Extramammary Paget's disease

Two patients with extramammary Paget's disease are being reported. The first case was a 60-year-old male who presented with gradually progressive pruritic ulcerated lesion over perianal region not responding to various topical medications. Dermatological examination revealed a large erythematous sharply marginated scaly plaque in the perianal region extending into the anal canal, covered with grayish crusts and shallow erosions. Skin biopsy from the lesion was suggestive of Paget's disease. The second patient, a 60-year-old male, with a three year long history, presented with similar lesions and findings. Skin biopsy in this case was also consistent with Paget's disease. These two cases of extramammary Paget's disease involving the perianal region are being reported because of their rarity and also to stress the fact that a high degree of suspicion is needed for the diagnosis of this uncommon disease entity.     

Perianal nodule in a young woman

A young woman presented with a perianal nodular lesion, which was found to have histopathological findings of hidradenoma papilliferum. The anal skin is an uncommon location for this neoplasm.     

Recurrent solitary giant trichoepithelioma located in the perianal area; a case report

A case of giant solitary trichoepithelioma of the perianal region is described. Following excision the lesion recurred with a similar histological appearance. This is the first report, to our knowledge, of recurrence of such a lesion.     

Eccrine nevus presenting as a perianal skin tag: a case report and review of the literature

We present the case of a 9-year-old girl with a perianal skin tag. This asymptomatic lesion was removed for cosmetic reasons and demonstrated a polypoid lesion with a slightly acanthotic epidermis. The dermis was filled with mature-appearing eccrine ducts and glands but with no other cutaneous appendages, adipocytes, or abnormal vascularity. We believe this to represent a polypoid eccrine nevus and that its presentation is unique.     

Perianal dermatofibrosarcoma protuberans: a case report

A 67-year-old man presented with a perianal lump that had increased in size. On examination he had a 3-cm irregular, mobile, elevated, red, polypoid lump at the edge of the anus at the 8-o'clock position. Biopsy results unexpectedly revealed a spindle cell lesion extending deep into the subcutaneous tissue with occasional mitoses. The lesion was positive for CD34 and negative for epithelial markers, consistent with dermatofibrosarcoma protuberans (DFSP). Magnetic resonance imaging of the pelvis showed the mass extending deep into the ischiorectal space with no involvement of the external or internal anal sphincter. He underwent excision of the lesion with circumferential margins of 1 cm and formation of a skin rotation flap to achieve primary closure. Histology confirmed DFSP. Both the deep and lateral resection margins were involved. He proceeded to have a wider excision of margins, which was free of any remaining tumor. Dermatofibrosarcoma protuberans is a rare lesion. It most commonly occurs on the trunk; the perianal presentation in this case is unique. Surgical excision and preservation of functionality with cosmesis was an issue in this case, as DFSP is a locally aggressive tumor with a high recurrence rate.     

Extramammary Paget's disease associated with stomach cancer

A case of extramammary Paget's disease in the perianal region is reported. The disease presented as a whitish lesion with central erosion. Examination revealed an underlying adenocarcinoma of the stomach. The disease was treated by cryosurgery.     

A Case of Tuberculosis Cutis Orificialis with Perianal Involvement

Tuberculosis cutis orificialis (TCO) is a rare manifestation of cutaneous tuberculosis that is caused by auto-inoculation of mycobacteria in patients with advanced internal tuberculosis. TCO occurs in oral, perianal, or genital mucosa and adjacent skin. The tongue is the most frequently affected site, but the perianal area can also be affected. A 39-year-old male presented with a 4-month history of painful ulcers on the perianal area. The histopathologic findings revealed granulomatous infiltrates composed of epithelioid cells and Langhans-type giant cells in the dermis, and a few acid-fast bacilli noted on Ziehl-Neelsen staining. The polymerase chain reaction (PCR) was positive for Mycobacterium tuberculosis and the chest X-ray showed findings consistent with active pulmonary tuberculosis in both upper lung zones. The skin lesion showed complete resolution 2 months after the start of treatment with antituberculosis agents. We report a case of TCO with perianal involvement in a patient with underlying active pulmonary tuberculosis.     

Perianal abcess in infant

INTRODUCTION: Perianal abcess and fistula-in-ano are particular when happening in infants of less than 1 year of age. A congenital abnormality of crypts of Morgagni may be at the origin of the disease. There is no evidence for predisposing condition at this age. CASE REPORT: A 7-week-old boy had a painful swollen perianal lesion suggesting the presence of perianal abcess. Biological tests did not show any neutropenia nor inflammatory syndrome. The infant was admitted to the surgical center to undergo a perianal abcess drainage. During the intervention, no fistula was found. No recurrence was observed. DISCUSSION: Perianal abcess and/or fistula-in-ano are relatively common conditions in infants of less than 1 year of age. This disease is suspected to originate from anal cryptitis, which will later form a perianal abcess. Androgen excess during the foetal stage could be the cause of the formation of abnormal crypts of Morgagni, which encourages cryptitis and abcess formation. In childhood some predisposing factors can exist, as immunodeficiency or colopathy. On the opposite, a predisposing condition is rare in infancy. Twenty-eight to 85 per cent of infants with perianal abcess may progress to form a fistula. The usual treatment of perianal abcess is incision and drainage; it may be completed by fistulotomy or fistulectomy. The antibiotherapy is not systematic. The identification of the corresponding crypt may be important to avoid recurrence. On the opposite, a recent prospective study proposes a non operative management of perianal abcess and fistula-in-ano in healthy infants.     

