Apocrine Carcinoma of the Groin Possibly Associated with Extramammary Paget's Disease

Apocrine carcinoma is a rare malignancy with invasive potential. It presents as painless, slow-growing, firm or cystic, red nodules with focal ulcerations. The tumor is capable of hematogenous dissemination to the liver, lungs, and bone as well as lymphatic spread. In addition, apocrine carcinomas cause intra-epidemial pagetoid spread. We report a case of an apocrine carcinoma related with extensive extramammary Paget''s disease (EMPD). The relationship between apocrine carcinoma and EMPD remains to be understood. Co-existing cases with apocrine carcinoma and EMPD are discussed to better understand the relationship between these two malignant apocrine tumors.     

Photodynamic Therapy in Bowen Disease of the First Web Space of the Hand

Bowen disease (BD), or intraepithelial squamous cell carcinoma (SCC), may progress to an invasive SCC. Although surgery is preferred because of the low recurrence rate, it can result in hypertrophic scarringor contracture, particularly in lesions on the hands. We report a case of BD in the first web space of the hand, which was treated with ablative fractional laser-assisted photodynamic therapy (AFXL-assisted PDT). After multiple AFXL-assisted PDT sessions, the lesion showed no clinical or pathological abnormalities. Thus, we believe that PDT can be an alternative treatment for BD occurring in the web space of the hand.     

Extramammary Paget's Disease: A Report of 22 Cases in Chinese Males

Extramammary Paget's disease (EMPD) is more frequently seen in Caucasian females than in males (3.2:1 female: male ratio). During the past 14 year period, we have collected 22 patients, all Chinese males, with EMPD. They presented with eczema-like lesions in the early stages in the genital or perianal regions. Histological sections showed Paget cells within the epidermis or skin appendages and even within the dermis. No underlying adnexal carcinoma or adjacent internal carcinoma could be detected after thorough examinations. Mode of therapy and outcome are presented. EMPD seems to affect more males than females in Orientals. The incidence of concomitant malignancy in Chinese male patients with genital Paget's disease seems to be much lower than that in Caucasians. However, if EMPD involves the glans penis or perianal area, a search for internal malignancy is still warranted.     

Topical imiquimod in the treatment of extramammary Paget's disease: A 10 year retrospective analysis in an Asian tertiary centre

Extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma usually found in apocrine‐rich areas. Although surgery remains standard treatment, topical imiquimod has emerged as a promising drug for the treatment of EMPD in recent years. We present our experience in treating EMPD in Asian skin successfully with topical imiquimod 5% cream, over the past 10 years in our tertiary institution.     

Extramammary Paget's disease treated with topical imiquimod 5% cream

Extramammary Paget's disease (EMPD) is a rare cutaneous, intraepithelial adenocarcinoma usually found in the apocrine gland bearing areas. It is traditionally treated with surgery but has a high rate of recurrence. Of late, topical imiquimod 5% cream has come into use as another treatment option. We present two cases of EMPD in Asian skin treated successfully with topical imiquimod 5% cream.     

Polarized dermoscopy of mammary Paget disease

Mammary Paget''s disease is a rare intraepithelial adenocarcinoma, located on the nipple/areola complex, highly associated with breast cancer. Although the international literature emphasizes the dermatoscopic pattern of mammary Paget''s disease pigmented variant, the authors describe the dermoscopic findings of classical Paget''s disease and demonstrate the presence of chrysalis-like structures, criteria recently described in the literature and not yet reported in Paget''s disease.     

