ABSTRACT
Partial unilateral lentiginosis is a rare pigmentary disorder which is characterized by multiple grouped lentigines in unilateral or segmental distributions. The ocular involvement of the disease is extremely rare and only four cases have been reported until today. The exact pathophysiology is still unknown. An 18-year-old woman is being presented with unilateral hyperpigmented macules on her left forehead, eyelid, cheek, lip and chin which all demarcated sharply at the midline of her face. The lesion has been presented since the age of 1. Also, there are discrete brownish pigmentations on her left bulbar conjunctiva. Biopsy specimen is being obtained from the left chin and the histopathological examinations revealed increasing basal layer of pigmentations and mild elongation of rete ridges. The histopathological features are also consistent with lentigo simplex. Herein, we present a rare case of partial unilateral lentiginosis with an ocular involvement. We have also proposed that the partial unilateral lentiginosis has a possibility of ocular involvements.
Subject(s)
Adolescent , Female , Humans , Biopsy , Cheek , Chin , Conjunctiva , Eyelids , Forehead , Lentigo , Lip , PigmentationABSTRACT
La lentiginosis unilateral parcial (LUP) es un raro desorden pigmentario caracterizado por numerosos lentigos, dispuestos en grupo, que se presentan durante la infancia, asintomáticos, y comprometen de manera unilateral un segmento del cuerpo: cabeza, cuello, tronco, extremidades, etc. Pocas veces sobrepasan la línea media, permaneciendo estables en el tiempo. Presentamos cinco casos clínicos y una breve revisión del tema...
Partial Unilateral Lentiginosis (PUL) is a rare pigmentarydisorder characterized by numerous asymptomatic andstable lentigines confined to one side of the body.It begins in childhood, affecting different body segmentsincluding the head, neck, trunk and extremities.Lentigines rarely surpass the midline.We present five cases and briefly review the subject...
Subject(s)
Female , Lentigo , Extremities , Head , Neck , Skin , Skin DiseasesABSTRACT
Partial unilateral lentiginosis (PUL) is an unusual pigmentary disorder that's characterized by multiple lentigines in a unilateral distribution. The lesions often have a segmental pattern with a sharp demarcation at the midline. This is sometimes combined with other disorders such as neurofibromatosis or cafe-au-lait macules (CALMs). The presence of multiple CALMs in the same distribution as the lentigines on a PUL patient makes it difficult to differentiate PUL from segmental neurofibromatosis. We present here a 25-year-old woman with an unusual combination of several caf?-au-lait macules and scattered numerous lentigines involving the left side of the abdomen, flank and back.
Subject(s)
Adult , Female , Humans , Abdomen , Lentigo , NeurofibromatosesABSTRACT
The term, bilateral segmental neurofibromatosis had been used to refer to patients who had unilateral neurofibromas with contralateral pigmented lesions or bilateral neurofibromas. Partial unilateral lentiginosis is characterized by numerous lentigines localized to a body segment. The coexistence of bilateral neurofibromas and partial unilateral lentiginosis raises the possibility that partial unilateral lentiginosis could be a variant of segmental neurofibromatosis. Nevus of Ota arise from dermal melanocytes and it can be associated with neurofibromatosis. A 60-year-old man presented with bilateral segmental neurofibromatosis with partial unilateral lentiginosis on the right arm and right leg, nevus of Ota on right forehead, and one cafe-au-lait spot on the abdomen.
Subject(s)
Humans , Middle Aged , Abdomen , Arm , Cafe-au-Lait Spots , Forehead , Leg , Lentigo , Melanocytes , Neurofibroma , Neurofibromatoses , Nevus of Ota , NevusABSTRACT
Bilateral segmental neurofibromatosis is a rare disease characterized by bilateral neurofibromas, with or without pigmented lesion, or unilateral neurofibromas with contralateral pigmented lesion, limited to a body segment. Partial unilateral lentiginosis is characterized by numerous lentigines localized to a body segment, often corresponding to one or more dermatome. Bilateral segmental neurofibromatosis combined with partial unilateral lentiginosis is very rare, and to our knowledge, only 2 cases have been reported in English literature. We herein report another case of bilateral segmental neurofibromatosis with partial unilateral lentiginosis in a 46-year-old woman.
Subject(s)
Female , Humans , Middle Aged , Lentigo , Neurofibroma , Neurofibromatoses , Neurofibromatosis 1 , Rare DiseasesABSTRACT
Partial unilateral lentiginosis is a rare pigmentary disorder characterized by the numerous unilateral lentigines on the otherwise normal skin. A 13-year-old woman presented with asymptomatic multiple brownish macules on the left periorbital area. Her mother and maternal grandfather had the same clinical features on the left abdomen. Histologic examination of the pigmented lesion revealed findings consistent with lentigo simplex. We report an interesting case of partial unilateral lentiginosis with familial occurrence.
Subject(s)
Adolescent , Female , Humans , Abdomen , Lentigo , Mothers , SkinABSTRACT
BACKGROUND: Partial unilateral lentiginosis (PUL) is a rare pigmentary disorder characterized by grouped multiple lentigines on otherwise normal skin that histologically have the typical features of lentigo. This entity has been only rarely reported in the Korean population. OBJECTIVE: The purpose of this study was to evaluate clinical and histopathologic characteristics, association with other disorders, and differential diagnosis of PUL. METHODS: We reviewed our experiences of thirteen cases of PUL which had been collected in our dermatology clinic during the 6-year period between 1993 and 1998. RESULTS: Twelve patients were female and one was male. In 3 patients the lesions appeared after the age of 20 years. Ten patients had the lesions on the upper part of the body, the neck being the most common location. No bias was shown in terms of the side of the body affected. Cafe-au-lait macules (one to three) were found in six patients, axillary freckles were observed in two. Histopathologic examination of biopsy specimens commonly showed hyperpigmentation of the basal layer, elongation of rete ridges, and an increased number of melanocytes. There ,was no evidence of associated disorders or family history. CONCLUSION: Based on this data, we confirmed that PUL is a benign, idiopathic lentiginosis with no commonly associated abnormalities. Furthermore, we believed that PUL is not uncommon in Korean people.
Subject(s)
Female , Humans , Male , Bias , Biopsy , Dermatology , Diagnosis, Differential , Hyperpigmentation , Lentigo , Melanocytes , Melanosis , Neck , SkinABSTRACT
Partial Unilateral Lentiginosis (PUL) is a rare pigmentary disorder characterized by the numerous lentigines confined to a body segment, with a sharp demarcation at the midline. We report two cases of PUL. A 38-year-old woman had asymptomatic discrete small hyperpigmented macules that were scattered on the T7~L1 dermatomes on the left side of her trunk and were clearly demarcated in the midline on both anterior and posterior sides. In the other case, an 18- year-old woman had hyperpigmented macules that were scattered on the left neck, shoulder, and anterior chest. Other anomalies including neurofibromatosis, neurologic anomalies, and multiple lentiginous syndrome were not related in both cases. We report two cases of PUL having no other anomalies.
Subject(s)
Adult , Female , Humans , Lentigo , Neck , Neurofibromatoses , Shoulder , ThoraxABSTRACT
Partial unilateral lentiginosis(PUL) is a rare pigmentary disorder charaeterized by lentigines limited to one side of the body with or without neurologic abnormalities. We report two cases of PUL unassociated with any other defect, One patient was 20-year-old female who had lentigines confined to the right side of the face and the other was 19-year-old male who had lentigines confined to the left side of the neck and upper trunk and left upper extremity. Histologic examination of the pigmented lesion revealed findings consistent with lentigo simplex.