case_images: PMC3002416_01
case_images: Family pedigree
case_images: IJT-2-53-g001.jpg
case_images: PMC3002416_01_IJT-2-53-g001.jpg
case_images: F0001
case_images: The father of the two siblings married two sisters (second degree consanguinity); one child through his first wife is affected apart from these two siblings borne through his second wife [Figure 1]. A cousin of the affected siblings is also affected [Figure 1].
case_images: Woolly hair
case_images: IJT-2-53-g002.jpg
case_images: PMC3002416_01_IJT-2-53-g002.jpg
case_images: F0002
case_images: Clinical examination of the scalp, palms and soles of the siblings revealed sparse, fine, pale, curly, brittle hair having a woolly appearance [Figure 2] and longitudinal hyperkeratosis of striate form of palmoplantar keratoderma [Figure 3a and b, respectively].
case_images: (a) Striate palmar keratoderma, (b) striate plantar keratoderma
case_images: IJT-2-53-g003.jpg
case_images: PMC3002416_01_IJT-2-53-g003.jpg
case_images: F0003
case_images: Clinical examination of the scalp, palms and soles of the siblings revealed sparse, fine, pale, curly, brittle hair having a woolly appearance [Figure 2] and longitudinal hyperkeratosis of striate form of palmoplantar keratoderma [Figure 3a and b, respectively].
case_images: Right middle lobe syndrome
case_images: IJT-2-53-g004.jpg
case_images: PMC3002416_01_IJT-2-53-g004.jpg
case_images: F0004
case_images: Contrast Computed tomography (CT) of the chest showed bilateral midzone and lingular bronchiectasis with middle lobe syndrome on the right side [Figure 4].