Congenital Glaucoma and Cutis marmorata telangiectatica: Report of the Second Case

E Vázquez, MD, B. López, MD, L. Requena, MD, A. García-Pérez, MD, Dpto. Dermatología, Hospital Clínico ‘San Carlos’, E– 28040 Madrid (Spain) To the editor Described in 1922 [1], cutis marmorata telangiectatica congenita (CMTC) is a very rare disorder. It is characterized by: persistent cutis marmorata, phlebec-tasia and telangiectasias. Ulcers and atrophy are less frequently seen [2]. The disorder is usually present at birth or few months later. It is generally agreed that CMTC improves with age. Up to now, no recognizable pattern of inheritance has been reported. Skin biopsy shows only dilatation of blood vessels with increase in number. In about 50% of patients, it tends to be associated with various congenital anomalies, being hypertrophy or atrophy of an involved extremity the most frequently associated anomaly [3]. Congenital glaucoma has been described in only 1 patient [4]. We had a new case that we consider of interest to communicate. This Caucasian boy was the first child of a 26-year-old woman. No family history was evident and no consanguinity between parents was related. At birth, the skin surface showed widespread reddish-blue reticular network (fig. 1). The extremities manifested this pattern most severely. Ulcers, phlebectasies or masses were not observed. The lesions increased with cold and crying and blanched with pressure. Mucous membranes were not involved. Extremities were equal in size and development. Physical examination was otherwise normal with the exception of right ocular glaucoma diagnosed by intraocular pressure measurements. Results of the following tests were normal: Chemistry group, complete blood count, differential count, uri-nalysis, chest, skull, abdomen, spine and upper and lower limb X-rays were also normal as were brain scan, abdominal echography and EKG. Skin biopsy was not done. Follow-up examination during 2 years revealed persistence of the reticular pattern, without developing ulcers or atrophy. Development alterations, mental retardation or visual disturbances were not observed. Goniotomy was performed as treatment of the congenital glaucoma. This is the second report of congenital glaucoma associated with CMTC. In the first report [4], glaucoma was bilateral and other anomalies were present