Carcinoma anexial microquístico: la cirugía micrográfica de Mohs como tratamiento de elección / Microcystic Adnexal Carcinoma: Mohs Micrographic Surgery as the Treatment of Choice

Introducción y objetivos. El carcinoma anexial microquístico (CAM) es un tumor raro y agresivo que clínicamente se manifiesta como un nódulo subcutáneo localizado en las regiones de cabeza y cuello. Esta tumoración puede ser clínica e histológicamente confundida con otras lesiones cutáneas benignas o malignas, lo que con frecuencia conduce a un tratamiento inicial erróneo. La principal complicación del CAM es la alta morbilidad y la elevada tasa de recurrencia tras escisión local amplia. Estudios preliminares recientes han mostrado unas mayores tasas de curación mediante cirugía micrográfica de Mohs (CMM). Material y métodos. Se revisaron las historias clínicas de 6 pacientes consecutivos con CAM tratados mediante CMM en nuestro Servicio de Dermatología entre 1995 y 2007. Resultados. Todos los casos se localizaron en la cabeza y el 100 % fue tumor primario. En el 70 % de los casos el tumor fue inicialmente mal diagnosticado de carcinoma basocelular. No se detectó invasión perineural en ninguno de los casos y la recurrencia estuvo ausente en todos los pacientes tras un periodo de seguimiento comprendido entre 1 y 12 años posteriores a la CMM. Conclusiones. La ausencia de afectación perineural y de una importante atipia celular puede deberse al carácter primario de la tumoración. Ello justificaría la necesidad de un tratamiento definitivo radical inicial del tumor primario para evitar las complicaciones que implica una recidiva tumoral. La localización y la ausencia de recurrencia en todos nuestros casos tratados mediante CMM apoyan el uso de esta técnica como el tratamiento de elección para el CAM (AU)

Introduction and objectives. Microcystic adnexal carcinoma is a rare and aggressive tumor that manifests clinically as a subcutaneous nodule located on the head or neck. The tumor can be confused clinically and histologically with other benign and malignant skin lesions, often leading to inappropriate initial treatment. The chief concern with microcystic adnexal carcinoma is the elevated morbidity and the high rate of recurrence after wide local excision. Recent preliminary studies point to higher cure rates with Mohs micrographic surgery. Material and methods. We reviewed the medical histories of 6 consecutive patients with microcystic adnexal carcinoma who underwent Mohs micrographic surgery in our dermatology department between 1995 and 2007. Results. In all cases, lesions were located on the head and were primary tumors. Seventy percent of the tumors were wrongly diagnosed initially as basal cell carcinoma. Perineural invasion was not detected in any patient, and all were free of recurrence after between 1 and 12 years of postoperative follow-up. Conclusions. The absence of perineural involvement and substantial cell atypia can be attributed to the lesions being primary tumors. This would provide a rationale for definitive radical treatment of the primary tumor from the outset to avoid the complications associated with recurrence. The site and the absence of recurrence in all our patients who underwent Mohs micrographic surgery support the use of this technique as the treatment of choice in microcystic adnexal carcinoma (AU)

Dactilitis crónica indolora como hallazgo inicial de un adenocarcinoma pulmonar diseminado / Chronic Painless Dactylitis as the Initial Finding in Disseminated Lung Adenocarcinoma

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[Microcystic adnexal carcinoma: Mohs micrographic surgery as the treatment of choice]. / Carcinoma anexial microquístico: la cirugía micrográfica de Mohs como tratamiento de elección.

INTRODUCTION AND OBJECTIVES: Microcystic adnexal carcinoma is a rare and aggressive tumor that manifests clinically as a subcutaneous nodule located on the head or neck. The tumor can be confused clinically and histologically with other benign and malignant skin lesions, often leading to inappropriate initial treatment. The chief concern with microcystic adnexal carcinoma is the elevated morbidity and the high rate of recurrence after wide local excision. Recent preliminary studies point to higher cure rates with Mohs micrographic surgery. MATERIAL AND METHODS: We reviewed the medical histories of 6 consecutive patients with microcystic adnexal carcinoma who underwent Mohs micrographic surgery in our dermatology department between 1995 and 2007. RESULTS: In all cases, lesions were located on the head and were primary tumors. Seventy percent of the tumors were wrongly diagnosed initially as basal cell carcinoma. Perineural invasion was not detected in any patient, and all were free of recurrence after between 1 and 12 years of postoperative follow-up. CONCLUSIONS: The absence of perineural involvement and substantial cell atypia can be attributed to the lesions being primary tumors. This would provide a rationale for definitive radical treatment of the primary tumor from the outset to avoid the complications associated with recurrence. The site and the absence of recurrence in all our patients who underwent Mohs micrographic surgery support the use of this technique as the treatment of choice in microcystic adnexal carcinoma.

