Endocrine Abstracts

Introduction: Thyroid dermopathy usually takes the form of pre-tibial myxedema, which may rarely be caused by Hashimoto’s thyroiditis. We present an extreme variant of pre-tibial myxedema, called Elephantiasis nostras verruca (ENV), in a hypothyroid patient.

Case: A 51-year-old woman presented with a progressively worsening growth on her left leg and reduced mobility, constipation, cold intolerance and severe self-neglect. She was slow to respond to queries. She was obese and bradycardic with dry skin, patchy scalp alopecia, husky voice and no palpable goitre. There was a large ichthyotic mass affecting the left leg. The lower limbs were lymphedematous, hyperkeratotic and hyperpigmented. The patient was severely hypothyroid (free T₄ <2 pmol/l, TSH 93.9 mU/l) with positive anti-thyroid antibodies. HIV, hepatitis and syphilis screens were negative. A skin biopsy was undertaken. Viable epidermal tissue showed spongiosis and pseudoepitheliomatous hyperplasia. The dermis was oedematous with granulation tissue infiltration, and no neoplasm or infection. The patient was commenced on thyroid hormone replacement, with topical emollients and dressings to prevent super-infection. Our working diagnosis is severe thyroid dermopathy secondary to autoimmune hypothyroidism. She will be followed up jointly in the endocrine and dermatology clinics.

Discussion: Our patient has a rare and severe form of dermopathy known as Elephantiasis nostras verruca, which is the result of progressive lymphedema with a cobblestone-like appearance deforming the skin. There are multiple causes including tumours, obesity, scleroderma, and autoimmune thyroid disease. It is usually associated with Graves thyrotoxicosis, but has been reported with Hashimoto’s thyroiditis. Biopsy reveals pseudoepitheliomatous hyperplasia, dilated lymphatic spaces, and chronic inflammation. Prolonged accumulation of protein-rich interstitial fluid induces fibroblast proliferation and increases susceptibility to infection and inflammation. Treatments for ENV are challenging and include conservative measures to reduce stasis, diuretics, and prevention of recurrent infection. Surgical debridement or amputation is needed in recalcitrant cases.