Acrokeratosis paraneoplastic L85.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Acrodermatitis psoriasiformis paraneoplastic; acrokeratosis neoplastica; acrokeratosis paraneoplastica; basex syndrome; Bazex Syndrome; Paraneoplastic acrodermatitis psoriasiformis; Paraneoplastic acrokeratosis

History
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Bazex, 1965

Definition
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Skin disease belonging to the so-called obligatory paraneoplasias and accompanied by chronically persistent, erythematosquamous, hyperkeratotic plaques on the acres. The acquired skin changes may occur simultaneously with, but also several years before (in about 15% of patients) a clinically evident carcinoma. A clinically confirmed diagnosis has therefore a high diagnostic relevance.

Manifestation
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Almost exclusively men after the age of 40.

Underlying malignancies are mainly squamous cell carcinomas (tongue, pharynx, larynx, esophagus, stomach, lung).

Localization
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Finger and toe ends, nails, nose, auricle; in the late stage extensive spreading to the whole integument possible.

Clinical features
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Scaly, blurred, symmetrically appearing, psoriasiform plaques, later oedematous swelling of the acres. Hyperkeratosis of the nail bed with lifting of the nail plate and subsequent onychodystrophy. In the face erythematosus-like skin changes. Hyperkeratotic plaques on the ear helix and at the tip of the nose were also described. If they persist for a long time, they may spread to the entire skin surface. This leads to psoriasiform clinical pictures.

Histology
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Non-specific superficial dermatitis with acanthosis, ortho- and parahyperkeratosis.

Differential diagnosis
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Therapy
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Tumour search and repair, then in the best case, suspension or regression of the skin changes.

External therapy
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Externally, in the case of low levels, externally, vitamin A acid-containing externa, tretinoin (e.g. Cordes VAS). Keratolysis of the palms and soles of the feet, e.g. with 5-10% salicylic acid-containing topicals (e.g. salicylvaseline Lichtenstein) as an occlusive measure over 2-4 hours. Subsequently, 15-minute hand and foot baths, followed by careful mechanical removal of the hyperkeratoses by means of curettage or with a horn plane.

Radiation therapy
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Successes with systemic PUVA therapy are described.

Internal therapy
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If the associated skin lesions persist after tumor resection or inoperable tumor: Treatment with retinoids, e.g. acitretin (neotigason) at a dosage of 0.2-0.5 mg/kg bw/day for 4 weeks, followed by a clinically adapted dose.

Case report(s)
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Medical history: 65-year-old patient; since 6-8 months increasing, symptomless keratinization disorders on palms of hands and soles of feet /here painful under stress (pain was the cause of the visit to the doctor). At the same time increased keratinization of the areolae mammae and multiple seborrhoid keratoses in the sternal area and on the back.

Findings: Painless keratoses up to 0.2 - 0.4 cm in size, distributed over both palms, firmly adherent, yellow-brownish, partly wart-like, partly flatly sunken keratoses. At pressure sores there is extensive aggregation of the keratoses. Here "spike-like" aspect. Occasionally also "cornu-cutaneum-like". Endoscopic evidence of a previously unknown adenocarcinoma of the colon.

Comment: An acquired, generalized keratinization disorder of the skin which occurs unusually fast should always be the reason for a thorough general tumor diagnosis.

Literature
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  1. Bazex A, Salvador R, Dupré A, Christol B (1965) Syndrome paranéoplasique à type d'hyperkératose des extrémités. Guérison après le traitement de l'épithélioma laryngé. Bull Soc Franc Derm Syph 72: 182
  2. Bolognia JL, Brewer YP, Cooper DL (1991) Bazex syndrome (acrokeratosis paraneoplastica). An analytic rewiev. Medicine - Baltimore. 70: 269–280
  3. Buxtorf K et al (2001) Bazex syndrome. Dermatology 202: 350-352
  4. Gill D et al (2001) Bullous lesions in Bazex syndrome and successful treatment with oral psoral phototherapy. Australas J Dermatol 42: 278-280
  5. Hinzenstern v J et al. (1990) Paraneoplastic acrokeratosis Bazex - Course under palliative therapy of a basic tongue carcinoma. Dermatologist 41: 490-493
  6. Hoepffner N et al (1992) Special form of acrokeratosis Bazex in small cell lung cancer. dermatologist 43: 496-499
  7. Kofler L e, Kofler H (2015) Acrokeratosis paraneoplastica Bazex 6 years before diagnosis of gastric cancer. dermatologist 66: 542-544

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Last updated on: 29.10.2020