Congenital myofibroma of the skin mimicking a piloleiomyoma

Myofibroma is an uncommon benign soft tissue disorder, which is usually congenital or present in early infancy. Myofibroma usually manifests as a single mass. When there are multiple lesions, the term myofibromatosis is used. The characteristic histopathological feature of the myofibroma is the coexistence of two distinct areas. One area mainly contains plump spindle cells with thin blunt‐ended nuclei and eosinophilic cytoplasm, thus indicating myoid characteristics. The other area contains either round or polygonal cells with slightly pleomorphic, hyperchromatic nuclei or small spindle cells typically arranged around a distinct hemangiopericytoma‐like vascular pattern. In the present case, the majority of the tumor was composed of the plump myoid spindle cells. This led to an initial diagnosis of a piloleiomyoma. However, the tumor cells were not immunohistochemically positive for desmin. Moreover, careful examination revealed a hemangiopericytoma‐like vascular pattern characterized by the presence of high cellular areas with irregular vascular spaces. These features led to the final diagnosis of the myofibroma. It is therefore important to recognize the leiomyoma‐like variants of myofibromas. Inoue T, Sada A, Mori T, Misago N, Narisawa Y. Congenital myofibroma of the skin mimicking a piloleiomyoma.     

Nipple leiomyoma: A rare neoplasm with a broad spectrum of histologic appearances

Cutaneous leiomyomas are rare benign smooth‐muscle tumors. These lesions are distinguished based on their cell of origin and are subclassified as pilar leiomyoma, angioleiomyoma, and genital‐type leiomyoma. Nipple leiomyoma is the least common genital‐type leiomyoma, arising from the dartoic muscle cell of the nipple. Histologic examination of the lesion is necessary for definitive diagnosis, and these uncommon tumors can pose a diagnostic challenge. We describe herein a series of six nipple leiomyomas with a spectrum of histologic appearances.     

Genital leiomyoma: surgical excision for both diagnosis and treatment of a unilateral leiomyoma of the male nipple

We describe a rare case of unilateral leiomyoma of the nipple in a man presenting with pruritus of the nipple for 3 months. A conservative surgical excision is performed for diagnosis. Histologic examination and immunostaining confirmed the diagnosis of leiomyoma. The leiomyoma is completely excised, and the patient denies pruritus after surgery. Conservative surgical excision of a persistently hard and pruritic nipple can be effectively used as both diagnostic measure and treatment modality.     

Ossified soft tissue leiomyoma in a patient with sickle cell anemia

Osseous metaplasia in leiomyomas is extremely rare. Here, we report the case of an ossified subcutaneous leiomyoma in a 34-year-old African American man with sickle cell thalassemia who presented with a painful nodule of the scapular region, which appeared as a heavily mineralized soft tissue mass on chest radiographs. Histopathologic and immunohistochemical examination of the resected nodule revealed a benign soft tissue leiomyoma composed of intersecting fascicles of spindle cells that strongly expressed smooth muscle actin and caldesmon. Extensive intratumoral calcification and ossification were noticed. Only eight cases of ossified leiomyoma have been reported, of which two arose in the deep soft tissue.     

Genital leiomyomas of the male nipple: two cases

BACKGROUND: Genital leiomyoma is a rare benign solitary skin tumor, not painful, developed from smooth muscle. Genital leiomyoma arising from the nipple is extremely rare, especially in males.CASES REPORT: A 47 year-old male had a 2 cm cutaneous plaque with nodules located on the right nipple. This plaque was circumscribed, erythematous, pruriginous and was not painful. The lesion had been noticed by the patient two years ago. A 37 year-old male showed a 1.5 cm cutaneous plaque located on the left nipple. The plaque was slightly erythematous, pruriginous, not painful and had been noticed by the patient 5 years earlier. Histology provided the diagnosis of genital leiomyoma in both cases. No surgical therapy was performed.DISCUSSION: Cutaneous leiomyomas are classified in 3 types regarding their origin: multiple or solitary piloleiomyoma, arising from arectores pilorum muscles, solitary genital leiomyoma, arising from the dartoic, vulvar, or mammillary muscles, and solitary angioleiomyoma, arising from the vein muscles. Clinically, genital leiomyoma is a 1 cm diameter solitary erythematous, firm nodule. According to many authors, genital leiomyoma is asymptomatic, but in the 2 patients, the lesions were pruriginous. Surgical excision is usually performed.     

