Age: 1.1
Sex: F
Vaccine: HIBV, MMR, PPV
Symptoms: Bloch-Sulzberger syndrome, Condition aggravated, Dermatitis bullous, Erythema, Papule
Clinical narrative: Initial unsolicited report received from the literature on 01 September 2016. INTRODUCTION Incontinentia pigmenti (IP; Bloch-Sulzberger syndrome) is an X-linked dominant genodermatosis that can have several cutaneous, dental, skeletal, neurologic, and ocular manifestations, including retinal detachment, seizures, paralysis, developmental delay, hair loss, and abnormal dentition. Lethal for affected males in utero, IP presents overwhelmingly in females as a result of mutant X chromosome inactivation. Lyonization of the X chromosome is what contributes to the reticular or whorled vesiculobullous pattern pathognomonic for IP. Eighty percent of IP patients carry mutations in the NEMO gene, which codes for nuclear factor kB (NFkB) essential modulator. NFkB is crucial for the regulation of tumor necrosis factor (TNF)-induced apoptosis. Reactivation is thought to occur when specific triggers (possibly infection, fevers, or vaccinations) reactivate pathways in residual mutant cells. This case involves a 13-month-old female patient who was vaccinated with a dose of MEASLES, MUMPS AND RUBELLA VACCINE (LIVE) (Measles, Mumps, And Rubella), HAEMOPHILUS TYPE B (HIB) VACCINE (Haemophilus Influenzae Type B) and PNEUMOCOCCAL CONJUGATE VACCINE (Pneumococcal Conjugate Vaccine) (Batch number, expiration date, dose, route and site of administration was not reported for all the vaccines) on 11 December 2012 for her 12-month immunizations. Patient's medical history included initial incontinentia pigmenti lesions involved the left thigh, extending to the groin, and her left flank as a neonate. After the vesiculobullous stage, she maintained stable whorled hyperpigmented patches for months, with slightly atrophic areas interspersed with prominent vasculature. Mild ocular findings were noted, which were checked regularly by the patient's ophthalmologist. Patient's mother had a personal history of incontinentia pigmenti and currently presents with both hyper- and hypopigmented patches on her torso and left thigh. Concomitant medications were not reported. On an unspecified date, in the 2 weeks after immunization, erythematous, vesicular plaques developed on patient's left hand and right foot. It was reported that first vesiculobullous lesions to appear had, according to the mother's report, subsequently transformed into verrucous papules. Later, examination of the left thigh and flank found slightly hyperpigmented, with prominent vasculature. Reactivation of incontinentia pigmenti after 12-month immunizations was diagnosed. According to the patient's mother, her previous immunizations had not resulted in similar lesions. It was reported that no diagnostic tests were ordered because she already carried a biopsy-supported Diagnosis of IP completed at birth, and the classic appearance of the reactivation lesions was sufficient to make the diagnosis. Corrective treatment was not reported. The outcomes of the events were not reported. Upon internal review, the company decided to consider the event serious because of important medical event Incontinentia pigmenti reactivation/vaccination-induced reactivation of IP. List of documents held by sender: none. Sender's Comments: Incontinentia pigmenti reactivation/vaccination-induced reactivation of IP was reported after administration of MEASLES, MUMPS AND RUBELLA VACCINE (LIVE) and HAEMOPHILUS TYPE B (HIB) VACCINE. In this literature patient's mother also had a personal history of IP and currently presents with both hyper and hypopigmented patches on her torso and left thigh. However, investigation results ruling out a concurrent infection and other etiologies would be helpful to further assess this case. More over patient received more than one vaccine simultaneously hence role of the particular vaccine cannot be assessed.