M.J. González-Beato

Nodular lesions arising in a large congenital melanocytic naevus in a newborn with eruptive disseminated Spitz naevi.

Congenital malignant melanoma within a pre‐existing large congenital melanocytic naevus (CMN) is exceedingly rare. Its incidence is difficult to determine due to the small number of reported cases and because of problems associated with diagnosis. Some benign nodular proliferations (called proliferative nodules) arising in CMN, while rare, are significantly more common and can mimic malignant melanoma clinically or histologically. There are no reported cases of congenital melanoma or benign proliferative nodules in CMN in patients who also had eruptive disseminated Spitz naevi. We describe a girl who was noted to have a dark‐brown plaque with several large erythematous nodules affecting the scalp at delivery, in addition to multiple erythematous dome‐shaped papules that developed in a disseminated manner over several months, beginning at 10u2003days of age. It was difficult, not only clinically but also histologically, to determine the benign or malignant nature of all of these lesions. As primary cutaneous melanoma, atypical proliferative nodules in CMN, bland CMN or CMN with foci of increased cellularity and Spitz naevi show clear differences in the genetic aberration patterns, comparative genomic hybridization (CGH) could be a diagnostic help in ambiguous cases such as this. CGH performed on this patient showed multiple DNA copy number changes in the most atypical nodule, but such alterations could not be found in the remainder of the lesions. CGH showed differences between the nodular lesions that occurred in the CMN and helped us in supporting the diagnosis of this unique case of benign proliferative nodules and a possible congenital melanoma arising in a large CMN, associated with multiple widespread eruptive Spitz naevi.

Tongue ulcer in a child: Riga-Fede disease

JEADV 2006, 20, 1328–1399

Dermatoscopic characteristics of acrochordon-like basal cell carcinomas in Gorlin-Goltz syndrome

BACKGROUNDnGorlin-Goltz syndrome (GGS) is an autosomal-dominant disease characterized by the early onset of multiple basal cell carcinomas (BCCs), among other findings. Clinically, the BCCs may appear as soft pedunculated neoplasms that can be mistaken for true acrochordons.nnnOBJECTIVEnWe sought to describe the dermatoscopic characteristics of small acrochordon-like or polypoid BCCs in a child with GGS, and to perform histopathologic correlation.nnnMETHODSnAcrochordon-like growths from a child with GGS were studied. Clinical records and digital dermatoscopic images were collected, and excision and histopathologic examination of the most representative lesions were performed.nnnRESULTSnSome acrochordon-like lesions showed specific dermatoscopic criteria for BCC, including multiple blue-gray globules and arborizing telangiectasia. Other polypoid lesions, especially the smaller ones, exhibited characteristics that suggested BCC, such as isolated blue-gray globules, small blue-gray ovoid nests, and fine elongated telangiectases.nnnLIMITATIONSnConclusions are limited by the small sample size.nnnCONCLUSIONnDermatoscopy may be a useful diagnostic tool to analyze acrochordon-like lesions in children and to facilitate early diagnosis and treatment of BCCs in patients with GGS.

Enfermedad de Fox-Fordyce

Fox-Fordyce disease is an infrequent inflammatory disorder characterized by the appearance of pruritic papules with follicular distribution, localized in areas where apocrine glands are present. Its etiopathogenesis is not clearly known, and it involves the obstruction of the apocrine excretory duct as an early phenomenon in the process. We present a case of histologically confirmed Fox-Fordyce disease with high clinical expressivity in a 16-year-old female.

Subcutaneous mycosis produced by Aureobasidium pullulans in a renal transplant recipient.

JEADV 2006, 20, 214–238

Carcinoma sebáceo extraocular de presentación atípica

Resumen —El carcinoma sebaceo es un tumor cutaneo maligno poco frecuente. Aparece en el 75 % de los casos en localizacion oculopalpebral, rica en varios tipos de glandulas sebaceas. La cabeza y cuello son las regiones extraoculares donde se han descrito con mayor frecuencia. Su curso clinico es agresivo y son habituales las recurrencias locales y las metastasis a distancia. Se presenta un caso de una mujer de 79 anos que fue diagnosticada de un carcinoma sebaceo extraocular en mejilla derecha, varias hiperplasias sebaceas y un carcinoma intraepidermico con diferenciacion sebacea. Tras realizar una completa evaluacion de historia familiar y personal de la paciente se descarto la asociacion con el sindrome de Muir-Torre.

