| Abstract|| |
A 45 year old male presented with dry skin, facial puffiness, weight gain, constipation and lethargy for five months. He had been diagnosed as thyrotoxicosis fifteen years back and improved after oral antithyroid drugs. General examination revealed bradycardia, obesity, hoarseness, proptosis and acropachy. Dermatological examination revealed pretibal as well as generalised myxoedema with cold, hyperpigmented and xerotic skin. Hair was thin, coarse and brittle and nails brittle. Face appeared puffy, expressionless and eyelids wrinkled and drooping. Palms, soles had a yellowish hue. The neck revealed a diffusely enlarged thyroid. Hormone profile revealed reduced T3, T4 and raised TSH. Thyroperoxidase antibody was positive. ECG showed low voltage sinus bradycardia. X-ray hands substantiated thyroid acropachy. Skin biopsy confirmed pretibial myxoedema. He was diagnosed as Graves disease with past hyperthyroidism and present hypothyroid state and managed with oral thyroxine with improvement. The pretibial myxoedema was successfully managed with intralesional and topical steroids.
Keywords: Thyroid dermopathy, Exophthalmos, Acropachy, Graves′ disease, Autoimmunity
|How to cite this article:|
Chatterjee M. The spectrum of thyroid autoimmunity. Indian J Dermatol 2006;51:131-3
| Introduction|| |
Thyroid autoimmunity can cause several disorders and may lead to hypo as well as hyperthroidism. Graves' disease, thyroiditis and idiopathic hypothyroidism fall among this category of disorders. The classical manifestations of Graves' disease consist of diffuse thyroid enlargement, pretibial myxoedema (thyroid dermopathy), exophthalmos and thyroid acropachy. About 15% of patients who remit after treatment of this hyperthyroid condition develop hypothyroidism 10-15 years later due to continuing destructive autoimmune process. Important dermatological manifestations of hypothyroidism include generalized oedema/ myxoedema, thinning and drying of skin and appendages, brittle nails, reduced hair density, xanthomatosis, carotenaemia, purpura, telangiectases and delayed wound healing.
It is rare to see a patient with manifestations of hyper as well as hypothyroidism at the same time. Herein, we report one such case.
| Case Report|| |
A 45 year old male presented with dryness of the skin with puffiness of face of five months duration, with unquantified weight gain, constipation and lethargy. The patient's relatives complained of him becoming expressionless and slow in registering emotions. He gave history of having swelling of fingertips, skin lesions over the front of shins and protrusion of eyeballs since 6 years. Past history revealed that he was diagnosed as a case of thyrotoxicosis 15 years back and improved after carbimazole for 1 ½ years.
General examination revealed bradycardia (pulse 55/min regular) and weight was 85 kg (Height 160 cm). Voice was minimally hoarse. Eyes showed proptosis. There was bulbous enlargement of the tips of fingers with increased volume of distal phalanges, obliteration of the angle of the nail plate and increased curvature of the nail plate in all planes with a few nails showing dystrophic changes [Figure - 1]. There was no significant lymphadenopathy. Systemic examination was within normal limits.
Dermatological examination revealed multiple well defined roughly symmetrical thickened yellowish waxy plaques 2 x 3-5 x 6 cm in dimension over both shins, associated with prominent pilosebaceous follicles in a 'peau de orange' appearance [Figure - 2]. Also, there was diffuse non-pitting oedema of the legs and feet with hyperpigmentation of the skin, which was cold, hyperkeratotic and xerotic. Hair was thinned, coarse and brittle and nails brittle. The face appeared puffy and expressionless and eyelids wrinkled and dropping. Palms and soles had a yellowish hue. Examination of the neck revealed a diffusely enlarged thyroid [Figure - 3]. Ophthalmometric examination confirmed proptosis (22 mm). There were no xanthomas, purpura, telangiectases or vitiligo and mucosae were normal.
