Rare Disease Database).

[3] Who named it

[4] Syndrome paranéoplastique à type d'hyperkératose des extrémités. Guérison après le traitement de l'épithélioma laryngé. In: Bulletin de la Société française de dermatologie et de syphiligraphie , Vol. 72, 1965, p. 182