Erosive Lichen Sclerosus-A Clinicopathologic Subtype.

OBJECTIVE: The aim of the study was to identify whether erosive lichen sclerosus (LS) is a distinct clinicopathologic subtype. MATERIALS AND METHODS: The pathology database was searched for "erosion," "erosive," "ulcer," and "lichen sclerosus." Inclusion criteria were histopathologic diagnosis of LS and erosion or ulcer overlying a band of hyalinization and/or fibrosis. Exclusions were concurrent neoplasia and insufficient tissue. Histopathologic review documented site, epithelial thickness, adjacent epidermal characteristics, infiltrate, and dermal collagen abnormality. Clinical data included demographics, comorbidities, examination findings, microbiologic results, treatment, and response. RESULTS: Ten examples of erosive LS and 15 of ulcerated LS occurred in 24 women with a mean age of 67 years. Ulcerated LS was associated with diabetes and nontreatment at time of biopsy. Clinicians identified red patches in all but 1 case of erosive LS. Ulcerated LS was documented as fissure, ulcer, or white plaque, with 8 (53%) described as lichenified LS with epidermal breaches. Erosive LS favored hairless skin with normal adjacent stratum corneum sloping gently into erosion, whereas most ulcers in LS had an abrupt slope from hair-bearing skin. All cases were treated with topical steroids; 2 patients with erosive LS and 10 with ulcerated LS also had oral antifungals, topical estrogen, antibiotics, and/or lesional excision. Treatment yielded complete resolution in 50%. CONCLUSIONS: Erosive LS is an unusual clinicopathologic subtype characterized by red patches on hairless skin seen microscopically as eroded epithelium overlying a band of hyalinized or fibrotic collagen. In contrast, ulcerated LS is usually a traumatic secondary effect in an uncontrolled dermatosis.

[Pathology and histopathological evaluation of penile cancer]. / Pathologie und histopathologische Begutachtung des Peniskarzinoms.

BACKGROUND: Penile cancer is rare in Germany and in western European countries. Our understanding of the pathogenesis and pathology of this malignancy has increased considerably in recent years. OBJECTIVES: Clinical management has become more complex, with organ-preserving strategies being increasingly favored. Associated with these developments, the demands on the pathology reports of biopsies and surgical specimens from the penis have also increased. MATERIALS AND METHODS: According to guidelines and the relevant literature, this review outlines the most important aspects that must be considered in the classification and pathological reporting of penile cancer. RESULTS: Correct histological subtyping of penile cancer is important for prognostic and therapeutic considerations. There are also some peculiarities with the current TNM classification system of this tumor compared to other entities. CONCLUSION: Handling of specimens and histopathological typing must be performed by experienced pathologists according to recent developments in the pathogenesis, classification, and therapeutic strategies of penile cancer.

Actualización en la clasificación y el tratamiento de la esclerodermia localizada / Update on the Classification and Treatment of Localized Scleroderma

La morfea o esclerodermia localizada es una enfermedad inflamatoria distintiva que conduce a la esclerosis de la piel y los tejidos subyacentes. Incluye una serie de entidades que pueden distinguirse basándose en las manifestaciones clínicas y la estructura de la piel y los tejidos subyacentes involucrados en el proceso fibroso. Sin embargo, la clasificación de estos procesos resulta difícil desde el momento en que los límites entre ellos no siempre son claros y es frecuente el solapamiento. En esencia, se distingue entre la morfea en placas, la esclerodermia lineal, la morfea generalizada y la panesclerótica. Si bien no tiene, salvo excepciones, una repercusión sistémica grave, sí que puede ser causa de una gran morbilidad. Si las lesiones asientan en el polo cefálico, pueden acompañarse de complicaciones neurológicas y oculares. No existe un tratamiento realmente eficaz y universal por lo que es importante realizar una evaluación correcta de la extensión y la gravedad de la enfermedad antes de tomar una decisión terapéutica (AU)

Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. However, classification is difficult because the boundaries between the different types of morphea are blurred and different entities frequently overlap. The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea. With certain exceptions, the disorder does not have serious systemic repercussions, but it can cause considerable morbidity. In the case of lesions affecting the head, neurological and ocular complications may occur. There is no really effective and universal treatment so it is important to make a correct assessment of the extent and severity of the disease before deciding on a treatment approach (AU)

Distinctive association of p16INK4a overexpression with penile intraepithelial neoplasia depicting warty and/or basaloid features: a study of 141 cases evaluating a new nomenclature.

