Café au lait spot (CALS) is a light or dark brown spot that has various sizes (diameter of 0.5cm-30cm). Solitary or multiple lesions may occur on any parts of body except palms and soles at birth or in infancy. Histopathologically, since nevus cell does not exist in CALS and CALS is not changed into malignant lesion, therefore CALS is a benign pigmented disease [1,2]. That said, treatment is no...

BACKGROUND Urticaria pigmentosa (UP) is characterized by dense aggregates of mast cells in the dermis. There is consistent evidence from the literature that mast cells may play a pathogenetic role in the development of neurofibromas and other tumors. OBJECTIVE To study the concomitant appearance of UP with neurofibromas and neurofibroma-like neoplasms. METHODS We analyzed 31,752 records of ...

Tuberous sclerosis (TS) is an autosomal dominant disease that affects the brain, skin, eye, heart and kidney. The diagnostic criteria for tuberous sclerosis complex (TSC) have recently been revised. There are relatively few Indian studies on this disorder. Twenty-six patients diagnosed as having TS over a period of 18 years are being reported. The onset of seizures ranged from infancy to adoles...

Familial progressive hyperpigmentation is rarely described in the literature. We report on five patients from three different families presenting with a peculiar progressive pigmentary disorder. The patients show a progressive diffuse, partly blotchy, hyperpigmentation, intermixed with scattered small hypopigmented macules, a few large hypopigmented areas, occasional café-au-lait spots and, mos...

McCune-Albright syndrome is a rare fibro-osseous syndrome characterised by a classic triad of fibrous dysplasia (FD), café-au-lait macules and various underlying endocrinopathies. This case report describes how a patient was rediagnosed by a general dental practitioner following attendance for a routine dental examination. The patient had been previously diagnosed with the condition 28 years ea...

Six months later, she noticed proptosis of the right eye and her diplopia persisted. Our examination showed acuity of 20/20 OU and a left relative afferent pupillary defect. She had 3 mm of right proptosis, -3 adduction deficit in her right eye and -2 abduction deficit in her left eye with -1 elevation deficit bilaterally. There was no nystagmus. Saccades were normal. She had one Lisch nodule a...

McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, café-au-lait macules and hyperfunctioning endocrinopathies including human growth hormone excess. Acromegaly as a manifestation of endocrine hyperfunction with MAS is uncommon. Surgical excision may be challenging due to the associated severe fibrous dysplasia of the skull base. Through the endoscop...