Simulatoren des chronischen Analekzems

Several premalignant and malignant neoplasms clinically appear as chronic eczematous lesions of the perianal region and have to be distinguished from benign processes. Anal intraepithelial neoplasia (AIN) presenting as bowenoid papulosis and perianal Bowen disease is a precursor lesion for invasive squamous cell carcinoma. When AIN is widespread, persistent or progressive, an underlying immunosuppression should get excluded. Verrucous carcinoma of the perianal region is a highly differentiated variant of squamous cell carcinoma characterized as a slowly growing but locally invasive tumor. Extramammary Paget disease (EMP) is an intraepithelial adenocarcinoma of the skin with apocrine differentiation. Along with EMP, basal cell carcinoma, Langerhans cell histiocytosis and cutaneous T-cell lymphoma should be included in the differential diagnosis for lesions simulating chronic perianal dermatitis. In such situations, biopsies and histopathologic examination are imperative to avoid delayed diagnosis and assure prompt therapy.     

Infantile perianal pyramidal protrusion: report of a case and review of the literature

Infantile perianal pyramidal protrusion is an important, relatively newly recognized condition, which is thought to be under-reported in the pediatric literature. Previous reports refer to these lesions as skin tags or skin folds. More recently, the name infantile perianal pyramidal protrusion has been suggested, based on the characteristic presentation of the lesion in prepubertal children, especially girls. Recognition of these lesions has many implications regarding proper diagnosis and management. They may easily be mistaken for condyloma accuminata or as a sign of trauma, leading to an investigation of sexual abuse. This situation may be avoided with awareness of this entity in combination with thorough history taking and a classic presentation. We present a classic presentation of infantile perianal pyramidal protrusion and further discuss this condition.     

Squamous cell carcinoma in a renal transplant recipient with linear porokeratosis.

A 40-year-old man developed squamous cell carcinoma on a perianal lesion of linear porokeratosis after renal transplantation. The tumor metastasized to the left inguinal lymph node 25 months after the primary tumor was excised. p53 overexpression was observed in the tumor cells, but not in the porokeratotic lesion. Interestingly, continuous subcutaneous infusion of peplomycin for the lymph node metastasis significantly improved the warty lesions of porokeratosis. In this patient, immunosuppressive agents might have accelerated the development of carcinoma on a skin area with malignant potential.     

Human papillomavirus type 16 in a homosexual man. Association with perianal carcinoma in situ and condyloma acuminatum.

BACKGROUND--The association of anal carcinoma with human papillomavirus (HPV) type 16 infection is well documented. Anal carcinoma is also frequently associated with a history of anogenital condylomata. More than 90% of anogenital condylomata contain HPV type 6 or 11. It is rare for a condylomatous lesion to contain HPV 16. We report the unusual case of a homosexual man, testing positively for human immunodeficiency virus, with carcinoma in situ evolving within perianal condylomata infected with HPV 16. OBSERVATIONS--Microscopic examination of tissue specimens from ulcerated verrucous lesions on the perianal mucosa revealed changes of classic condylomata acuminata with contiguous focal squamous cell carcinoma in situ. Testing for HPV DNA by in situ hybridization identified HPV 16 in both the condylomatous and carcinoma in situ areas. CONCLUSIONS--The association of HPV 16-infected condylomata and adjacent carcinoma in situ implies that cutaneous genital condylomata may progress to high-grade lesions. Given that homosexual men are at high risk for perianal carcinomas, HPV typing of perianal condylomata specimens may help identify immunocompromised patients who are at risk for the development of carcinomas.     

光动力疗法治疗乳房外Paget病临床研究

目的 探讨荧光诊断指导下光动力治疗肛周皮肤湿疹样癌的临床意义。方法 肛周Paget病6例,前期3例行普通光动力治疗(PDT),后期3例行荧光诊断指导下光动力治疗,普通PDT组给以光敏剂Photofrin 2mg/kg,48h后给以功率100～150mW /cm2、能量密度150～300J/cm2,波长630 nm半导体激光照射病灶;荧光诊断指导PDT组（FPDT）给以局部20％ALA湿敷（激光照射前3h）和静脉给光敏剂喜泊分5mg/kg（激光照射前48h）,并给以同样剂量激光照射,激光照射前给以405nm蓝紫光行荧光诊断确定病灶的范围以指导治疗。治疗后48h和72h, 1、3和6个月观察患者病灶局部反应并长期随访。结果 治疗后48h 2组均可见病灶局部明显变暗、发黑、变硬,120h局部开始结痂,渗出减少,皮肤修复时间长。治疗后6个月普通PDT组CR1例,PR2例;FPDT组CR2例,PR1例。结论　光动力学疗法是肛周Paget病的有效治疗方法,荧光诊断对于确定病灶范围、指导临床治疗意义重大。     

