V. Alegre de Miquel

Two Cases of Eruptive Pyoderma Gangrenosum Associated with Cocaine Use

We present the cases of 2 regular cocaine users aged 30 and 37 years who developed a cutaneous condition with an identical presentation and a similar clinical course. The lesions first appeared as eruptions on the back and then spread to other parts of the body. They consisted of painful inflammatory nodules that developed into abscesses and ulcers that grew outwards. They had a granulomatous base and irregular violaceous edges. The results of histopathologic studies were compatible with the clinical diagnosis of pyoderma gangrenosum, and additional tests ruled out underlying diseases. After several therapeutic failures with usual treatments, the patients responded quickly to infliximab after interrupting their cocaine consumption. The association between pyoderma gangrenosum and cocaine use was clear as both suffered clinical relapses at the same time as they started using the drug again.

Cutaneous sarcoidosis in a melanoma patient under Ipilimumab therapy

A 46-year-old woman was diagnosed in 2013 with a choroid melanoma treated with plaque brachytherapy. A year later, metastatic disease in the liver was confirmed with biopsy and chemotherapy with Dacarbazine was administered. After three cycles, a computerized tomography (CT) evidence disease progression in the liver and therapy with the anti-CTLA4 antibody, Ipilimumab, was started (Dose: 3 mg/Kg. Total dose per cycle: 180 mg). One week after the fourth administration, the patient was remitted to our department because of generalized pruritus and cutaneous lesions on the lower limbs. Clinical examination revealed bilateral and symmetrical erythematous pretibial plaques and nodules (FIG. 1). Both lesions were biopsied, and pathology showed noncaseating granulomas in the dermis consistent with sarcoidosis (FIG. 2). Blood tests revealed an increased angiotensin-converting enzyme (107 U/L. Normal range, 8–52 U/L). The pretibial plaques were treated with topical clobetasol with a significant improvement. During radiological follow-up progression of the liver metastatic disease was detected and administration of Ipilimumab was stopped following the induction therapy, replacing the treatment. The patient is now participating in a new clinical trial. One month after discontinued Ipilimumab she reported progressive dyspnea. CT showed a new nodule on the right lower lobe and fibrous tracts and functional lung tests revealed severe decrease in carbon monoxide diffusing capacity (20% of predicted value). A bronchoscopy was normal and the broncho-alveolar lavage showed lymphocytosis with an inverted CD4:CD8 ratio (0.36). Although the CD4/CD8 ratio is usually high in pulmonary sarcoidosis, it was decided to initiate treatment with oral corticosteroids. After a few days the patient showed clinical improvement without relapse of dyspnea until now.

Biopsia de ganglio centinela en melanoma. Valor pronóstico y correlación con el índice mitótico. Experiencia en un hospital terciario

BACKGROUND The prognostic value of sentinel lymph node (SLN) biopsy findings and mitotic activity in melanoma has been confirmed in the literature, but the relation between them has not been well established. OBJECTIVES The main objective was to describe and analyze the correlation between SLN biopsy results and the mitotic rate in patients treated for melanoma in our hospital. METHODS A total of 139 consecutive patients who underwent SLN biopsy between May 2001 and May 2009 were included. The relation between the mitotic rate and SLN status was analyzed with the χ(2) test and the Fischer exact test. RESULTS The correlation between the 2 variables was nonsignificant (P =.071) in the patient series overall, but a significant association was found in the subgroup of patients with tumors of Breslow thickness between 1 and 4mm (P =.034). The likelihood (odds ratio) of SLN positivity with a mitotic rate of less than 1 mitosis/mm2 in this subgroup was 0.838 (95% CI, 0.758-0.926). CONCLUSIONS Our findings support use of the mitotic rate to predict SLN status in melanoma tumors of intermediate thickness. Our study also shows the need for further investigation of the relation between these 2 variables in thin and thick tumors.

