Three unusual histopathological presentations of angiolymphoid hyperplasia with eosinophilia.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a poorly understood benign vasculoproliferative disorder. Histopathologically, the lesions are composed of a dermal or subcutaneous proliferation of blood vessels of varying sizes with plump and bland endothelial cells, which often show vacuolization and protrusion of the cytoplasm into the lumen. The vascular proliferation is admixed with a diffuse inflammatory infiltrate composed of lymphocytes, eosinophils and mast cells. Lymphoid follicles with germinal centers can sometimes be seen. In addition, fibrosis is a common accompanying feature. Some clinical and histopathological variants of ALHE have already been described in the literature. In this report, we present 3 rare associations of ALHE that have not been previously described. Case 1 was a 73-year-old woman with a lesion on her right medial thigh. Examination showed ALHE admixed with a chronic lymphocytic leukemic (CLL) infiltrate. Case 2 was a 55-year-old woman with a lesion on her right anterior ankle, which was a syringocystadenoma papilliferum co-existing with an ALHE. Case 3 was a 54-year-old man with a lesion on the left medial thigh, which showed overlapping features of IgG4-related disease associated with areas of ALHE. Given these multiple and diverse associations, it seems likely that ALHE may be a reactive rather than neoplastic process.

Is Tumor of Follicular Infundibulum a Reaction to Dermal Scarring?

Tumor of follicular infundibulum (TFI) is currently believed to be a benign epithelial neoplasm with follicular differentiation. It has been suggested that TFI is associated with dermal scarring, but further investigation is needed to confirm this correlation. To approach this question, a retrospective study was presented, a total of 67 cases (64 lesions) were found in a search covering cases over a 10-year period. Overall, the presence of histological dermal scarring was noted in 34 of 64 (53.13%) cases. Of the cases where TFI was an incidental finding, the presence of dermal scarring was noted in 13 of 18 (72.22%) cases. Meanwhile, of the cases where TFI was the main diagnosis, the presence of dermal scarring was noted in 12 of 34 (35.29%) cases. This suggests that TFI may, in some cases, represent an epidermal reaction pattern to dermal scarring.

Características clínicas y cambios epidemiológicos de «tinea capitis» en la población pediátrica / Clinical features and epidemiological changes in tinea capitis on a pediatric population

El aumento de la población extranjera ha determinado cambios en los dermatofitos productores de tinea capitis en diversos países. Presentamos una revisión de los pacientes pediátricos atendidos en un hospital de segundo nivel con clínica de tinea capitis y cultivo de escamas cutáneas del cuero cabelludo positivo para dermatofitos, con el fin de conocer la posible variación en la epidemiología, la presentación clínica y la respuesta al tratamiento antifúngico de dicha infección en los últimos años. En el 60% de los pacientes el cultivo fue positivo para Microsporum canis; se manifestó predominantemente como una forma alopécica microspórica en pacientes autóctonos con buena respuesta a la griseofulvina oral. Trichophyton violaceum fue el segundo dermatofito en frecuencia, causante de tinea capitis en 6 pacientes de origen africano; la forma de presentación más habitual fue una descamación fina del cuero cabelludo, con escasa o nula alopecia, y la mejor respuesta terapéutica se obtuvo con la terbinafina sistémica. Realizamos una comparación de las diferentes características epidemiológicas, clínicas y terapéuticas entre ambos hongos dermatofitos (AU)

Increasing numbers of foreigners has led to some changes in tinea capitis etiological agents in several countries. We present a review of pediatric patients suffering from tinea capitis with scalp scales positives cultures for dermatophytes attended in the last years at a second level hospital, in order to know the epidemiological features, clinical and therapeutic response variations. Microsporum canis was isolated in 60% of the patients; it was mostly seen as an alopecic microsporic clinical form in native children with an adequate response to oral griseofulvine. Trichophyton violaceum was the second most frequently isolated dermatophyte, which caused tinea capitis in 6 African children; it often produced a thin shedding scale with null or little associated alopecia and systemic terbinafine obtained the best therapeutic response. We compare both etiological agents in terms of their different epidemiological, clinical and therapeutic features (AU)