A case of cutaneous symplastic leiomyoma - a rare variant of cutaneous pilar leiomyoma

We describe the case of a cutaneous symplastic leiomyoma in a 37-year-old woman who presented with a 4-year history of a painful slow growing lesion on the left upper arm. The lesion was excised and subjected to histological examination. A poorly circumscribed lesion was seen in the dermis composed of spindle shaped cells with marked nuclear pleomorphism. No mitotic figures or necrosis were seen. The cells stained strongly positive with desmin and smooth muscle actin, and negative with S100, melan A, MNF116 a mouse monoclonal antibody to cytokeratin and CK5/6. The diagnosis was felt to be in keeping with a cutaneous symplastic leiomyoma, a rarely reported variant of the pilar leiomyoma. Histologically, it shows features similar to the symplastic variant of uterine leiomyoma with cytological atypia, nuclear pleomorphism and minimal mitotic activity. Although the long-term outlook is probably benign, the presence of cytological atypia and mitoses in any spindle cell tumor is generally a concerning feature and warrants long-term follow up.     

Reiters' disease: a radiological assessment of joints

One married and one unmarried man with Reiters' disease are reported. The disease is characterized by well-recognized cutaneous lesions and conjunctivitis. However, affliction of the joints of the lower and upper extremities was striking. The conspicuous Roentgenographic features included extensive juxta-articular rarefaction, moderate to severe narrowing of joint spaces, subluxation/dislocation of small joints of the hands and feet, and fixed flexion deformity with ankylosis of the knees. Mutilating arthritis (Launois's deformity) of the feet was also contributory.     

Association of multiple familial cutaneous leiomyoma with a uterine symplastic leiomyoma

We describe a patient who has familial cutaneous leiomyoma in association with a symplastic uterine leiomyoma. This association has not been described previously.     

Solitary Pilar Leiomyoma of the Nasal Dorsum: Case Report and Literature Review

Cutaneous leiomyoma is an infrequently occurring benign tumor that arises from smooth muscle in the skin. Pilar leiomyoma, a subtype of cutaneous leiomyoma, arises from the arrector pili muscle associated with hair follicles. Pilar leiomyoma, particularly in the head and neck region, is rare and frequently misdiagnosed. We report one patient who developed pilar leiomyoma of the nasal dorsum, explore the differential diagnosis and review the characteristics of previously reported individuals with pilar leiomyoma of the nasal dorsum.     

Cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma: a rare occurrence and potential diagnostic pitfall

A case of cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma is described. The patient was a 60‐year‐old man who presented with a painless nodule on his chest. Thorough clinical examination did not reveal any evidence of tumor elsewhere. A punch biopsy was performed which showed a benign spindle cell neoplasm with focal cellular pleomorphism that had smooth muscle differentiation and was clearly originating from the arrector pili muscle, consistent with a cutaneous symplastic pilar leiomyoma. Immunohistochemical studies were performed and the tumor cells were strongly positive for smooth muscle actin (SMA) and desmin. Given the clinical information of a mass on the chest, clinical evaluation was recommended and a re‐excision was performed. Histologically, the re‐excision showed two distinct populations of cells. The upper portion of the tumor mirrored the initial biopsy; however, the base of the tumor showed hypercellular areas composed of spindle cells with marked pleomorphism and increased number of mitoses. The diagnosis of a cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma was given. This is the first report of this association of such occurrence reported in the literature. Fons ME, Bachhuber T, Plaza JA. Cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma: a rare occurrence and potential diagnostic pitfall.     

Leiomyomas in a naevoid distribution

A 59-year-old man developed gradually appearing and slowly progressive tender papulo-nodular lesions in a naevoid distribution on the left side of the trunk since the age of 49 years. The histopathological features were classical of leiomyoma.     

