ABSTRACT
Papular elastorrhexis is a rare entity, possibly a form of connective tissue nevi, characterized by asymptomatic white papules on the trunk and extremities first appearing during childhood or adolescence. Histopathologically, the elastic fibers are decreased and may appear in thin and fragmented forms. This rare condition has clinical and histological findings that overlap with those of other connective tissue nevi, making diagnosis a challenge for dermatologists. Herein we report the case of a 27-year-old Korean female presenting with asymptomatic 2~5 mm sized whitish papules on both extremities and showing histopathologic findings of increased layers of collagen fibers and decreased and fragmented elastic fibers in the dermis.
Subject(s)
Adolescent , Adult , Female , Humans , Collagen , Connective Tissue , Dermis , Diagnosis , Diagnosis, Differential , Elastic Tissue , Extremities , NevusABSTRACT
BACKGROUND: Papular elastorrhexis (PE), eruptive collagenoma (EC), and nevus anelasticus (NA) are described as multiple small papules with decrease, fragmentation, or lack of dermal elastic fibers. These diseases are suggested to be the same entity. The change of collagen fibers in the conditions has not been addressed to date. OBJECTIVE: We compared the clinical features of the 3 diseases and investigated changes in the collagen fibers involved. METHODS: Twenty-four cases of PE, 12 cases of EC, and 2 cases of NA found in PubMed and the Korean database were reviewed. Changes in dermal collagen fibers in 10 cases with histological figures were investigated. RESULTS: There were significant similarities between the 3 entities in terms of their clinical features. Four patients with PE and 2 with EC with fine, dense collagen fibers were women who had multiple white to hypopigmented, slightly indurated to firm, millimeter-size papules on the trunk and/or extremities that progressed gradually after developing in the patients' first to third decades. CONCLUSION: The 3 conditions are the same clinical entity in our opinion; such cases with fine, dense collagen manifest typical features.
Subject(s)
Female , Humans , Collagen , Elastic Tissue , Extremities , NevusABSTRACT
BACKGROUND: Papular elastorrhexis (PE), eruptive collagenoma (EC), and nevus anelasticus (NA) are described as multiple small papules with decrease, fragmentation, or lack of dermal elastic fibers. These diseases are suggested to be the same entity. The change of collagen fibers in the conditions has not been addressed to date. OBJECTIVE: We compared the clinical features of the 3 diseases and investigated changes in the collagen fibers involved. METHODS: Twenty-four cases of PE, 12 cases of EC, and 2 cases of NA found in PubMed and the Korean database were reviewed. Changes in dermal collagen fibers in 10 cases with histological figures were investigated. RESULTS: There were significant similarities between the 3 entities in terms of their clinical features. Four patients with PE and 2 with EC with fine, dense collagen fibers were women who had multiple white to hypopigmented, slightly indurated to firm, millimeter-size papules on the trunk and/or extremities that progressed gradually after developing in the patients' first to third decades. CONCLUSION: The 3 conditions are the same clinical entity in our opinion; such cases with fine, dense collagen manifest typical features.
Subject(s)
Female , Humans , Collagen , Elastic Tissue , Extremities , NevusABSTRACT
Eruptive collagenoma is a rare acquired type of connective tissue nevus that predominantly consists of collagen. It usually presents as multiple, asymptomatic, firm, flesh-colored or pink nodules or plaques that are symmetrically distributed over the trunk and upper extremities. Four cases of eruptive collagenoma in linear or zosteriform arrangements have been reported in the English literature. A 16-year-old boy presented with multiple asymptomatic erythematous papules and nodules on the left side of his sternal margin and the posterior side of his left neck. A punch biopsy taken from one of the nodules on the chest showed thickened collagen fibers in the dermis. Masson-Trichrome staining revealed an increase in the collagen fibers, which were broad and arranged haphazardly, and the elastic staining indicated a reduction in the elastic fibers in the dermis, which are findings that are consistent with collagenoma. We report a case of eruptive collagenoma that presented in a linear arrangement, which is a rare distribution pattern for connective tissue nevi.
Subject(s)
Adolescent , Humans , Male , Biopsy , Collagen , Connective Tissue , Dermis , Elastic Tissue , Neck , Nevus , Thorax , Upper ExtremityABSTRACT
BACKGROUND: A collagenoma or connective tissue nevus of the collagen type is a hamartoma consisting predominantly of collagen. Collagenomas are divided into the inherited and acquired types. The acquired forms include eruptive and isolated collagenoma. However, few studies have assessed the characteristics of patients with acquired collagenoma. OBJECTIVE: The aim of this study was to analyze the clinical characteristics of patients with acquired collagenoma. METHODS: We reviewed the medical records and clinical photographs of 23 patients who had been diagnosed histopathologically with acquired collagenoma by biopsy during the last 12 years, from January 2002 to December 2013. In addition, 11 cases of eruptive or isolated collagenomas previously reported in the Korean literature were added. RESULTS: In total, 34 cases of acquired collagenoma were assessed. Of the 34 cases, 53% were men and 47% were women. The mean age at diagnosis was 20.0 years for the patients with eruptive collagenoma and 29.1 years for the patients with isolated collagenoma. In the cases of eruptive collagenoma, multiple, flesh-colored or whitish papules on the trunk were predominant. On the other hand, in the cases of isolated collagenoma, solitary, flesh-colored plaques on the palms or soles were common features. Collagenomas in most cases were asymptomatic (79.4%). However, some patients with collagenomas had other symptoms such as tenderness or pruritus. There was no traumatic history in any case. CONCLUSION: Our study showed that the clinical features of patients with acquired collagenoma share many similarities with those in previously reported studies, with some differences. This study is expected to help us understand and obtain more information on the clinical diagnosis of acquired collagenoma.
