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Trichoblastoma with sebaceous and sweat gland differentiation

Sir, The Ehlers±Danlos syndrome (EDS) is a heterogeneous group of heritable connective tissue disorders characterized by articular hypermobility, skin hyperextensibility, and tissue fragility. Type IV (EDSIV), the ecchymotic or vascular type, is a rare EDS subtype which is characterized by spontaneous vascular rupture and bowel perforation. The disease is generally inherited in an autosomal dominant fashion, but frequently there may be single affected individuals in families; it has been proposed that autosomal recessive forms also exist. The gene for EDSIV has been identified as the COL3A1 gene, located at 2q31±q32, encoding the chains of type III procollagen. A 31-year-old white female was referred to our department with suspected EDSIV. She was the second child of unrelated parents. After a normal pregnancy, she was born prematurely at 24 weeks' gestation weighing 1750 g. She failed to thrive and remained in an incubator for 2 months. At the time of delivery, her father was aged 40 years and her mother 39 years. No other members of the family were reported to be affected by skin problems. The patient's skin changes and articular hypermobility were noted immediately after birth. A tendency toward easy bruising and bleeding was noted when she was 6 years old. At the age of 14 years, marked shortening of the right achilles tendon causing talipes equinovarus was noted. In addition, she had a history of recurrent bilateral pneumothoraces treated by pleural stripping when she was 19 years old. At the age of 29 years there was a subarachnoidal haemorrhage due to rupture of an intracranial aneurysm. On physical examination, she had a thin, pinched, curved nose, thin lips, tight skin, hollow cheeks, and large prominent eyes. Tight, firm, lobeless ears were also present. There was prominent capillary telangiectasia and prominent venules over her upper anterior chest (Fig. 1). The skin on the dorsum of her hands was thin and had a prematurely aged appearance. Acrocyanosis of both hands was also noted. Mild hyperextensibility of the digits, hands, and elbows was present. Cardiological examination revealed low-grade pulmonary valve stenosis and mitral valve prolapse with thickening and prolapse of the anterior mitral valve leaflet. A skin biopsy from the left hand showed both collagen depletion and relative elastic fibre accumulation with shortening and fragmentation. Biochemical analysis by sodium dodecyl sulphatase± polyacrylamide gel electrophoresis showed decreased secretion of collagen type III by skin fibroblasts from the patient (Fig. 2). Separation of the collagen molecules retained in the cell layer showed an accumulation of collagen type III chains that migrated with a slight smear, which could indicate the presence of slower migrating, mutant collagen type III molecules. In addition to these findings there was an additional band right above the band representing the a2(V) collagen chains. In situ cyanogen bromide digestion and electrophoresis in the second dimension confirmed that