From the pathogenic point of view, penile cancers may be grouped in human papillomavirus-related and unrelated tumors, each one of them with distinctive morphologic features. The former are predominantly composed of small, undifferentiated basaloid cells, with more or less prominent koilocytic changes, and the latter of keratinizing differentiated squamous cells. The same cellular types are observed in precancerous lesions. On the basis of these observations, we constructed a novel nomenclature for penile precancerous lesions and classified them as penile intraepithelial neoplasia (PeIN) of differentiated, warty, basaloid, and warty-basaloid types. The aim of this study was to test the usefulness of immunohistochemical p16 overexpression, considered as a surrogate for high-risk human papillomavirus infection, using this classification system. We pathologically evaluated 141 patients with PeIN, associated (123 cases) and unassociated (18 cases) with invasive cancer. Distribution of PeIN types was: differentiated, 72%; basaloid, 9%; warty-basaloid, 7%; warty, 4%; and mixed, 7%. There was a striking similarity in the morphology of in situ and invasive squamous cell carcinomas. Differentiated PeIN was commonly associated with usual, verrucous, papillary, and other low-grade keratinizing variants of squamous cell carcinoma whereas in basaloid and warty carcinomas the presence of in situ lesions with similar morphology was habitual. We evaluated p16 overexpression using a 4-tiered (0, 1, 2, and 3) pattern-based system. To properly distinguish differentiated PeIN from in situ lesions with warty and/or basaloid features only pattern 3, which requires full-thickness staining in all epithelial cells, was considered positive. Using this approach, there was a significant association of the negative patterns and differentiated PeIN and of the positive pattern and warty, basaloid, and warty-basaloid PeIN (P<0.0001). Basaloid variant had the strongest association. The sensitivity rate of p16 positivity for discriminating types of PeIN was of 82%, with a specificity of 100% and an accuracy of 95%. Lichen sclerosus was identified in 42 cases and their epithelial component was p16 negative in all cases. Although more studies are necessary to confirm these observations, p16 overexpression seems to be a useful tool for discriminating differentiated from warty, basaloid, and warty-basaloid PeIN.

[Patient with generalized guttate morphea and lichen sclerosus et atrophicus]. / Coexistencia de morfea en gotas generalizada y liquen escleroatrófico: a propósito de un caso.

Generalized guttate morphea is a very uncommon clinical entity, and few reports are available in the literature. We report the case of a 7-year-old boy who first attended our clinic in 1990 with guttate morphea on the trunk and upper limbs. These lesions were associated with plaque morphea on his right foot. Twelve years later, lesions with a different appearance to the previous ones were observed in the right pectoral region. Clinically and histopathologically, they resembled lichen sclerosus et atrophicus. Given that morphea and lichen sclerosus et atrophicus share certain clinical and pathologic characteristics, some authors believe that these entities may be related or even different presentations of the same disease. The most noteworthy aspect of our case is the type of morphea, as we were unable to find equivalent examples in the literature.

Vitiligoid lichen sclerosus: a reappraisal.

BACKGROUND: Many case studies of lichen sclerosus (LS) have reported an association of vitiligo. However, such an association is not reported from larger case studies of vitiligo, which happens to be a common disease. Autoimmune etiology suspected in both LS and vitiligo has been considered as the reason for their association in some patients. It has also been suggested that lichenoid inflammation in LS may trigger an autoimmune reaction against melanocytes. AIMS: To test this association, we reviewed clinical and histological features of 266 cases of vitiligo and 74 cases of LS in a concurrent study of both diseases. METHODS: All outpatients seen in our department between 2003 and 2006 and who were diagnosed as having LS or vitiligo on the basis of clinical and pathologic features were included in the study. RESULTS: Vitiligoid lesions were seen along with stereotypical LS lesions in three patients but all the three lesions had histological features of LS. Oral/genital areas were affected in 57 out of the 74 LS cases and of those, 15 were initially suspected to have vitiligo. These cases with a clinical appearance of vitiligo and histological features of LS were considered as 'vitiligoid LS', a superficial variant proposed by J. M. Borda in 1968. Association of LS was not observed in the 266 cases of vitiligo. CONCLUSION: Exclusive oral/genital depigmentation is a common problem and histological evaluation is essential to differentiate vitiligoid LS from true vitiligo. The association of vitiligo with LS may have been documented due to the clinical misdiagnosis of vitiligoid LS lesions as vitiligo as histological investigations were not undertaken in any of the reported cases.

