Hidradenoma papilliferum associated with pregnancy: a case report

We present the case of a 33‐year‐old female who developed a cystic nodule on the vulva during pregnancy. Immediately following Cesarean section, the lesion was biopsied and histologic examination revealed a dermal tumor composed of glandular structures arranged in a labyrinth pattern. The glandular structures displayed cytoplasmic vacuolization, large atypical nuclei, prominent nucleoli and scattered eosinophilic luminal secretions. Immunohistochemistry showed the tumor cells to be diffusely positive for CK7 and progesterone receptor with focal expression of mammaglobin and GCDFP‐15. The tumor cells were negative for estrogen receptor and CK20. These histologic and immunophenotypic findings were consistent with hidradenoma papilliferum. Our unusual (and to our knowledge first reported) case demonstrates hidradenoma papilliferum in association with pregnancy and raises the possibility of cytologic atypia and lactational change being secondary to hormonal changes in pregnancy.     

Hidradenoma papilliferum with mixed histopathologic features of syringocystadenoma papilliferum and anogenital mammary-like glands

A case of hidradenoma papilliferum with mixed features of syringocystadenoma papilliferum (SCAP) and anogenital mammary-like glands is reported. A single, fresh red-colored nodule developed in the sulcus between the labia majora and minora of a 49-year-old Japanese woman. Histopathologically, the tumor showed epithelial lining with apocrine secretion and slight connective tissues characteristics. Our case was unique because, like SCAP, the tumor was connected to the epidermis and cystic invaginations extended downward into the deep dermis. In addition, beneath the tumor, tubular structures that resembled normal mammary tissue were present in the subcutaneous fatty tissue. In this study, it has been suggested that this tumor might have been developed from these mammary-like glands.     

Hidradenoma papilliferum with mixed histopathologic features of syringocystadenoma papilliferum and anogenital mammary-like glands: report of a case and review of the literature

Hidradenoma papilliferum of the anogenital region was previously believed to originate from apocrine glands but has recently been accepted as originating from anogenital mammary-like glands. We describe a case of hidradenoma papilliferum with mixed features of syringocystadenoma papilliferum and mammary-like glands from the left labia majora of a 25-year-old woman. Histopathologically, the lesion showed an epithelial lining with apocrine secretion, and like syringocystadenoma papilliferum, the lesion extended from the epithelium as invaginations into the dermis. Adjacent to this lesion were ductal and glandular structures resembling normal mammary tissue. This review of the literature highlights the heterogeneity and complexity of lesions arising from anogenital mammary-like glands, and this case serves as further documentation of the association between anogenital mammary-like glands and hidradenoma papilliferum.     

Hidradenoma papilliferum with a ductal carcinoma in situ component: case report and review of the literature

Hidradenoma papilliferum (HP) is a benign cutaneous adnexal neoplasm occurring mainly in the anogenital region of adult women and has features analogous to intraductal papilloma of the breast. Malignant change in HP is extremely rare. Only a single case of ductal carcinoma in situ arising in HP has been previously reported. We present a new case of HP which, in addition to the typical appearance of HP, contained a focus of ductal carcinoma in situ that appeared as enlarged pleomorphic epithelial cells having a "blastic" appearance, exhibiting atypical mitotic figures and surrounded by myoepithelial cells. Molecular biological study identified human papillomavirus (HPV)-16, which, it may be argued, may have played a role in the development of the carcinoma.     

Pigmented viral warts: a clinical and histopathological study including human papillomavirus typing

Although clinical, histological and viral correlations have recently been established among pigmented warts, homogeneous intracytoplasmic inclusion bodies and related types of human papillomavirus (HPV) (HPV 65, 4 and 60), the causes of the pigmentation remain unknown. In this study, comparative histological and histochemical analyses were performed with 53 pigmented (34 HPV 65-induced, 12 HPV 4-induced and seven HPV 60-induced) and 73 non-pigmented warts (27 HPV 2-induced, 23 HPV 1-induced, 12 HPV 63-induced, six unknown HPV-type induced and five HPV 60 induced) to clarify the causes of the pigmentation. Electron microscopy was also used to examine the pigmented warts. Many melanin blockade melanocytes were identified in all of the pigmented warts with Masson–Fontana staining and electron microscopy, and increased melanin in keratinocytes was also noted in 22 pigmented warts, suggesting that the dispersion of melanin granules in the dendrites of the melanin blockade melanocytes and the increased melanin granules in keratinocytes are the primary contributors to the pigmentation of the warts. The homogeneous intracytoplasmic inclusion bodies might also play a part in the darkening of the warts, as only the cases which had the inclusion bodies as well as the melanin blockade melanocytes were clinically pigmented. Although melanin blockade melanocytes were seen in a few cases of HPV 1- and HPV 2-induced warts in which the homogeneous inclusion bodies were not observed, the warts were not clinically pigmented. Melanin blockade melanocytes were not seen in any of the HPV 63-induced non-pigmented warts. In conclusion, the pigmented warts were associated with one of the related types of HPV (HPV 65, 4 and 60), and the pigmentation of the lesions is thus thought to be caused primarily by melanin blockade melanocytes. The homogeneous intracytoplasmic inclusion bodies might also play a part in the darkening of the lesions. This is the first report dealing with the pigmentary disorder associated with specific types of HPV.     