Comparative study between cold air analgesia and supraorbital and supratrochlear nerve block for the management of pain during photodynamic therapy for actinic keratoses of the frontotemporal zone.

BACKGROUND: Photodynamic therapy (PDT) is an effective treatment for actinic keratoses, Bowen's disease and basal cell carcinoma. The main drawback of PDT is pain during application. OBJECTIVES: To compare the efficacy of supratrochlear and supraorbital nerve block with cold air analgesia to control the pain experienced during PDT. METHODS: A controlled open clinical trial was conducted in 34 patients having multiple actinic keratoses in the frontal region treated with PDT. On one side of the frontal region the supratrochlear and supraorbital nerves were blocked, while on the other side cold air was used as the method of analgesia. Pain was recorded on a visual analogue scale after treatment. RESULTS: Thirty-one of 34 patients reported less pain in the zone treated with nerve block. This difference was statistically significant. CONCLUSIONS: Nerve block is superior to cold air and is an easy, safe, effective means of controlling the pain associated with PDT.

[Adenoid cystic carcinoma]. / Carcinoma adenoide quístico.

Adenoid cystic carcinoma is an uncommon tumor of the head and neck. Although it is mainly located in the salivary gland, a skin location has also been described. Metastases are rare, but 50 % of the cases relapse. A 65-year-old male patient had a lesion in the upper lip. After resection, the histological diagnosis was adenoid cystic carcinoma. Treatment was completed with radiotherapy. Ten years later, a nodule was detected in the neck. Its histological diagnosis was lymphatic metastasis due to adenoid cystic carcinoma. Primary cutaneous adenoid cystic carcinoma is a very uncommon tumor in which treatment consists in extensive local excision with free margins. Radiotherapy is not curative and should be reserved for palliative treatments. Multicenter, prospective studies are necessary to determine the best treatment and especially the adjuvant treatment for adenoid cystic carcinoma.

Carcinoma adenoide quístico / Adenoid cystic carcinoma

El carcinoma adenoide quístico es un tumor poco común de cabeza y cuello, que si bien se localiza preferentemente en la glándula salivar, también se ha descrito su localización cutánea. Las metástasis son raras, pero un 50 % de los casos recidivan. Un paciente de 65 años de edad presentó una lesión en el labio superior, que tras la resección, el diagnóstico histopatológico fue de carcinoma adenoide quístico. El tratamiento se completó con radioterapia. Diez años más tarde se detectó un nódulo en el cuello cuyo diagnóstico histopatológico fue de metástasis linfática por carcinoma adenoide quístico. El carcinoma adenoide quístico, primariamente cutáneo, es un tumor muy poco frecuente en el que el tratamiento consiste en la exéresis local amplia con márgenes libres. La radioterapia no es curativa y debería reservarse para tratamientos paliativos. Se necesitan estudios multicéntricos prospectivos para determinar el tratamiento óptimo y, especialmente, el tratamiento adjuvante del carcinoma adenoide quístico

Adenoid cystic carcinoma is an uncommon tumor of the head and neck. Although it is mainly located in the salivary gland, a skin location has also been described. Metastases are rare, but 50 % of the cases relapse. A 65-year-old male patient had a lesion in the upper lip. After resection, the histological diagnosis was adenoid cystic carcinoma. Treatment was completed with radiotherapy. Ten years later, a nodule was detected in the neck. Its histological diagnosis was lymphatic metastasis due to adenoid cystic carcinoma. Primary cutaneous adenoid cystic carcinoma is a very uncommon tumor in which treatment consists in extensive local excision with free margins. Radiotherapy is not curative and should be reserved for palliative treatments. Multicenter, prospective studies are necessary to determine the best treatment and especially the adjuvant treatment for adenoid cystic carcinoma

Vesiculous prurigo pigmentosa in a 13-year-old girl: good response to isotretinoin.

