Cutis Tricolor Parvimaculata: A Distinct Neurocutaneous Syndrome with Brain Involvement: Case Report

Abstract

1410utis tricolor is an entity of a neurocutaneous syndrome that must be distinguished from tuberous sclerosis and neurofibromatosis. The characteristic lesions are hypo-and hyperpigmented macules, most likely representing twin spotting, located on normal skin.1,2 The location and size of the macules are extremely variable.3,4 Thus, it is clear that cutis tricolor is not one distinct clinical entity, it should rather be taken as a cutaneous sign of several different types of mosaicism.5 Cutis tricolor parvimaculata describes the smaller disseminated twin spotting different from commonly known lesions.5 Cutis tricolor may be associated with multisysCutis Tricolor Parvimaculata: A Distinct Neurocutaneous Syndrome with Brain Involvement: Case Report A AB BS ST TR RA AC CT T Cutis tricolor is a skin disorder characterized by the coexistence of congenital hypoand hyperpigmented maculer lesions, in close proximity to each other on a background of normal skin. Cutis tricolor parvimaculata describes the form consisting of smaller spots. These skin macules are called twin spotting and represent a part of a neurocutaneous malformation syndrome. Cutis tricolor may accompany various multisystem birth defects including craniofacial and brain abnormalities. It must be distinguished from other neurocutaneous syndromes such as tuberous sclerosis and neurofibromatosis. We described cutis tricolor parvimaculata in a 3-year-old girl, the reported youngest patient in the literature, with diffuse pigmentary spotting on the skin, facial anomalies, developmental delay and brain involvement. Hayrullah ALP,

A novel association of an uncommon pigmentation pattern: coexistence of cutis tricolor with intracranial teratoma and holoprosencephaly

“…Similarly, neurological manifestations could be explained by ataxia-telangiectasia in the case report by Khumalo et al [17]. In a more recent case report [15] enlarging subcortical and periventricular mass lesions were found on cranial MRI of a 3-year-old girl with a disseminated presentation of cutis tricolor. The authors used further imaging techniques and concluded that these lesions suggested tumoral growth, but couldn't prove their diagnosis because they weren't able to biopsy the lesions owing to their location.…”

“…Nicita et al emphasized in their article [5] that cutis tricolor is not a single entity. It has been reported in association with various abnormalities or as an isolated cutaneous finding (Table 1 [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]). Its coexistence with facial dysmorphism, neurological and behavioral abnormalities and skeletal dysplasia has been defined as Ruggieri-Happle syndrome [2][3][4][5].…”