Hallermann Steriff Syndrome With Cutaneous Manifestations : A Case Review
1305 WordsSep 12, 20166 Pages
Hallermann-Steriff Syndrome with cutaneous manifestations: A case review
Anugrah Parikh M.D; Apeksha Solanki M.D; Rishi Patel MBBS; Dr Rashmi Arora MBBS
Department of Dermatology, GK General Hospital, Gujarat Adani Institute Of Medical Sciences, Bhuj, Gujarat
Keywords-Hallermann-Steriff Syndrome, Nevus anemicus, Hypospadias.
Summary- Hallermann-Steriff Syndrome is a very rare genetic disorder that is characterized primarily by head-face abnormalities,hypotrichosis and various ophthalmic abnormalities. We report here a 14 year old boy with Hallermann-Steriff syndrome with nevus anemicus and review the literature.
The first description of Hallerman Steriff syndrome seems to have been made in 1893 by Aubry and later it was described completely in 1948 by Hallermann and then by Steriff in 1950 (1)
HSS is primarily characterized by head-face abnormalities. Patients have bird like facies, dental abnormalities, hypotrichosis with various ophthalmic abnormalities(2).
Seven essential signs described by Francois as diagnostic criteria for HSS are presented in table (2)
1. Dyscephalia and bird like facies
2. Abnormal dentition
4. Atrophy of skin especially on nose
5. Bilateral Microphthalmia
6. Congenital Cataracts
7. Proportionate Dwarfism
A 14 year old boy came to the Department of Dermatology in our hospital with complaints of hair loss from scalp since birth and altered hair color, hypopigmented macular lesion on the trunk since