Zosteriform connective tissue nevus: a case report

Because of its distinctive clinical features and histopathological characteristics, zosteriform connective tissue nevus is considered a separate entity from other connective tissue nevi. Only two cases have previously been reported in the worldwide dermatological literature. Here we report a zosteriform connective tissue nevus in a 3-year-old boy with similar clinical presentation.     

Linear connective tissue nevus

Abstract:  Multiple connective tissue nevi in linear arrangements, which was previously described as zosteriform connective tissue nevus, is a rare variant of connective tissue nevus. We herein report the case of an 8-year-old Japanese boy with this disease. He developed a small mass on the upper region of his right inner ankle at the age of one-half years. Then, other lesions had appeared on the inner side of his right lower leg, thigh, and groin within the next 2 years. Furthermore, multiple new lesions in linear arrangements had appeared on the right half of the abdomen and on the extensor side of the right forearm at the age of 8 years. A biopsy specimen revealed abnormalities in both the collagen bundles and the elastic fibers. Since the distribution was along Blaschko's lines, not dermatomes, we propose that the diagnosis "linear connective tissue nevus" is suitable for this clinical study.     

Cerebriform connective tissue nevus of lumbar

Connective tissue nevi represents a kind of hamartoma, and coalescence of the lesions in a cerebriform mode in the lumbar region without Proteus syndrome is rarely seen. Here, we report a 26‐year‐old woman presenting with nodules and plaques in her left lumbar region of 26 years in duration. Histopathological examination and Masson‐trichrome stain showed increased dermal collagen bundles in a haphazard array. The diagnosis of connective tissue nevi was made. This is the first case report on cerebriform connective tissue nevi without Proteus syndrome in the lumbar region.     

A new type of connective tissue nevus: isolated exophytic elastoma

An unusual case of isolated exophytic elastic tissue nevi in the scrotal region of a 64-year-old man is described. The histological and ultrastructural findings were those of abundant abnormal elastic fibers and increased reticulin in the dermis. This hamartomatous lesion possesses clinical and histological features previously undescribed in connective tissue nevi.     

Multiple connective tissue nevi

Connective tissue nevi are uncommon, and rarely suspected clinically because of their diverse morphologic presentations. Histologically, we define connective tissue nevi as discrete areas within the papillary or recticular dermis where a clear predominance or depletion of collagen, elastin, or glycosaminoglycans may be found. We report a case of multiple connective tissue nevi with a predominance of dermal collagen deposition, without extracutaneous findings and no family history of connective tissue nevi. These lesions can thus be classified as being of the eruptive collagenoma type.     

Connective tissue nevi collagens. Study with picrosirius red and polarizing microscopy.

Biopsy specimens of five connective tissue nevi were examined under crossed polars after staining with Picrosirius red. One biopsy specimen was from a solitary nevus, another from a Shagreen patch. The other three specimens were of erupted nevi. In all cases, thick (as well as thin) collagen fibers appeared green to yellow. In contrast, thick fibers of normal human dermis appeared orange to red. The findings indicate that the collagen of collagenous connective tissue nevi is less well packed than normal collagen. Examination of the polarization colors of Picrosirius red-stained sections is a useful procedure for diagnosing collagenous connective tissue nevi.     

Hair Follicle Nevi and Accessory Tragi: Variable Quantity of Adipose Tissue in Connective Tissue Framework

Abstract: Controversy exists about the histologic differences between hair follicle nevi and accessory tragi. We examined 10 congenital lesions histologicaiiy, possible diagnoses of which were hair follicle nevi or accessory tragi. Two specimens out of the 10 had tiny, mature hair follicles surrounded by thick fibrous root sheaths, a few fat cells, and no cartilage. The subcutaneous fat cells of their bases were segmented by a connective tissue framework. They had histologic features of hair follicle nevi. One specimen had cartilage and abundant fat cells with a connective tissue framework in the nodule, as well as a conglomeration of numerous well-differentiated hair follicles. It possessed both elements of a hair follicle nevus and an accessory tragus. Seven specimens had abundant subcutaneous fat and showed a prominent connective tissue framework. These were typical accessory tragi. The present study suggests that the number of fat cells in the nodule or papule differs between these two conditions. All the lesions studied revealed a connective tissue framework in the subcutaneous fat. Histologic features of both hair follicle nevi and accessory tragi can coexist in a single lesion. Hair follicle nevi may represent incomplete accessory tragi with scant fat cells.     