Hyperkeratotic lesion of the nipple revealing cutaneous leiomyoma

BACKGROUND: Leiomyoma of the nipple and areola is a rare benign neoplasm. We report the case of a patient with leiomyoma of the nipple presenting as a hyperkeratotic plaque. OBSERVATION: A 23-year-old patient presented with a five year history of a papillomatous, hyperkeratotic, painful plaque originating in her right nipple. Histological examination of a punch biopsy showed hyperkeratosis of the epidermis with dilatation of the lymphatic vessels within the dermis. Surgical excision revealed a proliferation of smooth muscle fibres, leading to diagnosis of leiomyoma. DISCUSSION: The clinical and histological features were initially consistent with idiopathic naevoid hyperkeratosis of areola. However, associated pain is uncommon in idiopathic lesions. This unusual feature led us to surgical excision enabling the diagnosis of leiomyoma. A hyperkeratotic lesion of the nipple may be associated with benign or malignant neoplasms, hamartoma or chronic dermatoses, or it may be idiopathic. In the present case, the hyperkeratotic lesion revealed subareolar leiomyoma. This is an uncommon clinical presentation not previously seen in medical observations, since leiomyoma usually presents as a firm, painful lump in the subareolar region.     

Kavernöses Riesenhämangiom der Kopfhaut

Cavernous hemangiomas of the scalp associated with vascular tumors of the skull occur rarely in older patients. Giant hemangiomas can cause complications secondary to extensive bleeding. Here we report on a 78-year-old female presenting with a giant ulcerated cavernous hemangioma of the scalp. Before admission to the hospital, she had suffered from severe bleeding of the vascular tumor, with resulting anemia. Because of her cardiovascular disease, the tumor mass, and possible vascular connections to osseous hemangiomas, complete surgical removal of the hemangioma was not indicated. Therefore the superficial ulceration was covered with a split skin transplant. After complete wound healing, no further bleeding occurred.     

面部多发性毛发平滑肌瘤一例

【摘要】 报告1例面部多发性毛发平滑肌瘤。患者男,43岁,右侧面部及额头部红色斑丘疹,伴冬季疼痛20年余,近4年加重。临床表现为粟粒至黄豆大小,高出皮面的丘疹和结节分布于右侧面颊部以及额部右侧,呈淡红色至红色,簇集状,质地坚实,基底稍呈浸润状。冰块实验阳性。患者无其他系统疾病。皮损组织病理检查：表皮大致正常,真皮内见肿瘤细胞不规则聚集或交织成束,肿瘤细胞细长,胞浆嗜酸,胞核两端钝圆或呈雪茄样,免疫组化显示平滑肌强阳性的特征：Desmin和SMA（+）,结合临床诊断多发性毛发平滑肌瘤。由于该患者皮损面积大,未能手术切除,随访。     

Symplastic hemangioma: report of two cases

Symplastic hemangioma is characterized by degenerative atypia of vascular smooth muscle and interstitial cells within a pre-existing vascular lesion with minimal endothelial cell atypia. We describe an additional two cases of this distinctive but poorly recognized entity. On histology, both lesions revealed a cirsoid aneurysm-type appearance with thick-walled and variably dilated blood vessels. The vascular endothelial cells showed mild nuclear hyperchromasia with no multilayering or mitoses. The atypical cells, either located within the vascular smooth muscle wall or within the interstitium, were spindle or epithelioid with varying degrees of hyperchromasia, nuclear enlargement, pleomorphism, and multinucleation. Perivascular hemorrhage, vascular thrombosis, and focal papillary endothelial hyperplasia were uniformly present. The variably fibrous to edematous stroma showed hemosiderin deposits and a mononuclear inflammatory infiltrate. Clusters of adipocytes were present within the superficial dermis. Rare atypical mitoses and occasional bizarre lipoblast-like stromal cells were identified in one tumor. Immunohistochemistry showed focal smooth muscle actin positivity in the pleomorphic cells of the vascular walls. CD68 and CD34 stained occasional stromal cells in the interstitial location. Both the cases showed no recurrence. The bizarre cytologic changes are interpreted as degenerative in nature and probably akin to that observed in ancient schwannoma and uterine symplastic leiomyoma.     

