PubMed · 2225591
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R L Barbieri. 1990. Hyperandrogenic disorders.. https://doi.org/10.1097/00003081-199009000-00028
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More than 400 cases of acanthosis nigricans maligna (ANM) and 23 cases of "florid cutaneous papillomatosis" (FCP) were published. ANM is an obligatory paraneoplastic symptom. A 28-year old female patient with an adenocarcinoma of the stomach presented with ANM. We review the number and types of associated cancer with ANM. In most cases and adenocarcinoma of the gastrointestinal tract has been present. We feel that FCP is not an entity of its own but a variant of ANM; the literature supports this view.
We describe 8 patients affected with Costello syndrome including an affected sib pair and review the literature on 29 previously reported cases. We emphasize an association with advanced parental age, which is consistent with autosomal dominant inheritance with germline mosaicism. The pathogenesis appears to involve metabolic dysfunction, with growth disturbance, storage disorder appearance, acanthosis nigricans, hypertrophic cardiomyopathy, and occasional abnormalities of glucose metabolism. Although the cause is currently unknown, Costello syndrome is interesting because of a potential genetic-metabolic etiology.