ABSTRACT
Leopard syndrome is a neurocutaneous syndrome which may affect a variety of organ systems in the embryological aspects. It presents lentigines, EKG abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retardation of growth, and deafness as major teatures.We report a case of Leopard Syndrome with atypical psychotic features. The patient had many lentigenies on the face, cafe au lait spots on the part of the waist and the buttocks, mild atrial regugitation, clinodactyly, hyperextensibility of distal interphalangeal joint, flat foots, subclinical hypothyrodism, sensorineural deafness, and mild mental retardation. He also had autistic disorder, compulsion, pathologic collection, and violent behaviors as psychiatric problems. This raises a possibility that psychiatric diseases may be related to the variation of neuroectoderm. In clinical practice, when psychopathic patients with specific skin lesions are seen for consultation, the consideration of embryologically common aspects of both disease groups can be helpful to the discovery of abnormalities in other organs and to its treatment.
Subject(s)
Humans , Autistic Disorder , Buttocks , Cafe-au-Lait Spots , Deafness , Electrocardiography , Flatfoot , Genitalia , Hypertelorism , Intellectual Disability , Joints , Lentigo , LEOPARD Syndrome , Neural Plate , Neurocutaneous Syndromes , Panthera , Psychotic Disorders , Pulmonary Valve Stenosis , SkinABSTRACT
We report a case of a 27-year-old male showing Becker's nevus on his trunk and lower extremities. It represented symmetrically bilateral and extensive(about 50% of body surface) involvement. In addition to the usual clinical findings of Becker's nevus, grouped small darker spots were noted on the lesion of the buttock featuring agminated lentigine. Histologic findings of the brownish pigmented lesion consisted of mild acanthosis, hyperkeratosis, elongation of rete ridges, increase of basal layer pigmentation, and numerous terminal hair follicles. A dark macule in agminated lesions on the buttock showed more dense basal layer pigmentation and more regular elongation and fusion of rete ridges consistent with the histologic findings of lentigo simplex. This report describes a rare case of a giant Becker's nevus involving 50% of body surface in a bilateral and symmetrical distribution with associated agminated lentigine.
Subject(s)
Adult , Humans , Male , Buttocks , Hair Follicle , Lentigo , Lower Extremity , Nevus , PigmentationABSTRACT
We report a case of a 27-year-old male showing Becker's nevus on his trunk and lower extremities. It represented symmetrically bilateral and extensive(about 50% of body surface) involvement. In addition to the usual clinical findings of Becker's nevus, grouped small darker spots were noted on the lesion of the buttock featuring agminated lentigine. Histologic findings of the brownish pigmented lesion consisted of mild acanthosis, hyperkeratosis, elongation of rete ridges, increase of basal layer pigmentation, and numerous terminal hair follicles. A dark macule in agminated lesions on the buttock showed more dense basal layer pigmentation and more regular elongation and fusion of rete ridges consistent with the histologic findings of lentigo simplex. This report describes a rare case of a giant Becker's nevus involving 50% of body surface in a bilateral and symmetrical distribution with associated agminated lentigine.