Úlcera vulvar aguda de transmisión no sexual asociada a infección por virus influenza A

La úlcera vulvar aguda de transmisión no sexual (úlcera de Lipschütz) es una entidad poco frecuente, caracterizada por el desarrollo de una o más úlceras de instauración brusca, grandes y profundas que aparece en pacientes sin antecedentes patológicos previos. La etiología es desconocida, existen en la literatura casos relacionados con la primoinfección por diferentes agentes infecciosos como VEB, CMV, influenza, Toxoplasma y Salmonella. En la serie de Farhi et al1 un 33% de pacientes mostraron serología positiva IgM para VEB y con técnicas de PCR se pudo demostrar la presencia del virus en las biopsias de las lesiones. Presentamos un caso de úlcera vulvar aguda que se relacionó con la infección por virus influenza A. Presentamos el caso de una paciente de 14 años, sin antecedentes de interés, que acudió al Servicio de Urgencias por úlceras genitales dolorosas de 36 horas de evolución, que habían recibido tratamiento como una primoinfección herpética (fig. 1). Refería no haber mantenido nunca relaciones sexuales y a la exploración presentaba un himen íntegro. La paciente asociaba un cuadro de 7 días de evolución de odinofagia, fiebre alta y quebrantamiento del estado general con astenia y mialgias generalizadas tratado con antitérmicos. En el momento de la exploración la paciente se encontraba afebril y presentaba úlceras vulvares de 2 cm de diámetro bilaterales y simétricas (de disposición en espejo) en los labios menores, de aspecto úlcero-necrótico y fondo fibrinoso, exudativas, muy dolorosas y malolientes, con adenopatías inguinales bilaterales reactivas. Se pautó tratamiento antibiótico (amoxicilina-clavulánico 500 mg/ 8 horas durante 7 días), antiinflamatorios orales y fomentos secantes tópicos. El estudio hematológico y bioquímico realizado mostró resultados dentro de la normalidad. Los estudios serológicos mostraron negatividad para VIH I y II,VHC, VHB, VHS I y II, parvovirus y lues; con IgG positiva pero IgM negativa para Toxoplasma gondii, CMV, VEB, virus parainp a ú b e

Hair Collar Sign Associated with Scalp Aplasia Cutis Congenita

1. Mendez T, Otero I, Garcia R, Perez B. Sindrome de Klippel-Trenaunay-Weber: presentacion de un caso. Rev Cubana Oftalmol. 2001;14:47-9. 2. Bordel MT, Miranda A. Un caso atipico de sindrome de Klippel-Trenaunay. Piel. 2005;20:306-8. 3. Gimeno P, Perez P, Lopez-Pison J, Romeo M, Galeano N, Marco M, et al. Sindrome de Klippel-Trenaunay: a proposito de tres nuevas observaciones. An Esp Pediatr. 2000;53:350-4. 4. Lee CW, Choi DY, Oh YG, Yoon HS, Kim JD. An infantile case of SturgeWeber syndrome in association with Klippel-Trenaunay-Weber syndrome and phakomatosis pigmentovascularis. J Korean Med Sci. 2005;20:1082-4. 5. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44:1005-17. 6. Elster AD, Chen MY. Chiari I malformations: clinical and radiologic reappraisal. Radiology. 1992;183:347-53. 7. Yancey MK, Lasley D, Richards DS. An unusual neck mass in a fetus with Klippel-Trenaunay-Weber syndrome. J Ultrasound Med. 1993;12:779-82.

Síndrome de Buschke-Ollendorff

Buschke-Ollendorff syndrome is a hereditary disorder that primarily affects the skin and bones. Specifically, the condition is characterized by skin growths called connective tissue nevi and bone abnormalities, most commonly a pattern of increased bone density called osteopoikilosis. Buschke-Ollendorff syndrome is classified as a disorder of connective tissues, which provide support, strength, and flexibility to organs and tissues throughout the body.