Actualización en dermatopatología / Dermatopathology Update

Este último año ha sido rico en novedades dermatopatológicas que se vislumbran como el inicio de una nueva era. Los avances en las técnicas de biología molecular y su simplificación, que las hace asequibles al uso rutinario, han supuesto un cambio radical en el concepto de lesiones como el melanoma, cuya caracterización genética será en el futuro un requisito indispensable para establecer el diagnóstico, el pronóstico y la terapéutica. Las innovaciones tecnológicas también han llegado a los servicios de Dermatología; la introducción de ecógrafos ha propiciado el uso de técnicas de citología por punción-aspiración que pueden ser teñidas y estudiadas de manera inmediata, facilitando el diagnóstico de lesiones superficiales y ganglionares, y permitiendo la estadificación de neoplasias como el melanoma. La aparición de tratamientos oncológicos dirigidos contra «dianas terapéuticas» ha conllevado la introducción de sistemas más sensibles y específicos para su idetificación, ha reactivado el interés por patologías olvidadas como el carcinoma basocelular agresivo y ha comportado la aparición de reacciones dermatológicas que, junto con las secundarias a fármacos biológicos, empezamos a reconocer. Técnicas consolidadas como la inmunohistoquímica continúan progresando con la incorporación de nuevos anticuerpos que contribuyen de manera notable al diagnóstico. Este año, además, se han descrito o caracterizado nuevas entidades clinicopatológicas, y entre ellas 2 nuevos tipos de melanoma. También se ha avanzado en el conocimiento de otras, como el linfoma T pleomórfico CD4+ de células pequeñas/medianas. Estos temas, junto con novedades en tumores anexiales, alopecias y otras lesiones, serán comentados en esta revisión (AU)

This past year has seen a wealth of new developments in dermatopathology that appear to herald the dawning of a new era. Advances in molecular biology and the simplification of techniques have put molecular tests within reach of routine clinical practice and led to a radical change in our approach to lesions such as melanoma; in the future, the genetic characterization of these lesions will be an essential requirement for establishing diagnosis, prognosis, and therapy. Technological innovations have also reached dermatology departments: the introduction of ultrasound scans has propitiated the use of fine-needle aspiration cytology, which allows samples to be stained and studied immediately, thereby facilitating diagnosis of superficial and lymph-node lesions, and allowing staging of tumors such as melanoma. Targeted cancer therapies have led to the introduction of more sensitive and specific systems for identifying new targets, have reawakened interest in forgotten diseases such as aggressive basal cell carcinoma, and have led to dermatological reactions that, together with those caused by biologic drugs, we are just beginning to recognize. Consolidated techniques such as immunohistochemistry continue to advance with the addition of new antibodies that contribute considerably to improved diagnosis. New clinicopathologic diseases have also been described or characterized this year, including 2 new types of melanoma, and progress has been made in our knowledge of other diseases, such as primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma. These topics, together with new developments in adnexal tumors, alopecia, and other lesions, will be discussed in this review (AU)

Torsión anexial: experiencia en el Complejo Hospitalario Universitario de Albacete / Adnexal torsion: experience at the Albacete University Hospital Complex

Objetivo. Evaluar el manejo de las torsiones anexiales en nuestro centro durante un periodo de 10 años. Pacientes y métodos. Estudiamos los casos de torsión anexial en pacientes en edad reproductiva manejados quirúrgicamente entre los años 1997 y 2007. Analizamos retrospectivamente los hallazgos clínicos, ecográficos, quirúrgicos e histológicos, así como el tratamiento realizado y el seguimiento de dichas pacientes. Resultados. Identificamos 25 casos de torsión anexial en 24 pacientes. El 92% tenía dolor abdominal, el 32% náuseas y/o vómitos, el 4% fiebre y el 54,2% leucocitosis. El hallazgo ecográfico más frecuente fue de tumoración compleja (52,2%). Sólo sospechamos torsión en la primera valoración en el 8% de los casos y antes de la cirugía en el 36%. El 72% de las torsiones involucraron al anejo derecho y el 52% a un anejo tumoral. La sospecha de necrosis se confirmó en el 66,6% y el diagnóstico histopatológico más frecuente fue el de teratoma (28,6%). Realizamos tratamiento conservador mediante destorsión y/o quistectomía en el 40% y en estas pacientes el control posterior fue normal. Conclusiones. El síntoma más frecuente cuando existe una torsión anexial es el dolor abdominal y la sospecha clínica en la primera valoración es poco frecuente, siendo habitual el diagnóstico durante la cirugía. Cuando se torsiona un anejo tumoral suele haber un teratoma y en muchas ocasiones realizamos tratamiento conservador con buena evolución (AU)

Objective. To evaluate the management of adnexal torsion in our center during a 10-year period. Patients and methods. We studied cases of adnexal torsion in patients of reproductive age treated surgically between 1997 and 2007. The clinical, ultrasonographic, surgical and histological findings, as well as the treatment and follow-up of these patients, were retrospectively analyzed. Results. We identified 25 cases of adnexal torsion in 24 patients. Abdominal pain was present in 92%, nausea and/or vomiting in 32%, fever in 4% and leucocytosis in 54.2%. The most frequent echographic findings were complex tumors (52.2%). Torsion was suspected in the first evaluation in only 8% of the cases and before surgery in 36%. Most (72%) of the torsions involved the right adnexa and 52% a tumoral adnexa. Suspicion of necrosis was confirmed in 66.6% and the most frequent histopathological diagnosis was teratoma (28.6%). Conservative treatment was performed through detorsion and/or cystectomy in 40% and in patients whose subsequent clinical course was normal. Conclusions. When an adnexal torsion is present, the most frequent symptom is abdominal pain. Clinical suspicion in the first evaluation is infrequent, and diagnosis is usually made during surgery. When a tumoral adnexa is torsioned, a teratoma is usually present. Conservative treatment is often performed with good results (AU)