Subject(s)
Female , Humans , Male , Biopsy , Collagen , Collagen Type I , Connective Tissue , Diagnosis , Hamartoma , Hand , Medical Records , Nevus , PruritusABSTRACT
Multiple endocrine neoplasia type I or Wermer syndrome is characterized by primary hyperparathyroidism, enteropancreatic endocrine tumor, and a pituitary pathology. A 35-year-old male presented with visual field defects, hyperprolactinemia, and hypogonadism. He also had multiple infraumbilical skin-colored nodules. A syndromal association of Wermer syndrome was derived using the dermal, pituitary, parathyroid, and gastrointestinal hormonal manifestations of the tumor. The radiological and histological findings of lesion which underwent biopsy are discussed. The presence of collagenomas, lipomas, and hypopigmented macules in a patient with neuroendocrine symptoms should raise the suspicion of an underlying multiple endocrine neoplasia.
ABSTRACT
Collagenomas, or connective tissue nevi of the collagen type, are hamartomatous growths of otherwise normal collagen. They are divided into two groups: the inherited form that contains familial cutaneous collagenoma and shagreen patches in tuberous sclerosis, and the acquired form that contains eruptive collagenoma and isolated collagenoma. Among them, isolated plantar collagenoma with a cerebriform appearance is a relatively rare type of connective tissue nevus. It has been described as one of the major skin findings in Proteus syndrome. We present an unusual case of acquired isolated plantar collagenoma presenting as cerebriform hyperplasia, which was not associated with Proteus syndrome.
Subject(s)
Collagen , Connective Tissue , Hyperplasia , Nevus , Proteus Syndrome , Skin , Tuberous SclerosisABSTRACT
Eruptive collagenoma is an acquired connective tissue nevus composed predominantly of collagen. We present a case of eruptive collagenoma in a 13-years-old female who had asymptomatic multiple 3 to 9 mm sized flesh colored scattered papules on both limbs. In addition, the differential diagnosis and other types of cutaneous collagenomas are briefly discussed.
Subject(s)
Female , Humans , Collagen , Connective Tissue , Diagnosis, Differential , Extremities , NevusABSTRACT
Isolated collagenoma usually occurs on the plantar area and is frequently associated with Proteus syndrome. A few reports have described isolated collagenoma on extra-plantar areas such as palm, scalp, face, thigh and finger. Herein, we report a case of isolated collagenoma on the periungual area, which was confused with digital mucous cyst. A 26-year-old man visited our clinic with an asymptomatic, 0.8x0.7 cm-sized, firm, skin-colored nodule on the periungual area of the left fourth finger. The distal nail of the nodule showed a longitudinal groove. Histopathologic findings from periungual nodule showed thickened collagen bundles packing reticular dermis.
Subject(s)
Adult , Humans , Collagen , Dermis , Fingers , Nails , Proteus Syndrome , Scalp , ThighABSTRACT
Familial cutaneous collagenoma is a rare hereditary disease that is inherited in an autosomal dominant pattern. It is characterized by early onset of multiple, skin-colored, sometimes hypopigmented cutaneous nodules, which initially show a symmetrical arrangement on the trunk, and later on the neck and upper limbs. We report on a case of a 45-year-old female who presented with multiple oval to round hypopigmented papules measuring 5~15 mm on her trunk. Histopathologically, the lesions showed an increased amount of collagen fibers and decreased, fragmented elastic fibers in the dermis. The skin lesions were diagnosed as familial cutaneous collagenoma and no treatment was administered. To the best of our knowledge, our case is the first reported case of familial cutaneous collagenoma (FCC) in the Korean literature.
Subject(s)
Female , Humans , Middle Aged , Collagen , Dermis , Elastic Tissue , Genetic Diseases, Inborn , Neck , Skin , Upper ExtremityABSTRACT
Os colagenomas são nevos do tecido conjuntivo, em que ocorre, histologicamente, espessamento e proeminência da fibra colágena. Podem estar associados à esclerose tuberosa e à síndrome do colagenoma cutâneo familial. Em alguns casos, são adquiridos, como no colagenoma isolado e eruptivo. Descrevem os autores um caso de colagenoma eruptivo em um menino de 10 anos de idade, com história de aparecimento súbito de várias pápulas, cor da pele, na coxa direita, sem associação com doença concomitante, trauma e processo inflamatório local.