Dermoscopic findings and histological correlation of the acral volar pigmented maculae in Laugier–Hunziker syndrome

Laugier–Hunziker syndrome (LHS) is an acquired, benign, macular hyperpigmentation of the lips and oral mucosa, often associated with pigmentation of the nails. Volar acral maculae on the palms and fingertips of patients affected by LHS are a typical feature of this rare entity. Dermoscopic examination of these maculae has been described in a previous report, in which authors found a parallel‐furrow pattern. We describe two cases in which a parallel‐ridge pattern (PRP) was found on the dermoscopic examination of the pigmented acral lesions. Histological examination showed increased melanin in basal keratinocytes, which was most prominent in those located at the crista intermedia profunda, that is, in the epidermal rete ridges underlying the surface ridges. In our study, dermoscopic features of the pigmented maculae found on LHS differed from those previously described. In addition, by means of this case report, the histological features of these lesions are described for the first time, showing an excellent correlation with dermoscopy. The reported cases prove that although the PRP is very specific of melanoma, it is also possible to find it in benign lesions. Therefore, we must be familiar with the differential diagnosis of PRP, and take into consideration the clinical context in which we find it. Further studies are needed to increase our knowledge on the histological and dermoscopic features of acral pigmented maculae of LHS.

Childhood actinic lichen planus: Successful treatment with antimalarials

Actinic lichen planus (ALP) is a photosensitive variant of lichen planus, affecting mainly young adults of Middle Eastern descent. We report a case of ALP in a 9‐year‐old Colombian girl with an excellent response to hydroxychloroquine and photoprotection.

Tratamiento tópico de las metástasis cutáneas de melanoma con imiquimod

Resumen El tratamiento de las metastasis cutaneas de melanoma puede ser dificil en muchos casos debido a la edad de los pacientes, asi como al numero, tamano y localizacion de las lesiones. Se presenta el caso de un varon de 82 anos con metastasis cutaneas de melanoma en el cuero cabelludo que respondieron satisfactoriamente al tratamiento con imiquimod al 5% en crema. El imiquimod es un inmunomodulador de uso topico con actividad antiviral y antineoplasica. El presente caso, junto con otros recientemente publicados, apoya la utilidad de este tratamiento en casos seleccionados de metastasis cutaneas de melanoma, al menos con un fin paliativo.The treatment of skin metastases of melanoma can be difficult in many cases because of the patients age, as well as the number, size and location of the lesions. We present the case of an 82-year-old male with melanoma skin metastases on the scalp, which responded satisfactorily to treatment with 5 % imiquimod cream. Imiquimod is a topical immunomodulator with antiviral and antineoplastic action. This case, along with others that have recently been published, supports the usefulness of this treatment in selected cases of melanoma skin metastases, at least for palliative purposes.

Características clínico-patológicas de los melanomas cutáneos diagnosticados en un hospital terciario mediterráneo entre 1990 y 2004: comparación entre sexos, por grupos de edad y evolución a lo largo del tiempo

Resumen Introduccion En las ultimas decadas esta aumentando la incidencia de melanoma maligno (MM) y el diagnostico precoz sigue siendo esencial para conseguir su curacion. Nuestro objetivo fue analizar los MM cutaneos diagnosticados durante 15 anos en un hospital terciario para comprobar si existe una tendencia favorable hacia un diagnostico cada vez mas precoz e intentar identificar grupos de poblacion con MM de peor pronostico. Material y metodos Analisis retrospectivo de los melanomas cutaneos primarios analizados en el Departamento de Anatomia Patologica del Hospital La Paz entre 1990 y 2004. Resultados Se diagnosticaron en total 526 melanomas. El espesor medio de Breslow fue de 2,63 mm (desviacion estandar [DE] 4,84) y la mediana de 0,98 mm (0-65 mm); el tamano medio (diametro mayor) fue de 16,59 mm (DE 12,11); el tipo histologico mas frecuente fue el melanoma de extension superficial y la localizacion mas habitual el tronco. Los melanomas detectados en varones tendieron a ser mas grandes y de mayor espesor (pxa0=xa00,05) que los de las mujeres. Los mayores de 60 anos consultaron con tumores significativamente mas gruesos y de mayor diametro que los pacientes mas jovenes. A lo largo de estos anos, ha aumentado progresivamente el numero de MM, pero han disminuido el Breslow medio y el tamano. Conclusiones El diagnostico del melanoma en nuestro medio es cada vez mas precoz, pero los varones y los mayores de 60 anos en ocasiones aun consultan con tumores localmente avanzados.