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Clinically, mild glycogenic acanthosis is a normal finding, and does not progress to esophageal cancer or to stricture. [4] It is not related to leukoplakia, and is not dysplastic or premalignant. It was originally thought to be associated with gastroesophageal reflux disease (GERD), but the association is not entirely clear. [ 2 ]
It is often underdiagnosed due to variability in disease presentation, but 99% of patients report mucocutaneous symptoms by age 20–29. [2] Despite some considering it a primarily dermatologic condition, Cowden's syndrome is a multi-system disorder that also includes neurodevelopmental disorders such as macrocephaly .
Clear cell acanthoma is characterized by a sharply demarcated psoriasiform epidermal hyperplasia composed of a proliferation of slightly enlarged keratinocytes, and basal cells with pale-staining glycogen-rich cytoplasm, mild spongiosis and scattered neutrophils, which may form small intraepidermal microabscesses.
Acanthosis nigricans is a skin condition in which areas of the skin is of a dark and velvety discoloration, often seen in the body folds and creases such as the armpits, groin, and neck. [6] Within those affected by SADDAN, acanthosis nigricans develops early on, usually in infancy or early childhood. [4]
Crouzonodermoskeletal syndrome is a disorder characterized by the premature joining of certain bones of the skull (craniosynostosis) during development and a skin condition called acanthosis nigricans. [1] Some of the signs and symptoms of Crouzonodermoskeletal syndrome are similar to those seen with Crouzon syndrome.
A skin abnormality known as acanthosis nigricans, which involves a discoloration (hyperpigmentation) and "velvety" thickening (hyperkeratosis) of the skin around skin fold regions of the neck, groin and under arms is also a common symptom. [1] Symptoms will negatively impact the daily life of the patient, and will persist until treated.
Malignant acanthosis nigricans may also become evident, many times with the sudden eruption of multiple seborrheic keratoses, known as the sign of Leser-Trélat. [ 9 ] [ 10 ] [ 11 ] Florid cutaneous papillomatosis mandates a search for an underlying malignancy, recognizing that it may be seen in patients with multiple visceral carcinomas.
The management of Glycogen storage disease IX requires treatment of symptoms by frequent intake of complex carbohydrates and protein to combat the low blood sugar. A nutritionist will advise on suitable diets. Liver function is regularly monitored and problems managed as they arise.