All That Is Vesicular Is Not Herpes: Incontinentia Pigmenti Masquerading as Herpes Simplex Virus in a Newborn

Abstract

ABSTRACT. Incontinentia pigmenti is a multisystem genodermatosis characterized by cutaneous, neurologic, ophthalmologic, and dental abnormalities. The skin lesions associated with the disease progress through 4 stages, the first being erythematous vesicles linearly distributed along the lines of Blaschko. We report a case of an infant who had incontinentia pigmenti and presented with 2 crops of vesicles and was initially thought to have herpes simplex virus. Pediatrics 2004;114:e270 -e272. URL: http://www.pediatrics.org/cgi/content/full/114/ 2/e270; incontinentia pigmenti, herpes simplex virus, Block-Sulzberger syndrome.ABBREVIATIONS. IP, incontinentia pigmenti; HSV, herpes simplex virus; CNS, central nervous system; WBCs, white blood cells.

Incontinentia pigmenti in the neonatal period

“…IP is a rare genodermatosis characterised by abnormalities of the tissues and organs embryologically derived from ectoderm and neuroectoderm 1–3 6–8. The designation of IP is derived from the accumulation of melanin in the deep dermis as a free pigment and melanophages 3 4 6.…”

“…The designation of IP is derived from the accumulation of melanin in the deep dermis as a free pigment and melanophages 3 4 6. Originally described by Garrod in 1903 and Bloch in 1926, its pathogenesis has been clarified in 1928 by Sulzberger and cutaneous location by Happel in 1985 2 3 5 6 9…”

“…It is most often found in females with familial transmission in 50% of the cases 6 7 9. IP type 1 or hypomelanosis, can occur in most cases as a sporadic mutation through Xp11.21 3.…”

Incontinentia Pigmenti Mimicking a Herpes Simplex Virus Infection in the Newborn

“…[5] In newborns, many examples of erroneous diagnoses of vesiculopustular disorders, made as a result of suspicious findings, can be found in the literature, Herpes simplex being the most common one. [6,7,8] Neonatal HSV infection can show up any time from soon after birth up to and beyond the neonatal period. Seventy-five to 90% of infants with neonatal HSV are born to women with no history or physical findings suggestive of genital herpes.…”

Reintroducing the Tzanck Smear

“…[35] Because the disease progresses in a manner that mimics the erythematous, linearly distributed vesicles of herpes virus, the two diseases can be grossly indistinguishable. In fact, Faloyin et al [36] report a case of a female infant noted to have two linear crops of vesicles on her right arm and leg, suggesting herpes viral infection, yet all laboratory tests and cultures of the blister fluid were negative for HSV. Biopsy, on the other hand, showed eosinophilic spongiosis with dyskeratotic epithelial cells.…”