乳房外Paget病皮损中黏蛋白1和黏蛋白2的表达

目的 探讨黏蛋白1(MUC1)和黏蛋白2(MUC2)在乳房外Paget病(EMPD)中的表达情况.方法 生物素蛋白免疫组化法(SP法)检测19例EMPD皮损及19例美容切除术后正常皮肤组织上MUC1与MUC2的表达.结果 19例EMPD皮损常规HE染色显示,3例伴低分化腺癌,6例呈浸润性,10例为上皮内.MUC1在3例伴腺癌Paget病中有2例呈阳性表达,6例浸润性和10例上皮内Paget病均呈阳性表达.MUC2在3例伴腺癌Paget病和6例浸润性Paget病均呈阳性表达,在10例上皮内Paget病中有2例呈阳性表达.MUC1与MUC2在正常皮肤组织呈阴性表达.MUC1在上皮内Paget病中的表达显著高于伴腺癌Paget病和浸润性Paget病(P＜0.05).MUC2在伴腺癌Paget病和浸润性Paget病中的表达显著高于上皮内Paget病(P＜0.05).MUC1和MUC2的表达无明显相关性(r=-0.5,P＞0.05).结论 MUC1在EMPD中呈普遍表达,MUC2在伴有腺癌和浸润性EMPD中呈阳性表达.

Abstract：

Objective To study the expressions of mucin (MUC) 1 and 2 in extramammary Paget's disease(EMPD) lesions. Methods Tissue specimens were obtained from the lesions of 19 patients with EMPD and normal skin of 19 human controls during cosmetic surgery. Streptavidin-perosidase (SP) technique was used to detect the expressions of MUC1 and MUC2 in these specimens. Results As haematoxylin-eosin (HE) staining showed, 3 cases were accompanied by poorly differentiated adenocarcinoma, 6 were invasive Paget's disease and 10 were intraepithelial EMPD. MUC1 was expressed in 2 cases accompanied by poorly differentiated adenocarcinoma, and in all the cases of invasive and intraepithelial EMPD; MUC2 was observed in all the cases of adenocarcinoma-complicated EMPD and invasive EMPD, but only in 2 of 10 cases of intraepithelial EMPD.Neither MUC1 nor MUC2 was observed in normal control specimens. A significant increase was observed in the expression of MUC1 in lesions of intraepithelial EMPD compared with invasive EMPD and adenocarcinoma-complicated EMPD (both P ＜ 0.05), and in the expression of MUC2 in lesions of invasive EMPD and adenocarcinoma-complicated EMPD compared with intraepithelial EMPD (both P ＜ 0.05). The expression of MUC1 was uncorrelated to that of MUC2 (r= -0.5, P＞ 0.05). Conclusions MUC1 is generally expressed in the lesions of EMPD, while MUC2 is expressed in those of adenocarcinoma-complicated EMPD and invasive EMPD.     

Cystic fibrosis transmembrane conductance regulator is helpful in the distinction of extra-mammary Paget's disease from squamous cell carcinoma in situ (Bowen's disease)

Cystic fibrosis transmembrane conductance regulator (CFTR) represents a cAMP-dependent channel found in normal apocrine glands. The classification and histogenesis of extra-mammary Paget's disease (EMPD) remains controversial, but it is generally accepted that primary EMPD exhibits apocrine differentiation. Therefore, we examined the utility of CFTR in the differential diagnosis of EMPD and squamous cell carcinoma in situ (SCCIS). Twenty-five cases of SCCIS and 14 cases of EMPD were evaluated for immunohistochemical expression of CFTR. Expression was scored as 0 (<5% of cells positive), 1+ (5-75% of cells positive) or 2+ (>75% cells positive). Twenty-three of 25 cases of SCCIS showed no reactivity for CFTR, and the remaining 2 cases showed 1+ staining. Thirteen of 14 cases of EMPD showed 2+ staining, while 1 case showed 1+ staining. We recognize that the pathological appearance along with clinical history and site of occurrence are sufficient to distinguish EMPD and SCCIS in most instances. However, distinction between the two can become more challenging when the location and histopathology are not characteristic. We conclude that when an immunohistochemical panel is diagnostically necessary, the expression of CFTR favors a diagnosis of EMPD over SCCIS.     