Buschke-Ollendorff syndrome: three generations in a Japanese family

Buschke-Ollendorff syndrome is an autosomal dominant disease characterized by disseminated connective tissue nevi of elastic type and osteopoikilosis. We report a 6-year-old Japanese boy with connective tissue nevi that showed slightly grouped yellowish or skin-colored papules and nodules, distributed from birth over his right thigh, right buttock, and back. Radiologic skeletal survey revealed osteopoikilosis. A skin biopsy specimen obtained from a papule showed that collagen bundles in the dermis were thickened and homogenized. The elastic fibers were not increased but were coarse and clumped. The boy's father, at age 34, has had osteopoikilosis and similar papules and nodules on his left buttock and back for the preceding 18 years. We studied the paternal grandfather, aged 65. He had osteopoikilosis and similar skin lesions on his lumbar region. None of the three had a history of hearing loss or malignant tumor. To our knowledge, this is the first report of three generations of Buschke-Ollendorf syndrome in a Japanese family.     

Familial cutaneous collagenoma: a clinicopathologic study of two new cases

Two siblings with familial cutaneous collagenoma syndrome had the essential clinical features of multiple skin-colored nodules on the trunk and upper arms. On light microscopy, histopathologic findings included excessive accumulation of dense, coarse collagen in the dermis. Elastic tissue stains demonstrated a proportionately diminished number of abnormal elastic fibers intermingled with the collagen bundles. A predominance of densely packed collagen bundles of normal morphology with a marked decrease in abnormal elastic tissue were the major ultrastructural features. The diagnosis was therefore confirmed to be connective tissue nevi of the collagen type. The differential diagnosis of connective tissue nevi disorders is delineated.     

Isolated Collagenoma: A Case Report with a Review of Connective Tissue Nevi of the Collagen Type

A 22-year-old male had had five skin colored tumorous growths on the mid-palm, ring and little fingers of the right hand for the last 15 years. Histopathological examination with hematoxylin and eosin and special stains confirmed the diagnosis of connective tissue nevi of collagen type. There were no associated cutaneous or systemic findings; thus the case was designated as isolated collagenoma. A review of connective tissue nevi of the collagen type is presented.     

Blaschkitis in adults

Many dermatoses may exhibit lesions distributed in a linear fashion: the linearity may be related to anatomical structures (blood or lymph vessels, nerves), to mechanical factors (urticarial dermographism, striae distensae cutis, frictional melanosis) or to some other provocative injuries being themselves linear (for instance K?bner's phenomenon). Some congenital or acquired dermatoses, either inherited or sporadic, have a linear distribution following the embryonic lines described in 1901 by A. Blaschko. Most of them are nevoid skin lesions present at birth or having a later onset: epidermal nevi (naevus unius lateris, linear porokeratosis), adnexal nevi (linear sebaceous nevus, linear basal cell nevus), pigmented lesions (systematized linear achromic nevus) or intricated nevi of the connective tissue (angio-lipomatous nevus). More seldom, genodermatoses related to a X-chromosomal mosaicism, occurring in females only, exhibit also a linear arrangement of their skin symptoms following Blaschko's lines: incontinentia pigmenti, focal dermal hypoplasia, etc. There are also very common inflammatory skin diseases, without any genetical background, which sometimes present as linear dermatoses. Their pattern according to Blaschko's lines is obvious in some cases: linear lichen planus, linear lichen nitidus, linear scleroderma, linear (zosteriform) vitiligo, linear fixed drug eruption, linear chronic lupus erythematosus. In some other conditions, the arrangement following these lines is probable (lichen striatus, linear eczema) or dubious (unilateral nevoid telangiectasias). This case report concerns a 38-year old man exhibiting relapsing inflammatory linear lesions of Blaschko's lines on the chest, back and upper limbs. Histological examination showed a non specific spongiotic dermatitis with rare necrotic keratinocytes and exocytosis of lymphocytes in the stratum spinosum.(ABSTRACT TRUNCATED AT 250 WORDS)     

Depressed indurated plaque with elastorrhexis as a distinctive lesion in Buschke-Ollendorff syndrome

Buschke-Ollendorff syndrome (BOS) is a rare autosomal dominant genodermatosis caused by heterozygous mutations in LEMD3 and characterized by connective tissue nevi and sclerotic bone lesions known as osteopoikilosis. We report a family with three individuals affected by BOS, two of whom manifested clinical and histopathological peculiarities, presenting with a depressed indurated plaque as the main cutaneous manifestation instead of the classic connective tissue nevi. Notable elastorrhexis was present in both biopsies.     