Tumoración anexial gigante en paciente con obesidad mórbida: abordaje multidisciplinario / Giant adnexal mass in a morbidly obese patient: multidisciplinary approach

Objetivo: Describir el manejo clínico y terapéutico de las masas anexiales gigantes en pacientes con alto riesgo operatorio debido a obesidad mórbida. Sujetos y métodos: Se presenta una paciente con un índice de masa corporal > 60, afectada de un fibroma ovárico de 13 kg que ocupa toda la cavidad abdominal. Resultados: La imposibilidad de utilizar técnicas de imagen accesorias llevó a realizar un abordaje laparoscópico para descartar malignidad. Se procedió una histerectomía con doble anexectomía laparotomía por un equipo quirúrgico multidisciplinario sin complicaciones. Conclusiones: El manejo de las pacientes con obesidad mórbida y patología anexial gigante por un equipo multidisciplinar es seguro y eficaz (AU)

Objective: To describe the clinical and therapeutic management of giant adnexal masses in morbidly obese patients at high surgical risk. Subjects and methods: We report the case of a patient with a body mass index of over 60 and a 13-kg ovarian fibroma occupying the entire abdominal cavity. Results: Due to the impossibility of using accessory imaging techniques, we employed a laparoscopic approach to exclude malignancy. Hysterectomy with bilateral salpingo-oophorectomy was performed by a multidisciplinary team without complications. Conclusions: Morbidly obese patients with giant adnexal masses can be safely and effectively managed by a multidisciplinary team (AU)

Intralesional interferon alpha-2b therapy for adnexal Kaposi sarcoma.

PURPOSE: To describe a case of adnexal Kaposi's sarcoma successfully treated with intralesional interferon alpha 2b injections. METHODS: Case Report. RESULTS: A 31 year old black Haitian male presents with severe nodular induration and erythema of both eyelids with hemorrhagic infiltration of the bulbar and palpebral conjunctivae, advanced on the right. Clinical, histological, and immunohistochemical examination yielded the diagnosis of Kaposi's sarcoma. The patient was subsequently diagnosed with AIDS, started on highly active anti-retroviral therapy (HAART) and systemic doxorubicin, and initially received topical 3 million IU per mL of interferon alpha-2b eyedrops (Intron-A(R), Schering Corporation, Kenilworth, NJ) every 6 hours to the right eye for 9 weeks. This systemic therapy resulted in minimal clinical response with a CD4 count increase from 12 to 61 cells per microliter. Progressive entropion and corneal compromise on the right prompted a trial of intralesional injections of interferon alpha-2b. Over a 4 week period, 3 injections (3 million IU in 0.5mL, 1.5 million IU in 0.5mL, and 1.5 million IU in 0.5mL) were given. This resulted in a dramatic reduction in tumor burden and lid edema, an improvement in lid position, and corneal healing. CONCLUSION: Ocular adnexal and conjunctival Kaposi's sarcoma was successfully treated with 3 adjuvant intralesional interferon alpha-2b injections leading to a dramatic decrease in tumor mass.

Rippled-pattern trichoblastoma with apocrine differentiation arising in a nevus sebaceus: report of a case and review of the literature.

A 36-year-old man developed a papular growth in a portion of a nevus sebaceus on the post-auricular scalp. Excision showed typical histologic changes of nevus sebaceus including epidermal papillomatosis with reduced numbers of hair follicles as well as numerous sebaceous glands high in the dermis that focally emptied directly to the overlying epidermis. Histologic sections of the papular growth at the superior pole of the nevus sebaceus showed a proliferation of cytologically bland basaloid epithelial tumor lobules both in the superficial dermis, with multiple connections to the epidermis, and within the deeper dermis in a nodular growth pattern demonstrating papillary mesenchymal bodies. Ductal structures with apocrine-type decapitation secretion were present. There was prominent palisading of nuclei in rows parallel to one another, alternating with bands of homogenous eosinophilic stromal material forming a ripple pattern resembling the Verocay bodies of schwannoma. The histologic features resembled those of rippled-pattern trichoblastoma with apocrine differentiation arising in a nevus sebaceus, an association not previously described. We discuss this case as well as review the literature on rippled-pattern trichoblastoma.

Adnexal clear cell carcinoma with comedonecrosis: clinicopathologic analysis of 12 cases.

