Carneys syndrome

Carotenemia

Synonym(s)

Carney myxoma-endocrine complex; myxoma-spotty pigmentation-endocrine overactivity

Definition

Familial multiple neoplasia and lentiginosis syndrome with primary pigmented nodular adrenocortical disease (PPNAD); primary adrenal form of hypercortisolism, lentigines, ephelides, and blue nevi of the skin and mucosae and a variety of endocrine and other types of tumors

Pathogenesis

Autosomal dominant trait; two genetically distinct forms: one type mapped to chromosome 17 (CNC type 1); second type mapped to chromosome 2

Clinical manifestation

Lentigines; nevocellular nevi; freckling; hamartomas of the oral cavity; striae; kyphosis; easy bruising; sparse or absent hair; atrial and skin myxomas Endocrinopathies: Cushing syndrome; acromegaly; hyperparathyroidism; prolac-tin-secreting tumor; multiple thyroid nodules

Differential diagnosis

Nevi; lentigines; McCune-Albright drome; neurofibromatosis; ephelides syn-

Therapy

Treatment of the endocrine overactivity; surgical excision of symptomatic myxomas

Synonym(s)

None

Definition

Increased carotenoid pigments from ingestion of foodstuffs containing these nutrients

Pathogenesis

Deposition of yellow-orange pigments in the stratum corneum after the prolonged or excessive consumption of carotene-rich foods

Clinical manifestation

Asymptomatic, yellow-orange discoloration, particularly at sites with a thick stratum corneum, such as the palms and soles; no change in scleral pigmentation

Differential diagnosis Jaundice; lycopenemia (orange-yellow skin discoloration due to the ingestion of large amounts of tomatoes); riboflavinemia; Addison's disease; drug reaction (e.g. quinic-rine)

Therapy

Decreased ingestion of carotene-rich foods

References

Leung AK (1987) Carotenemia. Advances in Pediatrics 34:223-248

Carrion disease

► Bartonellosis

References

Kiryu T, Kawaguchi S, Matsui E, Hoshi H, Kokubo M, Shimokawa K (1999) Multiple chondroma-tous hamartomas of the lung: A case report and review of the literature with special reference to Carney syndrome. Cancer 85(i2):2557-226i

Carrion's disease

► Bartonellosis

120 Cat-scratch disease

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