Hidradenoma papilliferum of the vulva in association with an anogenital mammary‐like gland

Hidradenoma papilliferum (HP) is a benign adnexal neoplasm which preferentially develops in the anogenital region of women. Although the origin of HP was previously thought to be an apocrine sweat gland, recent studies have suggested that it may derive from the anogenital mammary‐like gland (MLG). In this paper, we present a 43‐year‐old Japanese woman with hidradenoma papilliferum of the vulva. The lesion developed 7 years prior to her visit, and clinically appeared as a skin‐colored cystic nodule. Histopathological examination revealed that the neoplasm was formed by the tubular structures consisting of two types of pleomorphic cells, columnar cells in the luminal layer and cuboidal cells in the basal layer. Further, the surgical specimen contained a wide, divergent, lobular ductal structure located in the vicinity of the neoplastic lesion, which was consistent with MLG.     

发育不良性痣11例临床病理学分析

目的 探讨发育不良性痣的临床病理学特点。方法 对11例临床表现为色素性损害的手术标本行H-E染色，结合临床指标及评分进行研究和分析。结果 临床表现：皮损直径≥5mm者8例，多发性损害者7例，边界模糊者4例，外形不规则者6例，痣表面色素不匀者4例，基底色红者6例。光镜下：交界痣3例，复合痣8例。发育不良性痣较为特异的组织学表现为真表皮交界处雀斑样增生，痣细胞巢增生紊乱倾向于形成“桥型”融合。表皮下方的非典型黑素细胞在基底层呈“Paget”样蔓延，真表皮交界处的黑素细胞向周围延伸，并超过真皮内的痣细胞成分。非典型黑素细胞：细胞核较角质形成细胞核大，多形性，出现核仁，深染。结论 临床和组织病理相结合是诊断发育不良性痣的可行性标准，仅根据组织学的非典型性不能诊断发育不良性痣。     

Hybrid cysts: a clinicopathological study of seven cases

Hybrid cysts develop from more than two components of the pilosebaceous unit. The pathogenesis of this unusual disease has not yet been elucidated. The aim of this study was to assess the clinical and histopathological features of hybrid cysts. Histological sections of seven cases indexed as hybrid cysts were reviewed from 1996-2009 at the Department of Dermatology, Eulji Medical Center, Seoul, Korea. Hospital charts and slides were retrospectively evaluated. All cases had a combination of an epidermal cyst and a pilomatricoma with sharp transitional zones. The epidermal cyst lining was composed of thickened stratified squamous epithelium with a granular layer. Basophilic cells, shadow cells and the contents of the pilomatricoma were present in all lesions. There were no differences in the clinical features between patients with hybrid cysts and others with single cystic lesions. There were five women and two men, ranging in age from 11 to 50 years (mean 27 years). The most common sites were the upper extremities. None of the patients had any sign of Gardner's syndrome. Hybrid cysts are an interesting pathological phenomenon. Further study is needed to analyze hybrid cysts to improve our understanding of their pathogenesis and development from tumours of the pilosebaceous unit.     

Dermatofibrosarcoma protuberans: a clinicopathological study of 20 cases

AIM: To review the dinical and histological data of 20 cases of dermatofibrosarcoma protuberans presenting at two dermatology centres in Lisbon from 1978 to 1998. PATIENTS AND METHODS: The 20 subjects comprised nine males and 11 females ranging in age from 25 to 79 years, with highest frequency of subjects in the 30-50 year olds. We reviewed the clinical features, histopathological aspects, including morphologic variants and immunohistochemical studies. RESULTS: Median age at diagnosis was 51 years and the trunk was the most frequent location. The characteristic histologic storiform pattern was seen in all cases. Three subjects presented fibrosarcomatous areas, one with myoid differentiation and another with multinucleated giant cells. Immunohistochemical stains revealed CD34 expression in the 18 specimens tested, FXIIIa was negative, and these two antigens proved important for the differential diagnosis of this neoplasm. Local wide excision was performed in 13 cases and seven patients underwent Moh's micrographic surgery. Follow-up ranged from 2 months to 17 years and three recurrences were recorded, two following classical surgery and one after Moh's surgery; there was no difference in the rate of local recurrence (15%) for the two kinds of treatment in our series.     

