|Year : 1970 | Volume
| Issue : 3 | Page : 138-141
Scleroderma- a case report
Prabha Bhura, RK Mishra, BN Shrivastava
Department of Ophthalmology, Medical College, Jabalpur, India
Department of Ophthalmology, Medical College, Jabalpur
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Bhura P, Mishra R K, Shrivastava B N. Scleroderma- a case report. Indian J Ophthalmol 1970;18:138-41
Ocular involvement of scleroderma is well known. Involvement of lidskin and subcutaneous tissue is common but intraocular involvement is not well classified. Cornea (Meunier and Toussaint  ), lens and uvea (Dolfus) retina and optic nerve (Dolfus  , Jostens, Curtis and Jansen  ) involvement have been reported.
We are reporting this case of a well marked diffuse scleroderma with associated skin changes in lids, marked constriction of visual fields and pigmentary disturbances with colloid bodies spread over the retina.
| Case Report|| |
Patient G. B., female, 35 years was admitted in the medical college hospital, Jabalpur on 12-2-67 with complaints of (1) swelling on legs, feet, forearms and hands - seven months, (2) anorexia - six months, (3) pain in hip and knee joints - one month, (4) headache - one month. The onset was insidious. She first observed oedema on legs, feet and forearms. This was followed by development of articular pain and erythema. She later observed stiffness and pain in joints of hands and along with that hyperpigmentation of face.
Past illness:- Nothing- particular.
Personal history:- no: addiction to tobacco, sterilization operation four years ago, irritable by temperament.
On examination the patient had a set expressionless face [Figure - 1] with skin of forehead, maxilla, lids, fore. arms, hands, dorsum of feet and legs thin, tense and shining. The skin gave an impression of being stretched over the underlying tissues. Pigmentation was noticed over the skin. The angle of mouth was stretched [Figure - 2] giving a grinning appearance. There was a generalised stiffness of joints. The finger joints were particularly affected producing stiff claw like hands. [Figure - 3]. The fingers felt wooden hard and even passive movements were difficult. The stiff tendons could be felt and the grip was poor. She had noticed blue colouration of hands and feet when dipped in cold water. This could be demonstrated by dipping her hands in ice cold water. Of late she had developed some difficulty in swallowing.
The lid-skin was tense and shining and could not be lifted. The lids appeared to have lost their mobility and had become rigid. [Figure - 1]. They could not be moved by a pull over the skin and an appearance of ptosis was present. The levators appeared to be weaker than normal.
The movements of eyeball were normal. The cornea, conjunctiva and pupils showed no abnormality. The lens was clear and in position.
Fundus examination revealed normal discs, normal arteries and veins in calibre and ratio, but the: retinal ground showed colloid like lightly pigmented round deposits. Small pin point pigments were noticed on equatorial region. Retinal reflex was slightly dull. The macular area showed pigmentation. [Figure - 4]. Intraocular tension was normal and field of vision showed marked peripheral constriction with intact central vision. [Figure - 5].
| Investigations|| |
1. Urine and stool examination reports were negative.
2. Blood examination : E. S. R. - 20 mm at the end of first hour. Blood V . D . R . L . - negative, Blood urea - 28 mg %, Serum calcium - 16 mg %.
3. Plain X-ray of abdomen, chest and skull revealed no abnormality. On barium swallow it was found that the barium was held up at the lower end of oesophagus for 5 to 7 minutes. Slight irregularity was seen in the mucosal pattern. [Figure - 6].
4. E.C.G. tracing was normal.
5. Conjunctival biopsy showed nonspecific changes.
6. Skin biopsy - "Changes consistent with scleroderma" was reported.
| Discussion|| |
Scleroderma is an uncommon disease of unknown aetiology. However, collagen disease is considered to be the most likely cause by many but others believe it to be neurotrophic in origin with a definite connection with the autonomic nervous system. It is characteristic and pathognomonic of scleroderma that the seemingly unaffected portion of skin too reveals a definite prolongation of nervous impulse.
The diffuse variety of this disease is called scleroderma. The term morphoca is applied to the localised type. This progressive disease may occur at any age affecting females about three times more commonly as males. Internal organs like heart, oesophagus and lungs are involved and ultimately death occurs from intercurrent infection.
