Abstract
Familial progressive hyperpigmentation (FPH) is a rare genodermatosis characterized by hyperpigmented patches in the skin and mucous membranes, present in early infancy, and increase in size and number with age. The genetic basis for FPH remains unknown. We report an unusual case of familial progressive hypermelanosis in a 17-year-old male patient with family history, who presented with a peculiar progressive oral pigmentation disorder. Diagnosis was confirmed by a series of hematological, biochemical, and histopathological investigations. Our paper stresses the need for the dentist to be aware of the systemic conditions that can also manifest in the oral cavity.
Highlights
Familial progressive hyperpigmentation (FPH) is a rare genodermatosis first reported by Chernosky et al in 1971 [1]
Melanosis diffusa congenita, universal acquired melanosis, and familial universal or diffuse melanosis are just some of the other terms coined by various authors to describe patients with a generalized diffuse hypermelanosis without systemic symptoms, but often with a familial pattern [2]
This confirmed the provisional diagnosis of familial progressive hyperpigmentation (FPH)
Summary
Familial progressive hyperpigmentation (FPH) is a rare genodermatosis first reported by Chernosky et al in 1971 [1]. Mode of inheritance is still debated upon since a number of inheritance patterns are recognised, mainly: (1) autosomal dominant (Chernosky et al 1971 [1], Rebora and Parodi 1989 [4], Debao and Ting 1991 [5]); (2) autosomal recessive with germ-line mosaicism (Wende and Baukus 1919, Pegum 1955) [3]. Though such cases have been previously reported in dermatology, no such case has been reported in dental literature even though there are significant oral findings in these patients
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