Massive Retinal Gliosis in Neurofibromatosis Type 1 Neurofibromatosis type 1 (NF1), an autosomal dominant syndrome, has major extraocular expressions of bilateral ptosis, diffuse and plexiform neurofibromas, optic nerve gliomas, and dysplasia of the sphenoid bone. With respect to the eyeball, the spectrum of involvement includes enlarged corneal nerves, Lisch iris nodules, dysplasia of the ante...

Punctate leukoderma presents as numerous, distinct, round or oval depigmented spots. Recently, laser therapy-induced punctate leukoderma associated with various Q-switched laser and carbon dioxide laser have been reported. A 25-year-old man presented with numerous, discrete, round, confetti-like, depigmented macules on his left neck. He had undergone 3 sessions of 532-nm Q-switched Neodymium: Y...

Malignant peripheral nerve sheath tumor is a rare tumor occurring in 5%-10% of all malignant soft tissues sarcomas and triton tumor arising from neurofibromatosis type 1 (NF-1) is even rarer with associated high rate of mortality. No case of triton tumor has been reported in Nigeria to the best of our knowledge. We seek to report a case of lately detected retroperitoneal triton tumor presenting...

Neurofibromatosis-Noonan syndrome is a rare RASopathy syndrome. It occurs due to the mutation in NF1 gene and patients present with phenotypic features of both Neurofibromatosis Noonan Here case an early adolescent girl described who presented chief complaint primary amenorrhoea on evaluation was diagnosed be patient The index short-statured short broad neck. Physical examination revealed point...

Four year old girl was evaluated for precocious puberty. Breast development and pubic hair growth had started at age 2½ year, and had been followed by vaginal bleeding resembling periods. Gaf-au-lait spots were present in most of her skin surface. Bone x-rays showed multiple cysts in pelvis and the metaphysis of femur. Diagnosis of McCune-Albright syndrome was made. Concentrations of all hormon...

The significance of the cafe-au-lait spot in the diagnosis of certain disorders of childhood is still not as widely appreciated as it should be, partly due to lack of information as to the normal incidence of such pigmentation, and partly to confusion over its relation to other forms of congenital pigmentation. The varieties of congenital melanin pigmentation of the skin consist of a number of ...

To the Editor: Neurofibromatosis type 1 (NF-1) is a hereditary disorder commonly associated with cutaneous, neurologic, and orthopedic manifestations.1 Psoriasis is a chronic multifactorial skin condition characterized by erythematous scaly plaques that affect the scalp, trunk, extensor surfaces of the limbs, and genital region. The condition affects 2% to 4% of the population in Western countr...

Yaumatei Dermatology Clinic, 12/F, Yaumatei Specialist Clinic (New Extension), 143 Battery Street, Yaumatei, Kowloon, Hong Kong An eleven-month-old boy presented with increasing number of yellowish papules on scalp, trunk and limbs soon after birth. Skin biopsy revealed juvenile xanthogranuloma. He was also found to have multiple café-au-lait macules. The clinical course, complications and asso...

Several pigment-specific lasers can effectively treat epidermal and dermal pigmented lesions without complications using the basic principles of selective photothermolysis. Although such pigmented lesions as solar lentigines and nevi of Ota are relatively easy to treat using pigment-specific laser technology, café-au-lait macules and melasma show variable responses to treatment. New, long-pulse...