Pathogenetischer Beitrag zur Akrokeratose Bazex

Abstract

A 67-year-old male patient with typical signs of acrokeratosis Bazex is presented. The psoriasiform skin lesions appeared 9 months after diagnosis of an oesophagus neoplasm. Clinically there was a marked hepatopathy but no signs of metastases. Histological, immunofluorescence-histological, and ultrastructural findings revealed a capillaritis with humoral as well as cellular inflammation. With treatment of the neoplasm the paraneoplasia regressed under additional treatment with topical steroids. The vitamin A deficit could not be normalized by oral or parenteral vitamin A substitution. We discuss some mechanisms in formal pathogenesis.

Bazex syndrome (acrokeratosis paraneoplastica)

“…The occurrence of the skin lesions and cervical lymph node metastasis may prove to be a relevant step in the pathogenesis of the syndrome. Examination of the HLA state of patients with acrokeratosis paraneoplastica has been reported by a few authors [45,49,52,84,90]. A combination of HLA-A2 and HLA-B8 was found in three out of five cases, while the other two cases showed A2 and B8 alone.…”

“…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].…”

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

“…Common dermatological treatments (corticosteroids, tar, vitamin D 3 , keratolytics, emmolients, antibiotics, antifungals, etretinate, ultraviolet [UV]B irradiation) are generally unsuccessful but retinoids and psoralen plus ultraviolet A (PUVA) might be tried 5–7,10,17,22–33 . Complete resolution of the rash requires treatment of the underlying neoplasia 4–6,10,11,26 . Nail changes, however, frequently persist 5,7,17,26 .…”

“…[5][6][7]10,17,[22][23][24][25][26][27][28][29][30][31][32][33] Complete resolution of the rash requires treatment of the underlying neoplasia. [4][5][6]10,11,26 Nail changes, however, frequently persist. 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.…”

Akrokeratose Bazex – Eine obligate paraneoplastische Dermatose bei Plattenepithelkarzinomen aus dem Kopf-Hals-Bereich

“…Pecora et al [5] konnten bei 3082 Patienten mit Tumoren der oberen Luft-und Speisewege nur in 11 Fäl len (0.36%) die Diagnose einer paraneoplastischen Akrokeratose stellen. Als obligate kutane Neoplasie verhält sich die APB analog zum Tumorgeschehen: Besserung nach Entfernung, erneute Verschlechterung bei Rezidiv oder Tumorprogress [5][6][7], eine Therapieresistenz kann als indirektes diagnostisches Kriterium gewertet werden [1]. Die Diagnose einer APB wird durch das klinische Bild, den Tumornachweis und die Wechselwirkung von Tumor und Hautveränderungen gestellt.…”