Encephalocranial lipomatosis is a rare disorder that characteristically involves ectomesodermal tissues such as skin, eye, and the central nervous system. Here, we report a 3-year-old girl presented with developmental delay, seizures, limbal dermoid, and weakness of right lower limb. Imaging revealed hemiatrophy, arachnoid cyst, and polymicrogyria. The constellation of clinical finding and imag...

Localized scleroderma involves primarily skin but also muscle, bone and synovium. Further associations and transitional forms have been reported. We report here two cases showing associations between localized scleroderma and vasculitis, mononeuritis multiplex, juvenile chronic arthritis and hemiatrophy. In particular our cases both possess antibodies to double-stranded DNA, a finding not previ...

Two patients aged 21 and 50 years presented with facial hemiatrophy and unilateral spasms of the masticatory muscles. Masticatory muscle biopsy showed normal findings in both patients and facial skin biopsy specimens only showed atrophy, although morphoea (localised facial scleroderma) had been diagnosed nine years previously in the second patient. The involuntary movements consisted of brief t...

BACKGROUND Linear scleroderma "en coup de sabre" (LSCS) usually affects one side of the face and head in the frontoparietal area with band-like indurated skin lesions. The disease may be associated with facial hemiatrophy. Various ophthalmological and neurological abnormalities have been observed in patients with LSCS. We describe an unusual case of LSC. CASE PRESENTATION A 23 year old woman ...

FIG. 1 Linear and Whorled Nevoid Hypermelanosis: Linear and whorled hyperpigmented macules in a reticulate pattern along the Blaschko’s lines over trunk and limbs. A two-years-old boy presented with abnormal body pigmentation and body asymmetry noted since birth. A preceding vesicular eruption was absent. He was developmentally normal. Family history was insignificant. Examination revealed line...