Sistematización del estudio de las formas clínicas de Liquen escleroso y atrófico en adultos / Adult lichen sclerosus et atrophicus clinical forms study systematization

El liquen escleroso puede ser encontrado en cualquier grupo etáreo, sexo o raza. En la literatura se ha descrito varias formas deliquen escleroso. Objetivo: Facilitar el estudio de liquen escleroso, sistematizando sus caracteres clínicos. Materiales y métodos: Ensayar una clasificación sobre formas clínicas de liquen escleroso, de acuerdo a su frecuencia y la consistencia de cómo son definidos en la literatura médica. Resultados: Entre enero a noviembre de 2006, en 1417 pacientes encontramos 8 diferentes formasclínicas. Formas clásicas: en reloj de arena o forma de 8 (1 forma), balanitis xerótica obliterante (1), pequeñas placas en el pene (3) -relacionado a circuncisión previa por fimosis (2) -. Formas no clásicas: generalizada (1 forma), asociado a carcinoma escamoso de vulva (1), concomitante a hemorragia uterina disfuncional (1), superpuesta a morfea y dermatitis infectiva en una paciente infectada con el HTLV-I (1). Conclusiones: Las 8 diferentes formas de liquen escleroso encontradas en este trabajo sugieren la necesidad de continuar buscando una clasificación que permita un adecuado reconocimiento de estas diferentes formas.

Lichen sclerosus can be found at any age, sex or race. Several clinical forms of lichen sclerosus have been described in the literature. Objective: To facilitate lichen sclerosus study organizing its clinical features. Materials and methods: A classification of the clinical forms of lichen sclerosus was assayed. Clinical features were organized according to frequency and consistency as defined in medical references. Results: Between January and November 2006, we found 11 cases of lichen sclerosus in 1417 dermatology patients. Eight different clinic forms were found. Classical forms: æfigure of eightÆ shape (1 form),balanitis xerotica obliterans (1), small plaques in penis (3) related to previous circumcision for phimosis (2). Non-classical forms: generalized (1 form), associated to vulvar squamous carcinoma (1), concomitant to morphoea and dysfunctional uterine bleeding (1) and superimposed to morphoea and infective dermatitis in an HTLV-I infected patient (1). Conclusions: The finding of eight different forms of lichen sclerosus suggests the need to continue looking for an ideal classification to adequately recognize these different clinical forms.

Intralesional injection of triamcinolone in the treatment of lichen sclerosus.

OBJECTIVE: To assess intralesional vulvar injections of triamcinolone as an alternative to using topical treatment. STUDY DESIGN: This was an open trial, in eight patients, of intralesional injection of triamcinolone in patients with symptomatic lichen sclerosus who could not use primary topical treatments. The patients' pretreatment and posttreatment clinical symptoms and gross physical findings were reviewed. In some patients pretreatment and posttreatment biopsies were performed. RESULTS: There was a decrease in severity scores in the categories of symptoms and physical findings. In four patients who consented to posttreatment biopsy, there was a decrease in severity scores on histopathologic findings. CONCLUSION: Intralesional injection of triamcinolone hexacetonide into sites of vulvar lichen sclerosus seems to be an effective alternative to using topical agents.

Lichen sclerosus.

Lichen sclerosus, usually appearing in the dermatologic literature under the names of lichen sclerosus et atrophicus, balanitis xerotica obliterans, and kraurosis vulvae, is an inflammatory disease with a multifactorial origin. A past association of lichen sclerosus and genital squamous cell carcinoma is not as close as once thought. Once considered primarily a surgical problem, especially when the genitals were involved, lichen sclerosus will respond to a variety of systemic and topical therapies.

Treatment of vulvar lichen sclerosus in the elderly: an update.

Lichen sclerosus et atrophicus is a disorder of the skin that can occur anywhere on the body and in all age groups but mainly affects middle-aged and elderly women in the vulvoperineal area. It consists of ivory or pink papules or macules that eventually coalesce into thin, gray, parchment-like areas. Clinically, the main symptoms are severe and intractable itching and vaginal soreness with dyspareunia. Although it has been described to be associated with an increased risk for epithelial malignancy this, in fact, very rarely occurs. The exact nature of LSA is still unknown. The accumulation of evidence does little to clarify its pathogenesis and etiology. The different reports indicate at least three general possibilities; autoimmune, metabolic, and more recently infectious etiology. The coexistence of such diverse findings in one disease entity may indicate one of the two; either we are facing a group of very similar conditions, which will be separated in the future into several closely related clinical entities, each with its own etiology, or that all findings represent a complex multi-step single pathogenetic mechanism. The latter possibility seems more probable because it has previously been suggested that B. burgdorferi, a recent prime suspect in the pathogenesis of LSA, may induce both metabolic and autoimmune abnormalities in the course of infection. New therapeutic options and attitudes emerge that dramatically improved the conservative treatment of this disease (Table 5).(ABSTRACT TRUNCATED AT 250 WORDS)

[Vulvar lichen sclerosus]. / Lichen scléreux vulvaire.

Lichen sclerosus's pathogeny, the most frequent vulvar dystrophy predominant at the start of menopause, is still enigmatic. Its repercussions on the functional level can be disabling. Its clinical sides include atrophy and sclerosis. The evolution of the past towards great atrophies (kraurosis vulvae) may today be prevented by early diagnosis and treatment (essentially dermocorticoïde). Even if the risk of degenerescence is low, it's not negligible and these patients must be put under steady surveillance.