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We describe a new case of a rare syndrome characterized by ocular abnormalities and pathognomonic linear skin defects. This syndrome is the result of an unbalanced translocation resulting in a deletion of the distal end of the short arm of the X chromosome. We report the thirteenth case and review the clinical and cytogenetic aspects of this disorder. In addition we discuss new findings pertaining to the histopathology of the skin lesions.

We describe a case of a cutaneous perivascular myoma with features overlapping between the myofibromatosis and the myopericytoma type. The patient is a 58-year-old woman with a painless plaque-like and multinodular lesion in the pretibial dermis and subcutaneous tissue. She had repeated trauma to this site, first in her early youth that left an area of hyperpigmentation, and then again at age 40. The biopsy showed a biphasic pattern with a myofibromatosis-type component composed of spindle cell myoid nodules and more cellular round cell areas. The myopericytoma-like areas appeared to be infiltrating along vessels. These areas contained aggregates of immature-appearing cells arranged concentrically around vascular lumina in a manner reminiscent of pericytes. Immunohistochemical stains showed focal positivity for smooth muscle actin. Immunohistochemical and ultrastructural studies have showed these pericyte-like cells to be of a myoid origin. The reason for the neoplastic proliferation of perivascular myoid cells is presently unknown. The association of trauma and neoplastic transformation of the skin is rare. We report the first case of a cutaneous perivascular myoma arising in a chronic scar.

SUMMARY Twenty-seven patients with vernal conjunctivitis who remained symptomatic following treatment with corticosteroid drops and/or Opticrom drops (sodium cromoglycate 2%, benzalkonium chloride 0.01%) were treated with aspirin. Aspirin produced marked improvement in all patients apart from three. The possible mechanism of the efficacy of aspirin in vernal conjunctivitis is discussed.Vernal conjunctivitis is a severe perennial form of allergic conjunctivitis involving the conjunctiva and often also the cornea. The condition is found predominantly in children or young adults. The main symptoms are those of severe itching, photophobia, and the production of a tenacious, stringy, mucous discharge. The disease generally affects the upper tarsal conjunctiva with papillary hypertrophy and cobblestone appearance. In severe cases the limbal conjunctiva may also be affected, causing localised oedema and hyperaemia or fleshy isolated vegetations. Punctate keratitis and indolent corneal ulceration may also occur.The topical application of corticosteroids may be of considerable benefit. But control is not complete in all cases, and its long term use can produce glaucoma. The efficacy of sodium cromoglycate 2% (Opticrom) remains controversial. ' Aspirin therapy in mastocytosis was found to be beneficial, and, since the pathophysiology of both mastocytosis and vernal conjunctivitis involves mast cell abnormalities, the use of aspirin in treating the latter was tried in three patients.4We report here the effect of aspirin therapy on 27 patients with vernal conjunctivitis. Material and methodsTwenty-seven patients aged 4-18 years were treated with aspirin; 24 were males and three were females.

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