Primary cutaneous B-cell lymphoma successfully treated with highly active antiretroviral therapy alone: a case report and review of the literature

Cutaneous B-cell lymphoma (CBCL) is an unusual skin neoplasm with a great range of clinical presentations. Here, we report a case of CBCL in an AIDS patient presented as a single and nodular/ulcerative lesion in the perianal area. The patient was started on highly active antiretroviral therapy alone with a good clinical and oncological response. Two years later, the patient is asymptomatic with undetectable viral load and immune reconstitution.     

Giant Condyloma Acuminatum in an Infant

Abstract:   We report a 10-month-old boy who presented with a giant perianal condyloma acuminatum, and a similar lesion on the neck. These lesions were treated by surgical excision with satisfactory results. This size, extent, and early age of appearance make this case highly unusual.     

Perianal verrucous epidermal nevus masquerading as warts

Verrucous epidermal naevus(VEN) is a rare form of epidermal naevus. We present a case of VEN occurring over the perianal region of a 7-year-old boy. The lesion was initially thought to be an area of chronic dermatitis; however it was refractory to treatment. Histopathology confirmed the diagnosis of VEN. VEN in the inguinogenital region may be misdiagnosed as flexural psoriasis, genital warts or sexual abuse. This is what precisely happened to our patient for a duration of more than 1 year.     

Atypical apocrine proliferation involving anogenital mammary-like glands of the perianal region

Anogenital mammary-like glands (MLGs) are a normal constituent of the anogenital area showing similarities to breast glands. MLGs are recognized to be the possible origin for various neoplastic and reactive conditions that show homology to their mammary counterparts. We report the case of an 85-year-old woman presenting with 10 cm polypoid mass of the perianal region. Histopathological examination of the excised lesion showed atypical apocrine proliferation arising in a complex lesion with features of fibroadenoma, adenosis and hyperplastic and cystic change. Normal MLGs were observed at the tumor periphery. There was no recurrence after 3 years of follow up. This report represents an illustration of the complexity of lesions developed from MLG.     

Rectal carcinoma associated with pagetoid phenomenon

A 64-year-old woman was referred to our hospital with complaints of erosion in the anal region and rectal bleeding. The histopathological examination revealed Paget cells in an epidermis lesion of the skin and rectal carcinoma. The cells were positive for PAS, CEA, and CK20, and negative for GCDFP15 and CK7. Electron microscopic examination revealed the presence of many microvilli in the epidermal Paget cells as well as in the tumor cells and rectum itself. The results suggested that electron microscopy is a very useful technique to differentiate extramammary Paget's disease and pagetoid phenomenon. In the present case, the perianal skin lesion was proved to be due to intraepidermal spread of the rectal carcinoma, or so-called pagetoid phenomenon.     

Perianal hidradenitis suppurativa: early surgical treatment gives good results in chronic or recurrent cases

Hidradenitis suppurativa (HS) represents a chronic, recurrent, deep-seated folliculitis resulting in abscesses followed by the formation of sinus tracts and scarring. It is an uncommon disorder frequently involving the perianal region. We review the clinical and pathological features of 12 patients (10 men and two women) with perianal HS (PHS). The mean age at presentation was 43 years (range 24–65), and the mean duration of illness was 14 years (range 2–30). Two patients exhibited multiple areas of involvement, including the axilla and the nape of the neck, in addition to the perianal region. Histological examinations showed epidermis-lined sinuses surrounded by dense plasma cell infiltration in four of nine specimens. The complications related with PHS included anal fistula (seven patients), diabetes mellitus (four), malignant tumour (two) and sepsis (one). Three patients died of the complications. Eight patients underwent wide local excision. The defects were managed with a meshed split-thickness skin graft in five patients, and open treatment in the remainder. New lesions developed in untreated skin of two patients. However, no patient relapsed in the surgically treated sites. PHS is frequently complicated with anal fistula and is occasionally life-threatening as a result of the occurrence of malignant tumours or sepsis. Early surgical excision followed by meshed split-thickness skin graft can successfully cure the lesion without functional disturbance.     

Perianal Striated Muscle Hamartoma Associated with Hemangioma

Abstract: Striated muscle hamartoma is a rare entity which was described only recently. It is a congenital malformation characterized by the presence of striated, mature muscle fibers in the reticular dermis and hypodermis. We describe a striated muscle hamartoma of the perianal region in a female infant associated with an hemangioma in the same area that regressed completely. Including the present patient, only 14 cases of striated muscle hamartomas have been reported, but this association with an hemangioma is unique. The lesion was polypoid and histopathologic examination showed numerous striated muscle fibers located within the dermis.