Collagenomas are connective tissue nevi in which occurs a significant thickening of collagen fibers. They may be associated with tuberous sclerosis or familial skin collagenoma syndrome. In some cases, may be acquired as an isolated and eruptive collagenoma. We describe a case of eruptive collagenoma on a ten years old boy with a sudden onset of multiple skin-colored papules on the right thigh, not associated with concomitant diseases, injury or local inflammatory process
ABSTRACT
Collagenomas, or connective tissue nevi of the collagen type, represent a hamartomatous overgrowth of normal collagen. Isolated collagenoma is rare and most commonly presents in childhood. We herein report a rare case of congenital isolated collagenoma on the scalp without any associated abnormality. A 4-year-old boy with normal development presented with 2x1.5 cm sized, soft, dome-shaped brownish nodule on the scalp. Histopathologic study revealed collagen bundles with thickened appearance, a condition consistent with collagenoma.
Subject(s)
Collagen , Connective Tissue , Nevus , Child, Preschool , ScalpABSTRACT
Collagenomas are considered to be connective tissue nevi composed predominantly of collagen. They can be hereditary or sporadic. We report herein a rare case of isolated collagenoma on the left side of the face and the left thigh without associated abnormalities, in a 30-year-old man. Histopathologically, the lesion consisted of thick collagen fibers, which was compatible with collagenoma.
Subject(s)
Adult , Humans , Collagen , Connective Tissue , Nevus , ThighABSTRACT
PURPOSE: Collagenoma is an intradermal harmatomatous collagen proliferation among connective tissue nevi of the skin. Although there are some reports of isolated collagenomas that occurred on the sole and the palm, collagenoms at the finger has not been reported in Korea. METHODS: An 11-year-old girl presented with a growing mass on the distal interphalangeal joint of the left 5th finger. It was a skin-colored, oval and mild tender mass. There were no associated cutaneous or systemic abnormal findings. RESULTS: The nodule, at the subcutaneous level on the distal phalanx, was completely removed by excision. Grossly it was covered with normal skin, and its surface was smooth having a definite margin with a size of 1.5x1.2cm. Histopathological examination, the epidermis showed to be normal, increased thickness of collagens arranged with a whirl formation was found in the dermis. No signs of cellular component increase were observed. CONCLUSION: Isolated collagenoma can be aroused as a solitary nodule in at any place of the body. We report a rare case of a female patient with an isolated finger collagenoma.
Subject(s)
Child , Female , Humans , Collagen , Connective Tissue , Dermis , Epidermis , Fingers , Hypogonadism , Joints , Mitochondrial Diseases , Nevus , Ophthalmoplegia , SkinABSTRACT
Eruptive collagenoma is an acquired connective tissue nevus without family history. It is typically described as numerous small papules or nodules on the trunk and arms with histopathological features of decreased or degenerated elastic fibers. We report a case of a 16-year-old male who presented with multiple asymptomatic 2 to 5 mm sized yellowish grouped papules on the left calf. Histopathologically, the lesion showed thickened homogenized collagen fibers highlighted by Masson trichrome stain and decreased and fragmented elastic fibers stained by Verhoeff-van Gieson stain. The skin lesion was diagnosed as eruptive collagenoma and no treatment was provided.
Subject(s)
Adolescent , Humans , Male , Arm , Azo Compounds , Collagen , Connective Tissue , Elastic Tissue , Eosine Yellowish-(YS) , Methyl Green , Nevus , SkinABSTRACT
Collagenomas or connective tissue nevi of the collagen type are hamartomatous growths of otherwise normal collagen. They can be hereditary or sporadic. We report an interesting case of isolated collagenoma confined to the right palm, without associated abnormalities, in a 6-year-old boy. To our knowledge, only one case of isolated palmar collagenoma has been reported in the English literature and this has not been described previously in the Korean literature.
Subject(s)
Child , Humans , Male , Collagen , Connective Tissue , NevusABSTRACT
Eruptive collagenoma is a connective tissue nevus, which can occur even if there is no family history of the condition. We report a case of eruptive collagenoma in a 44-year-old woman, who had had multiple, variable-sized, yellowish papules on her neck since childhood. The histopathologic findings showed thickened, homogenized collagen fibers and decreased elastic fibers.
Subject(s)
Adult , Female , Humans , Collagen , Connective Tissue , Elastic Tissue , Neck , NevusABSTRACT
Collagenoma is a hamartomatous lesion consisiting of proliferation of normal collagen tissue. We report herein a rare case of isolated plantar collagenoma without associated abnormalities. A 50-year-old male presented with an asymptomatic, 1.5x1.5cm sized, firm, dome-shaped tumor on his left sole. There were no associated cutaneous or systemic findings. Histopathologically, the lesion consisted of thick collagen fibers, which was compatible with collagenoma.