Prurigo pigmentosa is a rare inflammatory disease of unknown origin, first reported from Japan, with only 33 cases described in non-Japanese patients. We describe a 13-year-old girl with a pruriginous symmetrical eruption of papules and vesicles affecting her back, neck and chest of 1 month duration. She remembered a similar, but lighter eruption, 2 months before. As the initial diagnosis was of a vesiculobullous form of Darier disease, treatment with isotretinoin 40 mg/day was started with good response. Histological study showed a superficial perivascular and interstitial dermatitis composed predominantly of lymphocytes. The epidermis was spongiotic, with exocytosis of lymphocytes and some neutrophils and necrotic keratinocytes. All these findings were consistent with prurigo pigmentosa. The lesions resolved leaving a light brown reticulate hyperpigmentation. Prurigo pigmentosa has never been reported in prepubescent patients, the vesiculobullous forms are unusual, and the only treatments used previously are sulphonamides, tetracyclines and macrolides. We report a 13-year-old Caucasian girl with vesiculobullous prurigo pigmentosa successfully treated with isotretinoin.

Multinucleate cell angiohistiocytoma: a new case report.

Multinucleate cell angiohistiocytoma (MCAH) was first described by Smith and Wilson-Jones in 1985. It is an uncommon entity but probably underdiagnosed because of lack of recognition by clinicians and pathologists. We report a 47-year-old man with asymptomatic grouped violaceous papules on the dorsum of the hands for 3 years. The histopathological and immunopathological features of our case revealed characteristics of MCAH similar to the initial description of Smith and Wilson-Jones and other reports.

Hiperqueratosis epidermolítica en forma de mosaico / Mosaic form of epidermolytic hyperkeratosis

Presentamos el caso de una niña de 6 años de edad con lesiones hiperqueratósicas distribuidas siguiendo las líneas de Blaschko en todo el hemicuerpo izquierdo. La biopsia cutánea demostró un patrón de hiperqueratosis epidermolítica. Se trató con acitretino a dosis de 0,5 mg/ kg/d con buena respuesta. Recordamos la importancia de este mosaicismo por la posibilidad de tener hijos afectados de eritrodermia ictiosiforme congénita ampollosa en la descendencia. (AU)

Metastatic umbilical cancer--Sister Mary Joseph's nodule: report of two cases.

Two cases of metastatic umbilical carcinoma (Sister Mary Joseph's nodule) are reported. In one of the cases, the umbilical metastasis was the first sign of the disease, whereas in the other case, the patient had a 5-year history of internal malignancy when the umbilical nodule appeared. In both cases, the cutaneous metastasis was followed by death in a short period of time.

[Skin involvement in Hodgkin's disease]. / Afectación cutánea por la enfermedad de Hodgkin.

We report a case of a previously diagnosed patient of Hodgkin's disease, nodular sclerosis type and IVLDB stage. This patient subsequently presented a dermatologic nodule. A specimen cutaneous biopsy was performed and the histopathologic study demonstrated a dermal infiltrate with many classic Reed-Sternberg giant cells. These findings were consistent with the skin involvement in Hodgkin's disease. Specific cutaneous lesions are rare in this lymphoma and therefore it may be argued that our patient had a lymphomatoid papulosis. However, the protracted clinical course would not support this concept, because the dermatologic lesion responded to chemotherapy and it is well-known that the clinical course of the lymphomatoid papulosis is unaffected by these treatments. We concluded that our patient is a case of specific skin involvement in Hodgkin's disease.

Prevalence and risk factors of hepatitis B in Spanish prostitutes.

Eighty prostitutes were tested by solid-phase radioimmunoassay for serum markers of hepatitis B virus (HBV). Of 8 (10%) with hepatitis B surface antigen (HBsAg), 6 (75%) also had hepatitis Be antigen (HBeAg). Antibodies to HBsAg (anti-HBs) and to hepatitis B core antigen (anti-HBc) were found in 52 (65%). Antibodies to HBeAg (anti-HBe) were positive in 32 (40%). Anti-HBc alone was found in 5 (6%) and anti-HBs alone in 2 (2%). Sixty-seven (84%) were positive for at least one HBV marker and 13 (16%) were still susceptible to infection. Hepatitis B markers were more prevalent in prostitutes than in the normal Spanish population. Age, a history of sexually transmitted diseases (STD), drug abuse and promiscuity are factors which were highly related to hepatitis B markers. We concluded that screening prostitutes for the presence of markers and vaccinating those who are negative would be worth while.

Tooth pits: an early sign of tuberous sclerosis.

The pit-shaped enamel defects seem to be pathognomonic of tuberous sclerosis and its detection may be an important help in the early diagnosis of this disease, especially in oligosymptomatic cases and when the dermatologic signs are still absent or only slightly evident.