Incontinentia pigmenti

Incontinentia pigmenti

Chris Schach

Author Bio -

-Red -Present at birth or within the first 2 weeks of life in 90% of patients

Stage 2: Verrucous

-Wart-like lesions
-May be present at birth but in 70-80% of patients evolves after the first stage

Stage 3: Hyperpigmented

-Skin is darkened in a swirled pattern
-Hyperpigmentation tends to fade slowly 

Stage 4: Hypopigmented

-Light colored lesions develop during adolescence and persist into adulthood
-Occur in 30-75% of patients


Key Points
*Initial diagnosis is based on appearance of the affected area and clinical examination
*Skin Biopsy may be performed to confirm diagnosis and rule out other conditions
*Other tests

Initial diagnosis of Incontinentia pigmenti is based on the appearance of the affected areas in a female infant in conjunction with clinical examination. A skin biopsy may be performed to confirm the diagnosis and to rule out the presence of other conditions. Other tests


Differential Diagnosis:

Stage 1 Lesions:  Arthropod reaction

Stage 2 Lesions: Linear epidermal nevus

Stage 3 Lesions: Linear and whorled nevoid hypermelanosis


*No definitive treatment exists
*Goal of treatment is supportive; control stmptoms and prevent secondary infections

There is no direct treatment to combat Incontinentia pigmenti. The goal of treatment is to prevent secondary infection and track the development of the disease. Regular dental care and persistent examination by an opthamologist for the first few years of the affected person's life is recommended to assist in prevention.