Routine haemogram revealed no abnormalities. Thyroid hormone profile revealed reduced triiodothyronine (0.2 Nanomol/l), tetraiodothyronine (5 Picomol/l) and raised Thyroid Stimulation Hormone (25 mU/l). Thyroperoxidase antibody was positive. Pulmonary function tests, liver function tests, blood sugar, lipid profile, serum electrolytes and serum calcium were normal. ECG showed sinus bradycardia with low voltage complexes. X-ray of the hands revealed soft tissue spindling and speculated periosteal new bone formation at right angles to the axis of the bones involving shafts of metacarpals and phalanges. Skin biopsy from the lesions over the shin revealed a frayed and oedematous dermis on haematoxylin and eosin staining and increase in alcian blue positive mucinous material with connective tissue fiber separation and reactive fibroblast proliferation.
He was diagnosed as Graves' disease with diffuse hyperpigmentation, exophthalmos, pretibial myxoedema and thyroid acropachy as manifestations of past hyperthyroidism and bradycardia, obesity, hoarsenss of voice, generalized pedal oedema, cold hyperkeratotic xerotic skin, coarse thinned brittle hair, brittle nails, puffiness of face, wrinkled dropping eyelids and carotenaemia of palms and soles as indicators of present hypothyroid state.
He was managed with oral thyroxine 25 microgram increased to 50 micrograms per day after 4 weeks, with improvement in hypothyroid manifestations. The pretibial myxoedema was managed with intralesional triamcinolone acetonide (40 mg/ml) every 4 weeks and topical steroid (clobetasol propionate 0.05%) under occlusion with gradual regression. The patients is presently improving on continued follow-up while on therapy.
| Discussion|| |
Among other theories, pretibial myxoedema has been proposed to be caused by antibodies against a thyroid antigen working on fibroblasts. HLA DR 3 positive individuals have been found to be more prone. Localisation to the lower extremity could be due to dependency, trauma or other local factors. Important confusing conditions include simple oedema, generalized myxoedema, chronic/lichenified dermatitis, hypertrophic lichen planus and urticarial phase of bullous pemphigoid.
Ophthalmopathy is caused by increased interstitial fluid accumulation with mycopolysaccharides and chronic inflammatory infiltrate in extraocular muscles. Thyroid acropachy is caused by diaphyseal proliferation of the periosteum with new bone formation in distal long bones. Common differential diagnoses of the latter include hypertrophic pulmonary osteoarthropathy and pachydermoperiostosis.
All grades of eye changes of exophthalmos occur in 40-50% of cases of thyrotoxicosis. Pretibial myxoedema generally follows the eye lesion and can occur in 0-4.3% of patients of Graves' disease and 15% of cases of ophthalmopathy. Acropachy is present in <1% of patients and is usually seen in conjunction with ophthalmopathy and dermopathy.
In addition to thyroid and thyroid stimulating hormone, thyroid antibodies estimation, and histological changes are useful in diagnosis if available, as in our case.
Topical steroids under occlusion have been found useful in pretibial myxoedema as they depress mucopolysaccharide synthesis and suppress the autoimmune phenomenon. Other treatment modalities are intralesional and systemic steroids, hyaluronidase, plasmapheresis, cytotoxic drugs, octreotide (a synthetic somatostatin analogue), immunoglobulins, pentoxifylline and surgery. Our patient responded very well to a combination of occlusive and intralesional steroids.
Though this condition with its classical manifestations has been well described, the complete gamut of manifestations presenting in the same patient is very rare and with thyroid hypofunction and its manifestations as well, even rarer. Our case is also interesting as glandular hypofunction has occurred after antithyroid drugs, whereas this condition occurs most frequently post-operatively or after isotope therapy.
To conclude, our patient, who developed Graves' hyperthyroidism 15 years back and was treated with antithyroid drugs for 18 months with remission, developed thyroid associated ophthalmopathy, dermopathy and acropachy 9 years later and features of hypothyroidism 14 ½ years thereafter, the course of illness showing the complete spectrum of thyroid autoimmunity.
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3]