Isolated plantar collagenoma: a case report

Collagenoma is a hamartomatous lesion consisting of proliferation of normal collagen tissue. We describe a 19-year-old girl with a firm, elastic 3 x 2 cm nodule located on her right plantar fossa. Histopathologically, dense, coarse, thick collagen fibers were located in the dermis. In addition, the number of elastic fibers was slightly decreased. Based on these findings, the case was diagnosed as isolated plantar collagenoma.     

Papular elastorrhexis. report of five cases

Papular elastorrhexis is a rare disorder that occurs predominantly during adolescence. We report 5 patients with asymptomatic white, nonfollicular, firm papules scattered over the trunk and extremities. Histologically, the papules demonstrate focal areas of collagen homogenization with decreased and fragmented elastic fibers. The clinical differential diagnosis includes papular acne scars, dermatofibrosis lenticularis disseminata (Buschke-Ollendorff syndrome), cutaneous collagenoma and nevus anelasticus, but histology clearly separates papular elastorrhexis from the other entities.     

Light and electron microscopy of eruptive collagenoma

Connective tissue nevi may be multiple or solitary, sporadic or familial. Eruptive collagenoma is a variant of the acquired collagenomas characterized by multiple sclerotic papules with an acute onset. A 13-year-old girl reported that in the past year, small asymptomatic lesions began to appear in her skin, 30 lesions were seen in the trunk, 5 in the cervical region and 1 in the face. Light microscopy with hematoxylin and eosin staining showed sparse collagen fibers, with Weigert staining diminished elastic tissue was observed. Scanning electron microscopy of the dermis showed individualized collagen fibers forming waved compact masses and not bundles. Transmission electron microscopy also showed sparse and loose collagen fibers with different diameters in cross sections.     

成人泛发性环状肉芽肿1例

患者女,57岁。全身出现散在环状或半环状红色浸润性斑块1年。皮损组织病理示:真皮浅层胶原纤维变性及肉芽肿形成;弹力纤维染色示:有少量弹力纤维断裂。阿新蓝染色示:胶原束间见嗜碱性黏液性物质沉积。诊断:泛发性环状肉芽肿。     

Familial cutaneous collagenoma: new affected family with prepubertal onset

Two siblings presented with clinical and histopathological findings of familial cutaneous collagenoma which is a rare connective tissue nevus, inherited in an autosomal-dominant pattern. A 13-year-old girl had oval-round, soft, painless papules, 5-10 mm in size and a total of 9-10 on her abdomen and flanks. Skin biopsy demonstrated dense, coarse collagen fibers in the dermis and a decrease in elastic fibers. Doppler echocardiography indicated an atrioseptal defect of the secundum type. Her 9-year-old brother was also examined; four lesions were discovered on his back but he was otherwise normal. Our cases comprise the sixth affected family to be reported in the medical published work and all lesions had appeared prepubertally.     

A case of eruptive collagenoma localized on the neck and shoulders

A 78-year-old woman, who had first noticed asymptomatic eruptions on her neck and shoulders eight years earlier, presented with papules and nodules 2 to 20 mm in diameter that had a normal to white hue and were flatly elevated. These lesions were scattered and multiple, some forming confluent plaques. Histopathologically, the epidermis was slightly atrophied, and collagen fibers in the dermis were coarse and proliferated. In addition, the number of elastic fibers was slightly decreased. No complications were evident. Based on these findings, the patient was given a diagnosis of mild eruptive collagenoma, a type of connective tissue nevus according to the classification of Uitto. This case is unique in that onset was at an advanced age and that distribution was localized on the neck and shoulders.     

The Importance of Collagen Tissue in Papular Elastorrhexis,Eruptive Collagenoma,and Nevus Anelasticus

BackgroundPapular 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.ObjectiveWe compared the clinical features of the 3 diseases and investigated changes in the collagen fibers involved.MethodsTwenty-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.ResultsThere 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.ConclusionThe 3 conditions are the same clinical entity in our opinion; such cases with fine, dense collagen manifest typical features.     

Acrokeratoelastoidosis of Costa: a primary disease of the elastic tissue?

A 24-year-old black woman from Uganda was seen for treatment of multiple papules on her hands and feet. The lesions corresponded microscopically to foci of hyperkeratosis and acanthosis. Acid orcein stain revealed marked elastic fiber fragmentation. Acrokeratoelastoidosis of Costa (AKE) was diagnosed. The same damage to the elastic fibers was also present in an additional specimen from grossly uninvolved skin. On electron microscopy there were pronounced changes of the elastic fibers with elastolysis in both specimens. This case with generalized damage of the dermal elastic tissue supports the view that elastorrhexis is the key feature of AKE. Accordingly, the disease could be regarded as a primary elastic tissue disorder. The marginal acral keratoderma that is seen in AKE patients could represent epidermal changes secondary to chronic trauma.     

