Case Reports of Incontinentia Pigmenti in Males
A 5-years-old Muslim male child presented with cutaneous hyperpigmentation on trunk, extremities, and face.\nThe boy was an only child, born to third degree consanguineous parents with full-term normal delivery. There was no history of abortion or death of a male child previously. Family history was non-conclusive.\nAs informed by mother, the skin lesions started appearing at 1 week of age in the form of vesicular lesions on the lower extremities, which gradually spread onto the trunk. These were followed by crusted plaques, which healed with hyperpigmentation.\nOn examination, the child had slate brown hyperpigmented macules on face and lower limbs in linear distribution. Widely distributed areas of pigmentation in whorled pattern were seen on trunk []. Oral cavity showed hypodontia with peg-shaped teeth []. The physical and intellectual development of boy was considered subnormal for age by pediatrician. His developmental milestones were delayed for age, and he had complaints of primary nocturnal enueresis. Systemic examination including ocular, central nervous system (CNS), and skeletal systems revealed nothing abnormal. Hair and nails were normal. Hematological tests showed peripheral blood eosinophilia. CT scan brain was normal. 2D Echo revealed patent ductus arteriosus.\nHistopathological examination revealed mild epidermal hyperplasia (acanthotic) with dyskeratotic cells with few whorls of squamous cells with central keratinization. Dermis reveals infiltrates of eosinophils and mononuclear cells.