Atypical tuberous myxedema Jadassohn-Dossekker E03.8

Jadassohn & Dössekker, 1916

Rare, atypically localized, dermatologic partial manifestation of Graves' disease, with circumscribed, coarse, doughy, or plate-like swellings. Also elephantiasis-like swellings with or without diffuse myxoedema.

Note: Typically, myxedema is localized pretibially in Graves` hyperthyroidism (see also Myxoedema circumscriptum symmetricum praetibiale). See also under Cutaneous Mucinoses.

See below Pretibial myxedema

Extremities, genitals, also face

Treatment is difficult. Relatively good success is achieved by consistent compression therapy (especially segmental compression; see lymphatic drainage below).

If moderate or unsuccessful, glucocorticoid crystal suspension such as triamcinolone (e.g., Volon A diluted 1:1 with LA such as scandicaine), external potent glucocorticoids under occlusion such as clobetasol (e.g., Dermoxin cream), surgical removal of the offending tissue if necessary. Recurrences often occur after discontinuation of therapy.

Promising success (and recommended as a therapeutic option), local radiotherapy was shown in a casuistry (Elsayad K et al. 2015).

Spontaneous healing may occur (after 3.5 years on average).

Plasmapheresis has been reported to have variable results.

A 63-year-old man was diagnosed with circumcurrent myxedema of the upper and lower extremities. History of thyroiditis with hyperthyroidism and endocrine orbitopathy in the context of Graves' disease were known. The patient was latently hyperthyroid after radioiodine therapy. The endocrine orbitopathy had already been resolved by endonasal microscopic orbital decompression. Clinically, the skin lesions on arms and legs presented as localized, partly symmetrical, partly asymmetrical (lower leg and dorsum of foot), infiltrative, fleshy-red to orange-brownish, shiny, coarse, nonpenetrating swellings. Their surface was peau d'orange-like in configuration with funnel-shaped indented follicular ostia.

1. Ali FR et al (2015) Dössekker's atypical tuberous myxoedema, a rare variant of scleromyxoedema. Lancet 385(9983):2222.
2. Dössekker, W (1916) On a case of atypical tuberous myxoedema. Arch. f. Dermat. 123, 76-129
3. Johnson BL et al (1973) Dermatomyositis and lichen myxedematosus. A clinical, histopathological and electron microscopic study. Dermatologica 147:109-122.
4. Stammler R et al (2023) Jadassohn-Dössekker's atypical tuberous myxedema: report of three cases and review of literature. J Eur Acad Dermatol Venereol doi: 10.1111/jdv.19029.