Nevus anelasticus: how should such lesions be classified?

Nevus anelasticus represents a rare entity that is most commonly classified as a connective tissue nevus. It typically presents before 20 years of age with asymmetrically distributed white‐to‐skin‐toned or pink‐to‐red papules or plaques on the trunk and upper extremities. The lesion is defined histopathologically by the absence or degeneration of elastic fibers in the dermis. We report the case of a healthy 17‐year‐old female who presented with an asymptomatic slowly progressive plaque on the right inferior areola. Histopathologic examination showed the absence of elastic fibers in the papillary and upper reticular dermis and fragmented elastic tissue fibers in the deep reticular dermis. Although there is ongoing controversy regarding the nosology of this uncommon disorder, we propose that it is a distinct entity based on its histopathologic and clinical features.     

Nevus anelasticus, papular elastorrhexis, and eruptive collagenoma: clinically similar entities with focal absence of elastic fibers in childhood

Focal absence of elastic fibers is a defining characteristic of three rare, clinically similar conditions: nevus anelasticus, papular elastorrhexis, and eruptive collagenoma. We present a 16-year-old with nevus anelasticus treated with intralesional steroid injections, resulting in erythematous atrophic depressions. We also review the English language literature on nevus anelasticus, papular elastorrhexis, and eruptive collagenoma. There may be sufficient clinical and histopathologic similarity to indicate that these three entities represent a single disease or disease spectrum. We believe that this entity should be referred to as papular elastorrhexis.     

发疹性皮肤胶原瘤1例

报告发疹性皮肤胶原瘤1例,患者男,38岁。背部,肩部出现多发性扁平丘疹,结节及斑块8年,皮损为正常肤色,直径数毫米至数厘米,部分融合成斑块,无自觉症状,无家族史,组织病理检查示,胶原纤维变厚,增殖,弹性纤维减少,诊断为发疹性皮肤胶原瘤。     

Eruptive collagenoma. Apropos of a case with ultrastructural study

A 29-year-old male patient was examined for numerous asymptomatic dermal nodules symmetrically distributed over the back and the shoulders. The nodules were firm, round to oval and varied in size from a few millimeters to 2 centimeters. They had appeared in normal skin without any known previous inflammation or injury, and they has been present without modification for nine years. At histological examination a dermal accumulation of abundant and large collagen bundles was observed. Elastic fibers seemed to be less numerous than normally. Alcian blue showed no increase in acid mucopolysaccharides. An ultrastructural study displayed normal, mature collagen fibers arranged in large bundles. Cutaneous collagenoma, initially described by Colomb as "eruptive collagenoma" is a rare disorder of unknown etiology occurring in young adults as symptomless dermal nodules without previous history of illness or injury and mostly localized to the back. No change is usually observed in these nodules for several years. Histologically, the nodules are characterized by a proliferation of collagen in the dermis; elastic fibers are generally reduced in number. Our ultrastructural study showed that proliferation of normal collagen fibers is the main morphological finding.     

Solitary giant cell collagenoma

Collagenoma or collagen nevus of the connective tissue is a hamartomatous malformation of the dermis, characterized by a proliferation of normal collagen tissue. This may be congenital or acquired, solitary or multiple, hereditary or sporadic. The solitary type are sporadic, not associated with any disease, and may appear on any part of the body. Histology studies show an increase of collagen fibers in the dermis and a variable decrease in elastic fibers. We discuss the case of a child with a solitary giant cerebriform collagenoma on the lumbar region of her back.     

Buschke-Ollendorff syndrome--differential diagnosis of disseminated connective tissue lesions.

We report a 5-year-old boy presenting with multiple elastic type nevi and osteopoikilosis who was diagnosed as having Buschke-Ollendorff syndrome at an early age. Connective tissue lesions may present as the main symptom of varying clinical entities with different outcomes. Differential diagnosis includes papular elastorrhexis, fibroelastolytic papules of the neck, papular acne scars, and late onset focal dermal elastosis. Rare genodermatoses, i.e. Buschke-Ollendorff syndrome, pseudoxanthoma elasticum, juvenile hyaline fibromatosis and familiar cutaneous collagenoma should be carefully evaluated to provide appropriate genetic counseling and to avoid unnecessary treatment procedures.     

d-Penicillamine induced elastosis perforans serpiginosa with involvement of glans penis

Elastosis perforans serpiginosa (EPS) is an unusual perforating disorder characterized by extrusion of altered elastic fibers through the epidermis. Clinically, it presents as keratotic papules with a tendency for serpiginous or annular distribution that most commonly involves the sides of the neck and the back. However, involvement of the penis has rarely been reported. We present a case of EPS involving the neck, axilla, and glans penis in a 42-year-old man who had received long-term d-penicillamine treatment for Wilson disease. Skin biopsy revealed perforating channels containing numerous altered elastic fibers, with a characteristic “bramble brush” or “lumpy-bumpy” appearance as demonstrated by an elastin stain. The latter is thought to be pathognomonic for penicillamine-induced degenerative elastosis. These degenerative changes occurring in glans penis have rarely been described in the literature. Prompt recognition of the rare presentation could lead to early discontinuation of the offending drug, to prevent further sequelae.     

