Atypical tuberous myxedema

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Atypical Tuberous Myxedema

Atypical Tuberous Myxedema (pronunciation: a-typ-i-cal tu-ber-ous myx-e-de-ma) is a rare form of cutaneous mucinosis, characterized by the deposition of mucin in the skin.

Etymology

The term 'Atypical Tuberous Myxedema' is derived from the Greek words 'atypikos' meaning not typical, 'tuberos' meaning swelling, and 'myxedema' meaning mucous swelling.

Definition

Atypical Tuberous Myxedema is a rare variant of myxedema, a condition that results from the accumulation of glycosaminoglycans in the skin and other tissues. Unlike typical myxedema, which is often associated with hypothyroidism, Atypical Tuberous Myxedema is not associated with thyroid dysfunction.

Symptoms

The primary symptom of Atypical Tuberous Myxedema is the presence of firm, non-pitting, flesh-colored or slightly erythematous nodules and plaques on the skin. These lesions are typically found on the extremities, particularly the lower legs.

Diagnosis

Diagnosis of Atypical Tuberous Myxedema is primarily based on clinical findings and histopathological examination of skin biopsies. The presence of mucin deposits in the dermis, as seen on histopathology, is a key diagnostic feature.

Treatment

Treatment options for Atypical Tuberous Myxedema include systemic corticosteroids, methotrexate, and intravenous immunoglobulin (IVIG). However, the condition is often resistant to treatment and may recur after discontinuation of therapy.

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