The disease probably starts as a vasospasm of the arterioles and oedema of the collagen bundles. A gradual occlusion; of the vessel follows and results in necrosis of the tissues. There is initial and subsequent atrophy of skin and underlying tissues. At times even bones may be affected.
There seems to be no definite pattern of ocular involvement. The lidskin shows induration, dispigmentation and atrophy which may extend upto tarsal plate and levator palpebrae muscle causing ptosis. Occasionally there is paresis of other ocular muscles like superior and medial recti (Cords, 1928). Lesions of the sympathetic nerve may lead to Horner's syndrome.
The corneal sensation is reduced in some cases. Intraocular involvement has not been regularly described but iritis (Dolfus  ) iris atrophy, eyelitis and cataract formation arc reported. Changes in fundus have been described by many authors. Meunier and Toussaint  (1958), Dolfus  (1958). Josten  (1958) observed retinal haemorrhages, new vessels in. the disc and tortuous venules and a general picture resembling thrombosis. Sheathing of the blood vessels has been reported by Meunier and Toussaint  . Curtis and Jansen  described an ipsilateral optic nerve neurocytoma with skin changes of sabre cut type of sclerodcrma in a patient aged four years who died of cerebral haemorrhage. Lauber  (1927) described changes left by subsiding uvcitis with a complicated cataract and a eyeball atrophy on the side affected by hemiatrophy. The ocular changes described in literature are a bit confusing as many of the cases do not clearly distinguish between sabre cut scleroderma and Romberg's hemiatrophy (cases of Kahler  , Osborne  , Franceschetti  and others). There is no doubt that these two conditions have a very close resemblance.
Segal, Jablonska and Mrzyglod  reported a case of sclerderma of the skin of the scalp, forehead and cheek in a man aged 43 years along with mild iritis, iris atrophy and deformed pupil.
In our case the most interesting finding was the pigmentary disturbance and colloid like bodies in the fundus associated with peripheral field constriction. The pigmentation was present in both eyes in an identical fashion. The shape and size of pigments were quite different from retinitis pigmentosa. There was no history of night blindness and the retinal vessels were normal. No explanation could be found for the field loss and for the pigmentary changes. The deposits seemed quiet and stationary. No evidence of glaucoma or raised intracranial tension was found. There is no reference for pigmentary changes in the fundus. Narrowed lumina of the capillaries may be responsible for pigmentary changes and peripheral field constriction.
| Summary|| |
A case of diffuse scleroderma with ocular changes is reported. The patient had typical changes in the lids. There were multiple colloid like exudates and pigments all over the fundus. The visual fields were markedly constricted.
| References|| |
Cords, R. E.: Cordlikc facial atrophy and the eye (in Geman). Bericht. d. Deutsch. Ophth. Gesell., 47: 53, 1928.
Curtis, A. C., and Janscn, T. G.: The prognosis of localized scleroderma. AMA Arch. Derniat., 78: 749, 1958.
Dolfus, M.: Another case of sclerodermia inn sabre-cut with multiple ocular lesions having evolved during more than 12 years. (In French) Bull. Soc. beig. Ophthal., 118: 377, 1958.
Franccschetli, A., Jadassohn, W., Paillard, R., Hofer, R, and Golay, M.: Hemiatrophy facialis or sclerodcrma inn sabre-cut. (In French) Dermatologica, 116: 331, 1958.
Josten, K.: Scleroderma in sabre-cut and the Eye. (In German) Min. Monatsbl. f. Augenh., 133 : 567, 1958.
Kahler. O.: A case of localised neural facial atrophy (in German). Prager Mediz. Wchschr., 6: 53, 1881. (ref by Cords in 1).
Lauber, H.: A case of progressive Hemiatrophy facialis with ocular participation. (In .
German). Ztschr. Augenh., 57: 492, 1925.
Meunier, A., and Toussaint, D.: Scleroderma in sabre-cut with lesions of the fundus of eye (in French). Bull. Soc. belg. Ophthal., 118: 369, 1958.
Osborne, E. D.: Morphoea associated with hemiatrophy of the face. Arch. Derm. & Syph., 6: 27, 1922.
Segal, P., Jablonska, S. and Mrzyglod, S.: Neurotrophic changes in the uvea in scleroderma in "coup de sabre". Klinika Oczna, 30: 381-388, 1960.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]