Papular xanthoma: a clinicopathological study of 10 cases

BACKGROUND: Papular xanthoma (PX) is one of several clinicopathologic variants of normolipemic cutaneous non-Langerhans cell histiocytoses (n-LCH). PX represents a monomorphous reaction pattern of n-LCH characterized by the presence of predominantly xanthomatized macrophages. OBJECTIVE: The purpose of this study was to identify the clinical, histological and immunohistochemical characteristics of PX. METHODS: A series of 10 cases of PX was identified and the results compared with the other histologic subtypes, namely the polymorphous and the remaining other monomorphous reaction patterns in n-LCH. RESULTS: In this clinicopathologic study, papular xanthoma presented clinically mainly as solitary papule, with a male to female ratio of4 : 1, in an age range from 13 to 57 years and a biphasic occurrence: in the young adolescence and middle ages. It was predominantly located on the trunk, the extremities, and rarely on the head. Clinically, PX was described as xanthoma, 'cutaneous tumor', but also as atheroma, keloid, histiocytoma, Spitz's nevus or clear cell acanthoma. Histology showed moderately well circumscribed exoendophytic papules with a regular epidermis and a dense infiltration of xanthomatized macrophages interspersed by numerous Touton type giant cells. Immunohistochemically mono- and multinucleated macrophages were consistently positive with KiM1p; while only giant cells were labeled with KP1 (CD68), the reactivity with HAM 56 was much more variable. Up to 50% of the xanthomatized cells labeled positive for the lectin peanut agglutinin. In one case the xanthomatized cells stained positive for CD34. Staining for factor XIIIa and CD1a were negative. CONCLUSIONS: This series confirms PX as a rare, but distinguished clinicopathologic entity in the spectrum of n-LCH of the skin.     

Atypical fibroxanthoma: a clinicopathological study of 89 cases

Atypical fibroxanthoma is a controversial entity with a disputed histogenesis. It has recently been suggested that most atypical fibroxanthoma are actually variants of squamous cell carcinoma. We reviewed 100 purported cases of atypical fibroxanthoma received over 4 years to perform clinical follow up and immunohistochemical markers. In particular, we focused on the detection of any recurrence or metastasis. Ten cases were subsequently excluded on the basis of either incorrect coding, or insufficient or absent paraffin blocks on file. A further case was interpreted as a malignant fibrous histiocytoma. Additional new markers, such as CD10 and CD99, were employed in a proportion of cases. Our cases were typical of the usual clinical presentation of atypical fibroxanthoma on the skin of the sun-damaged elderly. We found no cases of recurrent or metastatic atypical fibroxanthoma. Two patients developed a second primary atypical fibroxanthoma. CD10 proved to be a useful marker for atypical fibroxanthoma when used on 20 cases in the present study, as was CD99 in seven cases. The only case demonstrating positive staining for keratin also stained for CD10. It had dual features of atypical fibroxanthoma and squamous cell carcinoma. However, as the majority of atypical fibroxanthoma had no adjacent solar keratosis, our data suggest it is unlikely that atypical fibroxanthoma is a variant of squamous cell carcinoma.     

Vulvar vestibulitis syndrome: a clinicopathological study of 14 cases

BACKGROUND: Vulvar vestibulitis syndrome (VVS) is one of the most frequent causes of superficial dyspareunia in young women. VVS has a pronounced psychological impact. The results of pathological studies published thus far are controversial. PATIENTS AND METHODS: Fourteen women with VVS were included in this study and underwent vestibular biopsy. Vulvar biopsies were taken from the orifice of Bartholin's gland. The biopsy samples were stained with a standard stain and PAS and 25 serial sections were prepared for each specimen. RESULTS: The mean patient age was 26 years and VVS had been present for a mean 30 months. Extensive inflammation of mononuclear cells was observed in the vulvar chorionic epithelium. This inflammation was seen mainly around the minor vestibular glands. Mild exocytosis of lymphocytes was noted in the vestibular glands and ducts. DISCUSSION: Most studies concerning this disease report chronic inflammation of the vulvar vestibular mucosa. This inflammation is seen mainly around the minor vestibular glands. We report the same pattern in our study. Moreover, we observed some exocytosis into the epithelium of minor vestibular glands and the excretory duct. This aspect has not been reported to date, further supporting the individual nature of this entity.     

Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases

Cutaneous periarteritis nodosa (PAN) is a well-recognized entity characterized by tender subcutaneous nodules and livedo that may ulcerate. The pathogenesis of cutaneous PAN is not known. The objective of the study was to evaluate the clinical and histological features of 79 cases of cutaneous PAN and to investigate any clinical, pathological and immunological differences that may distinguish those cases likely to have a prolonged course. A retrospective analysis of 79 cases was conducted. Thirty-nine patients had ulcers during the course of their illness. Women were affected more than men. Painful nodules on the lower extremities, with oedema and swelling, were the most common clinical finding: 22% of patients had some evidence of neuropathy. Most of the laboratory findings were non-specific. There was no evidence for hepatitis B infection and hepatitis C infection was present in only one patient. Most patients (60%) had no associated medical condition. The disease course was prolonged but benign, and systemic PAN did not develop in any patient. Corticosteroids given systemically induced remission in most acute cases. The ulcerative form of disease was more prolonged and frequently associated with neuropathy.     

Desmoplastic malignant melanoma: a clinicopathological study of 25 cases

Sixteen cases of malignant melanoma which showed prominent desmoplastic and/or neurotropic features occurring throughout the tumour were compiled from the St John's Dermatology Centre histopathological archives. A further nine melanomas in which both conventional and desmoplastic melanoma were present concomitantly were also studied (three tumours with 66% desmoplastic change, two with 50%, and four with less than 50%). There were 14 males and 11 females, with a mean age of 64 years (range 39–86). The mean interval between presentation and diagnosis was 8 months. Eighteen of the 25 tumours were located on the head and neck, three were on the trunk, one was on the upper limb and three were on the lower limb. Histological review revealed 21 of 25 tumours with overlying atypical lentiginous hyperplasia, lentigo maligna melanoma, or superficial spreading malignant melanoma. Neurotropism was present in nine tumours, with the changes confined to local recurrences in two instances; neuroid differentiation was present in four tumours, and neural and perineural tumour spread was present in four tumours. The depth of invasion exceeded 6 mm in seven tumours, and was 2–6 mm in 16, and less than 2 mm in two. Eighteen of the 25 tumours were incompletely excised at the time of the first excision. Lymphoid aggregates were present in 16 tumours, but in most cases were limited to a few lymphoid foci. Melanin was identified in the dermal component of only five tumours, but not in areas showing typical histological features of desmoplastic malignant melanoma. Treatment was by surgical excision in all cases, and was preceded by radiotherapy in one case. Details of follow-up were obtained in all cases, and the duration ranged from 9 months to 10 years (mean, 3 years 11 months). Eleven patients had died: nine from melanoma and two from other causes. One patient was alive, with deep, inoperable local recurrence. Thirteen patients were alive and clinically free from tumour, including two patients in whom there had been local recurrence. A lower rate of neurotropism was present in the nine patients with partial desmoplastic change compared with those with desmoplastic change throughout the tumour, and represented the only significant difference between the two groups of patients.     