A Case of Fibroelastolytic Papulosis on the Neck of a Young Man

Fibroelastolytic papulosis of the neck (FEPN) encompasses a spectrum of two disorders that were previously reported as pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) and white fibrous papulosis of the neck (WFPN). The clinical presentation of FEPN is asymptomatic to mildly pruritic whitish-yellow papules that may coalesce into cobblestone patterned plaques that resemble pseudoxanthoma elasticum (PXE). The histology is characterized by a decrease or loss of elastic fibers in the papillary dermis and this is sometimes accompanied by a minimal or nodular increase of dermal collagen fibers. We report here on a 28-year-old Korean man with asymptomatic, multiple, skin-colored to slightly yellowish, match-head sized, cobblestone-patterned papules on the neck, and these were histologically consistent with FEPN and the papules showed slightly increased dermal collagen associated with decreased and fragmented elastic fibers, elastin and tropoelastin. The pathogenesis of FEPN in this case might have been related with mild dermal inflammation, followed by fragmentation, elastolysis and increased dermal collagen.     

Acquired perforating calcific collagenosis after topical calcium chloride exposure

A 24‐year‐old healthy man presented with a 6‐week history of numerous umbilicated coalescing erythematous papules with some scale and crust on his anterior medial thighs. The eruption began 1 to 2 weeks after he spilled calcium chloride rock salts on his pants while salting the sidewalk during a snow storm. The salts dissolved and remained in contact with his skin for at least 4 hours until he was able to change clothes. A skin biopsy shows thick and thin collagen fibers with partial calcification in the papillary and upper reticular dermis associated with a sparse infiltrate of neutrophils, lymphocytes and mononuclear histiocytes. There are foci of transepidermal elimination of calcified fibers with adjacent epidermal hyperplasia and ortho‐ and parakeratosis. Von Kossa stain highlights calcification of the fibers, and trichrome stain confirms the fibers are collagen. A Verhoeff‐van Gieson stain shows no abnormality of elastic fibers. The patient was treated with topical betametasone diproprionate cream twice daily for 3 weeks, as well as a short course of oral levofloxacin and topical gentamicin cream. The lesions resolved over 3 weeks with residual scarring. We report a unique case of acquired perforating calcific collagenosis secondary to topical calcium chloride exposure. Patel RR, Zirvi M, Walters RF, Kamino H. Acquired perforating calcific collagenosis after topical calcium chloride exposure.     

Atrophic dermatofibroma. Elastophagocytosis by the tumor cells

A 55-year-old woman presented with an asymptomatic red plaque on the left upper back for 6 or 7 years. The lesion was depressed in response to finger pressure. The clinical diagnosis was anetoderma. Histopathologically, the characteristic cells of cellular dermatofibroma proliferated within the thinned dermis, which showed atrophy of about 60 or 70%. The proliferated cells were positive for factor XIIIa and negative for CD34. The involved dermis showed the loss of elastic fibers on elastica van Gieson stain. Electron microscopically, the proliferating cells phagocytized the elastic fibers. We report a typical case of atrophic dermatofibroma and show the possibility that the cause of this disease might be elastophagocytosis between the collagen fibers by the dermatofibroma cells.     

Annular erythematous papules in the neckline

A 45-year-old woman with personal history of hypertension presented with an erythematous lesion in the neckline for a year and with a progressive growth. A physical examination revealed an annular lesion with erythematous papules in the edge. Histological exam showed phagocytosis of elastic fibers by multinucleated cells compatible with annular elastolytic giant-cell granuloma. The patient did not present any other associated systemic manifestation. Treatment with tacrolimus 0.1 percent ointment was prescribed with a very good response after two months.     

Focal acral hyperkeratosis associated with pitted keratolysis

Focal acral hyperkeratosis is characterized by the same clinical appearance as acrokeratoelastoidosis, but without abnormalities in the elastic fibers. We present the case of a woman with a 10-year case of dermatosis localized on the palms, soles and dorsum of the metacarpophalangeal joints, consisting of multiple polygonal papules and associated hyperhydrosis, clinically compatible with acrokeratoelastoidosis. Her father had a history of the disease. In addition, the patient presented with palmoplantar pitted keratolysis. The histopathological study ruled out elastorrhexis, and the pitted keratolysis was corroborated by the clinical appearance and the presence of coccoid elements in the stratum corneum, evident with a PAS stain. In our opinion, the focal acral hyperkeratosis is not a separate entity from the acrokeratoelastoidosis.