Photodynamic Therapy for Bowen's Disease of the Vulva Area

Bowen''s disease is a squamous cell carcinoma in situ and has the potential to progress to a squamous cell carcinoma. The authors treated two female patients (a 39-year-old and a 41-year-old) with Bowen''s disease in the vulva area using topical photodynamic therapy (PDT), involving the use of 5-aminolaevulinic acid and a light-emitting diode device. The light was administered at an intensity of 80 mW/cm² for a dose of 120 J/cm² biweekly for 6 cycles. The 39-year-old patient showed excellent clinical improvement, but the other patient achieved only a partial response. Even though one patient underwent a total excision 1 year later due to recurrence, both patients were satisfied with the cosmetic outcomes of this therapy and the partial improvement over time. The common side effect of PDT was a stinging sensation. PDT provides a relatively effective and useful alternative treatment for Bowen''s disease in the vulva area.     

A Case of Vitiligo after Kawasaki's Disease

Vitiligo is a common skin disease, but its pathogenesis has not been fully determined, though an autoimmune etiology is considered likely. Kawasaki disease (KD) is an acute multisystem vasculitis of childhood associated with coronary arteriopathy, and is diagnosed based on clinical criteria. Furthermore, vitiligo has been associated with several other diseases, but no report has been issued about the relationship between vitiligo and Kawasaki''s disease. The author''s report the case of an 8-year-old male child that presented with depigmented lesions, which developed from the desquamative skin lesions of Kawasaki''s disease.     

Mammary and extramammary Paget's disease

Paget''s disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen''s disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget''s disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.     

Penoscrotal Paget's disease

Paget's disease (PD) denotes an initially intra-epidermal adenocarcinoma that can later invade the dermis and metastasise. Among the extramammary forms of PD (EMPD), penoscrotal presentations are rarer than the vulvar and perianal forms. Once diagnosis has been confirmed by histopathological examination, a search for associated neoplasia must be conducted, although penoscrotal EMPD is less frequently associated with underlying neoplasia than mammary PD (MPD). The associated cancer most often involves a neighbouring organ, with prostate cancer being the most common, or in some cases consists of underlying cutaneous adnexal tumours. First-line therapy consists of surgical excision. Alternatives to surgery (imiquimod, CO₂ laser vaporisation, dynamic phototherapy) may be considered in certain cases.     

Extramammary Paget's disease with bowenoid histologic features accompanied by an ectopic lesion on the upper abdomen

We present a 79-year-old man who suffered from extramammary Paget's disease (EMPD) with bowenoid histological features accompanied by an ectopic EMPD lesion on his abdomen. He had had an erythematous plaque on his genital region for three years. Based on a biopsy specimen, he was referred to our hospital with the histological diagnosis of Bowen's disease. The histological findings of the genital lesion obtained by surgical resection showed typical areas of Paget's cells adjacent to areas characteristic of Bowen's disease. Immunohistochemical findings showed CEA and CK7 positive tumor cells in both areas, so the atypical cells showing the bowenoid pattern could be regarded as tumor cells of Paget's disease. Immunohistochemical staining for CEA and CK7 along with multiple biopsies can be helpful in making the diagnosis of Paget's disease with bowenoid histologic features.     

Mapping biopsy with punch biopsies to determine surgical margin in extramammary Paget's disease

