Leukoderma and poliosis will be present at birth in a characteristic ventral midline pattern. The most common finding is a white forelock in a triangular shape, which is present in up to 90% of cases of piebaldism (poliosis circumscripta).[6] The medial eyebrows and eyelashes may also be affected. The classic distribution of the well-circumscribed depigmented macules and patches includes the central forehead, anterior torso, and mid-portion of the extremities. Areas of hyperpigmentation may be present within or bordering the areas of depigmentation. Piebaldism is notable for the absence of extracutaneous manifestations.

The phenotypic findings of piebaldism will be apparent at birth. Despite incomplete penetrance and variable expressivity, detailed family history is useful to establish an inherited genetic cause. During the postnatal period, careful physical examination, including ophthalmic examination and neurologic examination, can help distinguish piebaldism from syndromic causes of leukoderma, such as PCWH and Waardenburg syndrome. Although Waardenburg syndrome may have a similar phenotypic appearance to piebaldism, there will also be extracutaneous features, such as congenital deafness and heterochromia. Concomitant Hirschsprung disease is concerning for PCWH. Wood's lamp examination is useful to distinguish between piebaldism and nevus anemicus and nevus depigmentosus.[7] A biopsy, including the full thickness of the epidermis, can be helpful in equivocal cases.

Although the depigmented areas of piebaldism are benign and stable, the appearance can be socially and psychologically debilitating for some patients. Treatment is challenging and focuses on improving cosmesis. Although temporary, cosmetic camouflage (make-up) and hair dye can improve quality of life.[1] The depigmented areas have been successfully treated using dermabrasion and split-skin grafting, autologous melanocyte transplantation, epidermal sheet grafting, and autologous cell suspension transplantation after superficial full surface ablation.[8][9][10][11] Due to the absence of the photoprotective effects of epidermal melanin, patients with piebaldism should receive education on proper sun protection of depigmented areas of the skin, including sunscreen and protective clothing. 

The differential diagnosis for piebaldism includes genodermatoses with defects in melanocyte development. Unlike piebaldism, these genodermatoses have associated extra-cutaneous manifestations and include[1]:

• Waardenburg syndrome (Types 1-4)
• Peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and Hirschsprung disease (PCWH)
• Tietz syndrome

The differential diagnosis also includes the following disorders of hypopigmentation and/or melanocyte destruction[1]:

• Vitiligo
• Nevus anemicus
• Nevus depigmentosus
• Tuberous sclerosis 
• Hypomelanosis of Ito
• Chromosome 4q12q21 deletions

Individuals with piebaldism will present at birth with stable leukoderma and poliosis. Unlike vitiligo, these areas of depigmentation do not progress after birth. However, reports exist of rare cases of spontaneous repigmentation.[3]

Isolated piebaldism has no known complications. 

• Upon making the diagnosis of isolated piebaldism, family members require reassurance that this is a benign condition that is unlikely to progress. 
• Due to the absence of the photoprotective effects of epidermal melanin, patients with piebaldism should receive education on proper sun protection of depigmented areas of the skin, including sunscreen and photoprotective clothing. 

• Piebaldism is a disorder of melanocyte migration and development
• A white forelock is present in approximately 90% of cases
• It is crucial to rule out associated syndromes at birth (e.g., Waardenburg)
• Treatment options include camouflage techniques, hair dyes, skin grafting, and cell transplantation

The management of piebaldism at birth requires an interprofessional team approach, including providers from dermatology, pediatrics, gastroenterology, and genetics. The reason for this approach is that this disorder is rare and acquired at birth. Secondly, it has features that mimic many other skin disorders, and often, an exhaustive workup is required. When the condition presents at birth or soon after, the patient should obtain a referral by the primary care provider to a dermatologist.

The first step is accurately diagnosing piebaldism as either isolated or part of a syndrome with extracutaneous manifestations. 

A dermatologist can help in the initial diagnostic workup to determine the cause (Wood's lamp evaluation, biopsy), the extent of lesions, and the presence of extracutaneous manifestations. Upon making the diagnosis of isolated piebaldism, family members need reassurance that this is a benign condition that is unlikely to progress. Affected individuals may experience significant social disability during childhood, adolescence, and adulthood. In these cases, referral to a mental health professional is a prudent course, and they can report back to the team on the results of therapy. Some patients may need cognitive behavior therapy. For the skin lesion, treatment to improve cosmesis can be sought with a dermatologist or plastic surgeon. 

Due to the absence of the photoprotective effects of epidermal melanin, patients with piebaldism should receive education from the pharmacist, nurse, or primary care provider on proper sun protection of depigmented areas of the skin, including sunscreen and protective clothing.

None of these actions can take place in a silo; all members of the interprofessional health team need to keep each other informed and up to date on their activities. While piebaldism is a benign condition, it does not obviate the health care team from collaborative activity. The education of the family must be coordinated by clinicians. [Level 5]