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Early evidence of scleroderma

BMJ 1995; 311 doi: https://doi.org/10.1136/bmj.311.7021.1714 (Published 23 December 1995) Cite this as: BMJ 1995;311:1714
  1. Jan Dequeker, professora,
  2. Ludo Vanopdenbosch, research medical studenta,
  3. Antonio Castillo Ojugas
  1. aArthritis and Metabolic Bone Disease Research Unit, K U Leuven, U Z Pellenberg, B-3212 pellenberg, Belgium
  1. Correspondence to: Professor Dequeker.Rheumatology Unit, Hospital “12 de Octubre,” Madrid, Spain Antonia Castillo Ojugas, professor.

    The painting of Archangel Raphael and Bishop Francisco Domonte by Murillo (1680) is in the Pushkin Museum of Fine Arts in Moscow (fig 1). The portrait of the bishop in the lower right hand corner shows several characteristic features of scleroderma. The most striking alterations of the skin are the numerous small telangiectases on the face, lips, and hands and the tight skin around the nose, cheeks, and forehead (fig2)). At the finger the transverse skin plicae of the knuckles are not visible and the fingers are globally swollen; in other words, the distal, proximal, and metacarpophalangeal joint delineations are no longer apparent.

    Fig 1
    Fig 1

    Murillo's painting of Archangel Raphael and Bishop Francisco Domonte. Reproduced with permission of Pushkin Museum, Moscow

    Fig 2
    Fig 2

    The bishop's face and hands

    Similar skin alterations on the face can be seen in a patient with systemic sclerosis; in such patients the diagnosis of scleroderma is not based entirely on telangiectases; the features of tight skin and loss of skin folds are also important. Telangiectasia commonly represents the effect of the wear and tear of the skin and is found particularly frequently on the skin of older people, on skin exposed to the sun or after trauma or an x ray examination. Osler-Weber-Rendu disease, also called hereditary haemorrhagic telangiectasia, has to be excluded. In this condition, however, there is no tight skin and puffiness associated with small vessel abnormalities.

    Francisco Domonte was born in Seville in 1618 and joined the Orden de la Merced in 1633. He studied in Salamanca in 1638 and 1639. He was known as a great theologian and preacher, professor of arts and superior of the Convent Merced Calzados in Seville. At the age of 50 he was sent to Lima in Peru to reorganise the Mercedaire province of Nueva Espana. He left Spain with three companions in June 1668 in the ship Neustra Senora de la Victoria. We have no documents about his activities in Peru. He returned to Spain in 1677. In 1680 he was nominated Bishop of Hipona by Pope Innocent III, but died the next year.

    The painting must have been done just after his nomination as bishop in 1680 when he was 62. His dying, a few months after he commissioned the painting, indicates that he was already seriously ill. These facts are in line with the diagnosis of systemic sclerosis, a progressive disease leading to life threatening lung fibrosis or renal hypertension.

    The portrayal of scleroderma-like lesions in Murillo's painting suggests that systemic sclerosis was known before it was first described by Carlo Cruzio in 1753.

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