Merkel cell carcinoma: a clinicopathological study of 11 cases

OBJECTIVE: To report our 12-year experience with Merkel cell carcinomas (MCCs) from a clinical and pathological point of view. SUBJECTS AND SETTING: Eleven MCCs were diagnosed at our institution between 1991 and 2002. METHODS: A retrospective clinical, histopathological and immunohistochemical study was performed. Age, gender, location, size, stage, treatment and follow-up data were collected. Histopathological pattern and immunohistochemical study with CAM 5.2, cytokeratin 20 (CK20), CK7, Ber EP4, neurofilaments, synaptophysin, chromogranin, S100 protein, p53 protein, CD117, leucocyte common antigen (LCA) and Ki-67 were accomplished. RESULTS: Six females and five males with a mean age of 82 years were identified. Tumours were located on the face (n = 6), extremities (n = 3) and trunk (n = 1). At diagnosis, one patient was in stage Ia, six in stage Ib, three in stage II and one in stage III. All but one patient experienced wide surgical excision of the tumour. Additional treatment consisted of lymph node dissection in two patients, radiotherapy in four patients and systemic chemotherapy in one patient. Local recurrence developed in five patients. Three patients died because of MCC after 14 months of follow-up. Intermediate-size round cell proliferation was found in all cases. Additional small-size cell pattern and trabecular pattern were observed in seven and six cases, respectively. Eccrine and squamous cell differentiation were found in three cases. A dot-like paranuclear pattern was observed in all cases with CAM 5.2 and neurofilaments, and in 89% of cases with CK20. Seventy-five per cent of cases reacted with Ber EP4, chromogranin and synaptophysin, 70% with p53, 22% with S100 protein, 55% with CD117 and none with LCA. Ki-67 was found in 75% of tumoral cells on average. Fifty per cent of MCCs reacted with CK7 and showed eccrine differentiation areas. CONCLUSIONS: MCC is an aggressive neuroendocrine tumour of the elderly. Wide surgical excision is the recommended treatment. Lymph node dissection, adjuvant radiotherapy and chemotherapy decrease regional recurrences but have not been demonstrated to increase survival. Immunohistochemically, MCC is an epithelial tumour with neuroendocrine features.     

Hypertrophic lupus erythematosus: a clinicopathological study of 14 cases

BACKGROUND: Hypertrophic lupus erythematosus (HLE) is a distinct and rare subset of lupus erythematosus (LE). It is characterized by verrucous lesions, chronic in its course, and resistant to treatment. The purpose of this study was to identify clinical and histological characteristics of HLE. METHODS: We review our experience with 14 cases of HLE identified in a group of 220 patients with different forms of LE, at the UNICAMP Hospital, between 1976 and 2002. RESULTS: All patients presented verrucous plaques concomitantly with discoid lesions. The most common sites of involvement were the face and the arms. Histology of HLE lesions revealed pseudoepitheliomatous hyperplasia engulfing elastotic material. Elastic fibers were seen in migration throughout the epidermis. Classic features of LE were noted in all cases. Three of the patients developed hyperkeratotic papules with central keratinous plug on their arms at the previous LE sites. These lesions resemble clinically and histopathologically keratoacanthomas. In one patient, HLE lesion progressed to squamous cell carcinoma (SCC), 26 years after the onset of the disease. CONCLUSIONS: Transepithelial elimination of the elastotic material may be a feature of HLE. Some HLE lesions may present as keratoacanthoma, but classical features of LE aid the correct diagnosis. SCC may arise on a long-standing HLE lesion; therefore HLE requires clinical and histopathological follow up.     

Follicular cutaneous T-cell lymphoma: a clinicopathological study of nine cases

Nine patients with follicular cutaneous T-cell lymphoma (CTCL), a recently described variant of lymphoma, are presented. On the basis of clinical manifestations and disease course, three groups of patients were distinguished: (i) two patients with follicular CTCL not associated with conventional lesions of mycosis fungoides (MF) and showing no evolution towards MF in follow-up periods of 3 and 6 years; (ii) one patient with follicular CTCL that evolved into conventional MF within 3 years; (iii) six patients showing conventional MF lesions either before or concurrently with the follicular lesions and thus representing follicular CTCL of the true MF type. The follicular lesions included hair-devoid patches or plaques with spiky hyperkeratotic papules (four patients), keratosis pilaris-like lesions (four), comedo-like lesions (four), follicular papules with alopecia (three) and milia-like lesions (three). Histopathological examination showed perifollicular and intrafollicular lymphocytes, without mucin deposition and with minimal or no involvement of the overlying epidermis. Significant syringotropism was also observed in three cases. Immunohistochemical analysis showed the predominance of CD4 + T cells, deletion of CD7 in some cases, Ki-67 + lymphocytes confined mainly to the follicular epithelium, and expression of keratinocyte intercellular adhesion molecule-1 exclusively in the hair follicle. T-cell receptor gamma gene rearrangement was positive in the one case studied from each group. Different treatment modalities were employed, the most commonly used as monotherapy being phototherapy: psoralen ultraviolet A in four patients, two of whom showed a complete clinical and histopathological remission, and ultraviolet B in one patient, who showed a complete remission (both clinical and histopathological). This study indicates that follicular CTCL is more common than reflected in the literature, has heterogeneous clinical manifestations, and is either an expression of or closely related to MF. The influence of the follicular involvement on the therapeutic response remains to be clarified. However, our therapeutic experience clearly suggests that some patients with follicular CTCL can benefit from phototherapy.