Leiomioma de la areola mamaria. Presentación de dos casos

—Cutaneous genital or dartoic leiomyomas are rare benign tumors originating in the smooth muscle fibers of the skin of the scrotum, vulva, nipple and areola. The latter are the less frequently observed.We report two cases of leiomyoma of the areola. Both presented as a solitary, small, nonulcerated nodule. Histopathological features shared similar characteristics: a non-encapsulated, ill-defined tumor in superficial and mid-dermis composed of spindle cells with elongated nuclei and eosinophilic cytoplasm, arranged in interdigitating fascicles. There were no atypia or mitoses. It was remarkable a reactive epidermal hyperplasia overlying the surface of the tumor, as is typically seen in the histiocytomas. Areolar leiomyoma is a benign lesion that is not known to undergo malignant transformation, although it may be confused clinically with carcinoma of the breast.     

Positive correlation of vanilloid receptor subtype1 and prostaglandin E2 expression with pain in leiomyomas

Cutaneous leiomyomas are benign smooth muscle tumors that are occasionally painful. The mechanism of pain related to leiomyoma is not fully understood. To investigate the possible involvement of algoneic factors in pain from cutaneous leiomyomas. We present a case of cutaneous leiomyoma with severe, diffused pain in a large area and collected 10 more specimens of cutaneous leiomyoma with or without pain in patient histories. We immunohistochemiacally examined the expression of algoneic factors: serotonin, histamin, Substance P, PGE2, BDKRB2, VR1 and CGRP. We compared the pain area and expression of algoneic factors to reveal possible correlations. We describe here a patient with a cutaneous leiomyoma 1‐cm in diameter, which caused severe pain diffused throughout an area of 20‐cm around the tumor. The pain completely resolved after surgical excision of the leiomyoma. We observed that the leiomyoma cells expressed CGRP, PGE2 and VR1 in this case. We found a positive correlation between VR1 and PGE2 expression in the leiomyoma cells and areas with pain around the tumors among 11 specimens in total. VR1 and PGE2 might be key algogenic substances in painful leiomyoma.     

Xerosis and callus formation as a key to the diabetic foot syndrome: Dermatologic view of the problem and its management

The diabetic foot syndrome is a major complication of diabetes mellitus. The two most important pathophysiologic factors are peripheral arterial occlusion and peripheral neuropathy. The cutaneous lesion is a plantar ulcer, often accompanied by soft tissue and bone infections which can require amputation. Triggers include poorly fitting shoes, poor foot care, or overlooked foreign bodies, often coupled with a structural foot deformity. Increased plantar pressure, especially beneath the metatarsal heads, and the resultant callus play an important role. The patients often already have xerosis of the plantar skin with scales, fissures, erosions and impaired barrier function, complicating the situation. Prompt neurologic and vascular diagnostic studies, coupled with routine examination of the feet and primary prophylactic measures are most important. The most important therapeutic goals are optimal control of the diabetes mellitus, relieving pressure points and avoiding or reducing callus formation.     

Acquired syphilis in a patient with late congenital syphilis

Late congenital syphilis was diagnosed in a 22-year-old woman on the basis of clinical, serologic and epidemiologic features. Clinical examination showed the "saddle-nose" deformity, Hutchinson's teeth, high-arched palate, Dubois little-finger sign, and small maxillae resulting in shallow depression of the central part of the face. Two years after receiving adequate treatment for late congenital syphilis, she presented with a primary syphilitic chancre; thus it was concluded that the woman had no immunity to reinfection with Treponema pallidum.     

管状鼻畸形的一期整复

目的描述一种罕见的管状鼻临床症状,探索一次手术完成管状鼻修复的技术。方法观察4例同一类型管状鼻患者的临床症状。以内眦血管为蒂,将管状鼻下移至患侧鼻部,用以修复患侧的鼻缺损,一次手术完成。结果4例均一期完成鼻的修复,转移的管状鼻组织均100%成活。结论 以内眦动脉为蒂,将位于内眦部的管状鼻带蒂转移至鼻部,并一期重建正常外鼻形态是完全可以的。