It is difficult to determine the appropriate resection margin of extramammary Paget's disease (EMPD). A high recurrence rate is reported in spite of using Mohs micrographic surgery (MMS), which is performed commonly. Preoperative mapping biopsy is easier to perform than MMS. In Japan, the following method is recommended instead of MMS: well‐defined border and margins histologically confirmed by mapping biopsy should be resected with 1‐cm margin and ill‐defined border with 3‐cm margin. This study aimed to evaluate the accuracy of the Japanese guideline and to assess our mapping biopsy method compared with MMS. Preoperative mapping biopsy specimens were obtained beyond the clinical border for at least four directions in each patient. To confirm the presence of residual Paget's cells postoperatively, narrow specimens were obtained along the surgical margin. Retrospective evaluation of 17 EMPD patients was conducted concerning histological spread of Paget's cells and recurrence ratio. There were 86 directions showing a well‐defined border, and in 9.3% (8/86), Paget's cells were still observed at 1‐cm resection line. On the other hand, there were 21 directions showing an ill‐defined border, and unnecessary radical resection was performed in 90% (19/21) of directions with 3‐cm resection line. Although postoperative histological examination showed residual Paget's cells in 47% (8/17) of patients and additional resections were not performed, recurrence rate was only 5.9% (1/17). The resection line of EMPD should be based not on clinical features, but on mapping biopsy. Mapping biopsy is equivalent to MMS concerning recurrence rate and, though conventional, is useful method to treat EMPD.     

A Case of a Surviving Male Infant with Incontinentia Pigmenti

Incontinentia pigmenti (Bloch-Sulzberger''s disease) is an X-linked dominantly inherited disorder which is usually lethal in hemizygous males, but rarely found in male infants. It can be explained by the presence of an extra X chromosome (Klinefelter''s syndrome), hypomorphic mutations, and somatic mosaicism. We herein report a rare case of incontinentia pigmenti with typical course of skin manifestation in normal karyotype (46, XY) male infant.     

An Unusual Clinical Presentation of Kimura's Disease Occurring on the Buttock of a Five-year-old Boy

Kimura''s disease is a rare inflammatory disorder of unknown etiology primarily seen in young Asian males. It is characterized by painless subcutaneous masses, blood and tissue eosinophilia, and markedly elevated serum immunoglobulin E (IgE) levels. Clinically, the subcutaneous nodules occur predominantly in the head and neck. However, we report the case of a five-year-old boy presenting with an asymptomatic solitary brown pigmented nodule on the left buttock diagnosed with Kimura''s disease. Here we describe the diagnosis and treatment of this unusual case.     

Bowen’s disease of the penile shaft presenting as a pigmented macule: dermoscopy,reflectance confocal microscopy and histopathological correlation

The penile localization of pigmented Bowen’s disease has been rarely reported and has been mostly related to human papillomavirus infection. Early diagnosis and treatment are important to prevent progression to invasive squamous cell carcinoma. However, diagnosis can be challenging because it may be difficult to distinguish from melanoma, even using dermoscopy. Reflectance confocal microscopy may be useful in suggesting the bedside diagnosis before the histopathological confirmation. A case of penile pigmented Bowen’s disease is described along with its dermoscopy and reflectance confocal microscopy findings and their correlation with histopathology.     

Extramammary Paget disease successfully treated with topical imiquimod 5% and tazarotene

Extramammary Paget disease (EMPD) is an intraepithelial adenocarcinoma usually localized in areas rich in apocrine sweat glands. Surgery remains the treatment of choice for EMPD. However, several nonsurgical treatments have been also described. Around 40 cases of EMPD treated with imiquimod 5% have been published; of these, only six correspond to nonresponses. We describe a recurrent vulvar EMPD with failure to respond to topical imiquimod 5% in monotherapy but a favorable response to its association with tazarotene.     

Orofacial Granulomatosis Associated with Crohn's Disease

Orofacial granulomatosis (OFG) is a term used to describe swelling of the orofacial area, mainly in the lips, secondary to an underlying granulomatous inflammatory process. OFG has been reported in association with systemic conditions such as sarcoidosis and Crohn''s disease (CD). OFG may precede gastrointestinal disease, such as CD, by several years and may be the only obvious focus of the disease. Herein, we report a patient with OFG and non-symptomatic ulcerations of the ileocecal valve. The patient received intralesional triamcinolone injections every 2 weeks. After 6 weeks, all oral lesions showed marked improvement. The favourable treatment response of this patient suggests that intralesional triamcinolone can be used as a treatment option for patients with CD that have oral lesions. In addition, patients presenting with OFG should be carefully evaluated for gastrointestinal signs and symptoms.