Case Report Dermatology 1992;185:59-61
R. A. F. L. G.
Cecchia Giomi3 T uct Brunetti3 Seghierib
Bilateral Segm ental Neurofibromatosis
Departments of Dermatology and Internal Medicine Spedali Riuniti. Pistoia. Italy
Abstract
Neurofibromatosis (NF) is a heterogeneous disorder clinically characterized by the presence of neurofibromas, multiple café au lait spots, intertriginous freckles and Lisch nodules. We describe an unusual case of NF with cutaneous neuro fibromas localized on the anterior chest, in a bilateral dermatomal distribution. No other signs were detected. The family history was negative for NF. The dif ferent subsets of NF are briefly discussed, with particular emphasis on the seg mental type.
Neurofibromatosis (NF) is now considered as a hetero geneous disease, clinically defined by the presence of neu rofibromas, multiple café au lait spots, intertriginous freckles and Lisch nodules [1]. Besides the classical autosomal dominant form, or von Recklinghausen’s disease, in most cases variable clinical expressions of NF may be present. Riccardi [1] described eight distinct subsets of NF, characterized by different clin ical and genetic features. In segmental NF the cutaneous lesions are limited to a single body segment, often in a uni lateral. dermatomal disposition [1,2]. In this communica tion we describe a patient affected by neurofibromas local ized on the anterior chest, in a bilateral segmental distribu tion.
Received: August 6. 1991 Accepted: December 16. 1991
Case Report A 45-year-old woman had multiple, isolated, papular and nodular red lesions on the anterior chest, which first appeared at the age of 35 years. In September 1990 the largest tumors were excised in a surgical department. Physical examination at the time of admission disclosed dark red, asymptomatic, small papular or nodular lesions and hyper trophic scars on both breasts in a bilateral distribution involving Th4 dermatomal segments. In addition comedones were present in some lesions as well as in uninvolvcd breast skin (fig. 1-3). No café au lait spots, axillary freckles or Lisch nodules were detected. The family history was negative for NF. Since the beginning of the disease the patient had noticed gradually increasing numbers of these lesions, limited to the same cutaneous areas. Complete blood cell count and chemical studies were normal, chest X-ray, a computed tomography scan of the head and nuclear magnetic resonance of the spinal cord were normal. A biopsy specimen of a cutaneous nodule showed a typical neurofibroma (fig.4).
Dr. Roberto Cecchi Divisionc Dermatológica Spedali Riuniti 1-51100 Pistoia (Italy)
© 1992 Kargcr AG. Basel 1018-8665/92/1851-
R. A. F. L. G.
Cecchia Giomi3 T uct Brunetti3 Seghierib
Bilateral Segm ental Neurofibromatosis
Departments of Dermatology and Internal Medicine Spedali Riuniti. Pistoia. Italy
Abstract
Neurofibromatosis (NF) is a heterogeneous disorder clinically characterized by the presence of neurofibromas, multiple café au lait spots, intertriginous freckles and Lisch nodules. We describe an unusual case of NF with cutaneous neuro fibromas localized on the anterior chest, in a bilateral dermatomal distribution. No other signs were detected. The family history was negative for NF. The dif ferent subsets of NF are briefly discussed, with particular emphasis on the seg mental type.
Neurofibromatosis (NF) is now considered as a hetero geneous disease, clinically defined by the presence of neu rofibromas, multiple café au lait spots, intertriginous freckles and Lisch nodules [1]. Besides the classical autosomal dominant form, or von Recklinghausen’s disease, in most cases variable clinical expressions of NF may be present. Riccardi [1] described eight distinct subsets of NF, characterized by different clin ical and genetic features. In segmental NF the cutaneous lesions are limited to a single body segment, often in a uni lateral. dermatomal disposition [1,2]. In this communica tion we describe a patient affected by neurofibromas local ized on the anterior chest, in a bilateral segmental distribu tion.
Received: August 6. 1991 Accepted: December 16. 1991
Case Report A 45-year-old woman had multiple, isolated, papular and nodular red lesions on the anterior chest, which first appeared at the age of 35 years. In September 1990 the largest tumors were excised in a surgical department. Physical examination at the time of admission disclosed dark red, asymptomatic, small papular or nodular lesions and hyper trophic scars on both breasts in a bilateral distribution involving Th4 dermatomal segments. In addition comedones were present in some lesions as well as in uninvolvcd breast skin (fig. 1-3). No café au lait spots, axillary freckles or Lisch nodules were detected. The family history was negative for NF. Since the beginning of the disease the patient had noticed gradually increasing numbers of these lesions, limited to the same cutaneous areas. Complete blood cell count and chemical studies were normal, chest X-ray, a computed tomography scan of the head and nuclear magnetic resonance of the spinal cord were normal. A biopsy specimen of a cutaneous nodule showed a typical neurofibroma (fig.4).
Dr. Roberto Cecchi Divisionc Dermatológica Spedali Riuniti 1-51100 Pistoia (Italy)
© 1992 Kargcr AG. Basel 1018-8665/92/1851-