CONTEXT: Cutaneous clear cell tumors can pose a diagnostic challenge even to the experienced dermatopathologist; this is partly because of limitations of existing diagnostic categories. OBJECTIVE: To describe a previously unrecognized, distinctive cutaneous adnexal carcinoma capable of an aggressive clinical course. DESIGN: Clinicopathologic analysis of a series of 12 cases. RESULTS: The patients were older individuals (median age, 71 years) with equal gender frequency. The lesions showed wide anatomic distribution with predilection for the head and neck area, especially the scalp. The lesions presented as rapidly growing, erythematous to flesh-colored, solitary papules/nodules that were capable of quickly reaching a size of several centimeters. Histologically, adnexal clear cell carcinoma with comedonecrosis was characterized by dermal proliferation of nests of epithelial cells showing distinctive zonal arrangement. The periphery of the tumor nests was formed by squamoid cells merging with centrally located clear cell areas containing foci of comedonecrosis. The lesions often showed multilobular or trabecular growth pattern and infiltrating border. Nuclear pleomorphism was variable; mitotic count ranged from 2 to 32/mm2 (median, 8/mm2). No ductal, cuticular, or apocrine differentiation was seen. All cases showed expression of epithelial membrane antigen and cytokeratin 17 in clear cells, with focal carcinoembryonic antigen expression in some cases. Follow-up (average, 37 months) revealed local recurrence (4 cases) and regional and distant metastases (2 cases). CONCLUSIONS: Adnexal clear cell carcinoma with comedonecrosis appears to be a distinctive adnexal neoplasm that has to be distinguished from more indolent squamous cell and tricholemmal carcinomas.

Female adnexal tumor of probable Wolffian origin diagnosed during the staging evaluation of extranodal diffuse large B-cell lymphoma.

Female adnexal tumor of probable Wolffian origin (FATPWO) is a rare neoplasm arising within the leaves of broad ligament or hanging from it or a fallopian tube. A 55-year-old female patient with the diagnosis of non-Hodgkin lymphoma is presented. The staging procedure revealed a pelvic mass that was not regressed by chemotherapy. Explorative laparotomy showed FATPWO. The extremely rare coexistence of FATPWO in the staging evaluation of non-Hodgkin lymphoma is reported. According to our knowledge, this is the first case of FATPWO coexisting with extranodal diffuse large B-cell lymphoma in the English-language literature.

Epidermal nevus syndrome associated with adnexal tumors, spitz nevus, and hypophosphatemic vitamin D-resistant rickets.

The epidermal nevus syndrome is the association of epidermal nevi with abnormalities in other organ systems, most commonly the central nervous system, the skeletal system, and the eyes. We present a patient with epidermal nevus syndrome associated with hypophosphatemic vitamin D-resistant rickets and multiple adnexal and spindle cell tumors.

Síndrome de Birt-Hogg-Dubé: presentación de un caso y revisión de la literatura / Birt-Hogg-Dubé syndrome: case report and literature review

Se presenta una paciente de 59 años de edad con síndrome de Birt-Hogg-Dubé. Se describen las características clínicas e histopatológicas, así como las asociaciones que pueden observarse en esta infrecuente enfermedad

Quiste triquilemal proliferante: presentación de un caso gigante y otro maligno / Proliferating trichilemmal cyst: presentation of a giant and a malign case

Se presenta el caso de un paciente de sexo masculino con un quiste triquilemal proliferante maligno y otro de una mujer con un quiste triquilemal proliferante gigante. Se discute la histogénesis de estos tumores, así como las características clínicas, histopatológicas y estudios complementarios que permiten detectar el comportamiento maligno de dichas lesiones para efectuar la terapéutica adecuada. Se resalta la utilidad del marcador de proliferación nuclear Ki 67 como indicador de estos tumores

Síndrome de Birt-Hogg-Dubé: presentación de un caso y revisión de la literatura / Birt-Hogg-Dubé syndrome: case report and literature review

Se presenta una paciente de 59 años de edad con síndrome de Birt-Hogg-Dubé. Se describen las características clínicas e histopatológicas, así como las asociaciones que pueden observarse en esta infrecuente enfermedad (AU)

Quiste triquilemal proliferante: presentación de un caso gigante y otro maligno / Proliferating trichilemmal cyst: presentation of a giant and a malign case

Se presenta el caso de un paciente de sexo masculino con un quiste triquilemal proliferante maligno y otro de una mujer con un quiste triquilemal proliferante gigante. Se discute la histogénesis de estos tumores, así como las características clínicas, histopatológicas y estudios complementarios que permiten detectar el comportamiento maligno de dichas lesiones para efectuar la terapéutica adecuada. Se resalta la utilidad del marcador de proliferación nuclear Ki 67 como indicador de estos tumores (AU)