A case of congenital mucinous nevus: a connective tissue nevus of the proteoglycan type

Mucinous nevus is a very rare entity and can be classified as both a cutaneous mucinosis (CM) and a connective tissue nevus (CTN). We describe the clinicopathologic features of an unusual case of mucinous nevus in a 14-year-old Korean boy who presented with zosteriform plaques of congenital onset.     

Zosteriform connective tissue nevus in a pediatric patient

Abstract:  We report an 8-year-old girl affected by hypochromic, asymptomatic, acquired lesions with a paving-stone aspect on the right lumbosacral area and proximal right leg. The results of serum and urine biochemical screening were normal, as was the bone survey A biopsy was performed. The clinical and histologic aspects led to the diagnosis of connective tissue nevus with zosteriform distribution.     

Trichoepithelioma and Pigmented Nevus. A Combined Malformation

The occasional occurrences of combined hamartomas composed of nevus cell nevi and adnexal tumors are not chance happenings. Pigmented nevi are malformations consisting of nevus cells together with various connective tissue and epithelial components. The proliferation of these components results in the combined malformations, whose occurrence, we believe, is more supporting evidence for the idea that pigmented nevi may have other components besides nevus cells.     

Eruptive Cutaneous Collagenoma: Report of Two Cases

Introduction:Eruptive cutaneous collagenoma is non familial connective tissue nevi of unknown etiology presented with an abrupt onset.To date,the literatures on...     

Nevus Lipomatosus Cutaneus Superficialis (Hoffmann-Zurhelle)

We are reporting seven cases with nevus lipomatosus cutaneus superficialis. A review of the literature indicates that this is a relatively rare type of connective tissue nevus with little more than 50 cases recorded. Localization of the cutaneous nodular lesions over the upper thigh and buttock and histologic findings of fat lobules within the substance of the nevoid malformation differentiate these lesions from other varieties of the connective tissue nevi.     

变形综合征1例

患儿男,2岁。右侧躯干、肢体肥大2年。手足脑回状结缔组织痣,腹部表皮痣,右侧肢体不成比例的过度生长,脂肪组织不规则分布,脉管畸形。皮损组织病理示:真皮浅中层见大量的成熟的脂肪组织。符合变形综合征的诊断标准。     

Agminated Fibroblastic Conective Tissue Nevus: A New Clinical Presentation

Connective tissue nevi are benign hamartomatous lesions in which one or several of the components of the dermis (collagen, elastin, glicosaminoglycans) show predominance or depletion. Recently, de Feraudy et al broadened the spectrum of connective tissue nevus, describing fibroblastic connective tissue nevus (FCTN), which is characterized by proliferation of CD34⁺ cells of fibroblastic and myofibroblastic lineage. Only solitary papules and nodules have been described. We present the first case of FCTN with multiple agminated lesions on the leg of an infant and the difficulties encountered in the differential diagnosis with dermatofibrosarcoma protuberans.     

Giant fibromyxoid tumors of the adventitial dermis. Forme fruste of trichodiscoma?

A 36-year-old paraplegic man developed multiple giant fibromyxoid tumors surrounding a meningomyelocele scar that we believe are forme fruste of trichodiscomas. Histologic examination showed a papillated, slightly hyperplastic epidermis surrounding a delicate fibrovascular proliferation set in a stroma of loose fibrillar connective tissue and stellate fibroblasts. A melanocytic nevus was incorporated into one of the lesions. We believe that these giant lesions are connective tissue nevi of the adventitial dermis that comprise a new clinical variant of trichodiscoma. The potential significance of the relationship between these abortive trichodiscomas and abnormalities of the neural crest is discussed.