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Linear and whorled nevoid
hypermelanosis
• develops within a few weeks of birth and progresses for
1–2 years before stabilizing

• linear and whorled hyperpigmentation following the lines of

Blaschko without preceding bullae or verrucous lesions
• Congenital anomalies, such as mental retardation, cerebral
palsy, atrial septal defects, dextrocardia, auricular atresia,
and patent ductus arteriosus may be present
 Neurofibromatosis (von Recklinghausen’s disease)

autosomal-dominantly inherited syndrome manifested by developmental changes in the nervous

system, bones, and skin
 Ataxia-telangiectasia
• known as Louis–Bar syndrome, ataxia-te
langiectasia consists of cerebellar ataxia,
oculocutaneous telangiectasia, and
sinopulmonary infection.
• It is familial and is usually first
• noted when the child begins to walk
 Familial benign chronic pemphigus (Hailey–Hailey disease)

1939, Hailey and Hailey described a familial

disease characterized by persistently
recurrent bullous and vesicular dermatitis of
the sides of the neck, axillae, and flexures

The onset is usually in the late teens or early

twenties.
• an autosomal-dominant manner.
30% patients express new mutations:genetic
defect in a calcium ATPase (ATP2C1) on
Chromosome 3q21.
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