Nevus lentiginoso atípico. Estudio clínico-patológico de 14 casos

BACKGROUND Atypical lentiginous nevus (of the elderly) is a peculiar form of dysplastic nevus. Clinically, this condition can resemble malignant melanoma and histologically, it has a lentiginous pattern with variable degrees of atypia and an absence of dermal nests. These features may lead to an erroneous diagnosis of lentigo maligna melanoma or lentiginous melanoma. MATERIAL AND METHODS We reviewed 14 cases of atypical lentiginous nevus diagnosed at the dermatology department of Hospital General de Valencia in Valencia, Spain between December 2007 and March 2009. We studied the clinical and histopathologic features of the lesions after hematoxylin-eosin, Melan-A, and Ki-67 staining and compared our results to data reported in the literature. RESULTS Four (28%) of the 14 patients (7 men, 7 women) were under 50 years of age. Clinically, most of the lesions (8/14) resembled atypical nevi and they were all located on the back. Histologically, they all had irregular lentiginous epidermal hyperplasia, with a proliferation of individual melanocytes only in the basal layer of the epidermis and an absence of dermal nests. Focal upward migration of melanocytes into the epidermis was present in just 4 cases. All the lesions had cellular atypia, which was moderate in 85% of cases. The Ki-67 proliferation index was low (<5%) in all the lesions analyzed. CONCLUSIONS Atypical lentiginous nevi, which can be classified as atypical pigmented lesions with a lentiginous pattern, may clinically and histologically resemble melanoma. Our findings support earlier reports that both clinical and histologic findings may suggest a diagnosis of dysplastic nevus. All of the patients in our series are healthy and free of recurrence after 18 months or longer.

CARTA AL EDITORSíndrome del cabello anágeno suelto. Utilidad diagnóstica del tricogramaLoose anagen hair syndrome. Diagnostic use of the trichogram

dad clı́nica, fenotipos infantil tardı́a clásica y juvenil. Catepsina D (CTS): CLN10 forma congénita. Posteriormente se harı́a el estudio genético. La forma CLN3 (juvenil o enfermedad de Batten) tiene presentación tı́pica con ceguera progresiva, y como screening, podrı́an buscarse linfocitos vacuolados y luego realizar genética CLN3, más común 1kb deleción. Si las determinaciones anteriores fueran negativas y persiste alto grado de sospecha de CLN, serı́a obligatoria la ME y si fuera positiva hacer genética orientada según ME de las formas menos habituales: CLN5 (forma finlandesa), CLN6, CLN7 (forma turca), CLN8 (forma turca y epilepsia del norte con retardo mental) y CLN4 gen todavı́a no identificado (forma del adulto o enfermedad de Kufs). Queremos destacar las ventajas que a nuestro juicio aporta la biopsia de piel, que permite: el estudio de la actividad enzimática (PTT1, TPP1, CTSD) y ME en las CLN; el estudio de otras enfermedades lisosomales y mitocondriales; y almacenar fibroblastos, tejido vivo, para futuros estudios en caso de no llegar a un diagnóstico.

Síndrome del cabello anágeno suelto. Utilidad diagnóstica del tricograma

dad clı́nica, fenotipos infantil tardı́a clásica y juvenil. Catepsina D (CTS): CLN10 forma congénita. Posteriormente se harı́a el estudio genético. La forma CLN3 (juvenil o enfermedad de Batten) tiene presentación tı́pica con ceguera progresiva, y como screening, podrı́an buscarse linfocitos vacuolados y luego realizar genética CLN3, más común 1kb deleción. Si las determinaciones anteriores fueran negativas y persiste alto grado de sospecha de CLN, serı́a obligatoria la ME y si fuera positiva hacer genética orientada según ME de las formas menos habituales: CLN5 (forma finlandesa), CLN6, CLN7 (forma turca), CLN8 (forma turca y epilepsia del norte con retardo mental) y CLN4 gen todavı́a no identificado (forma del adulto o enfermedad de Kufs). Queremos destacar las ventajas que a nuestro juicio aporta la biopsia de piel, que permite: el estudio de la actividad enzimática (PTT1, TPP1, CTSD) y ME en las CLN; el estudio de otras enfermedades lisosomales y mitocondriales; y almacenar fibroblastos, tejido vivo, para futuros estudios en caso de no llegar a un diagnóstico.

Signo del collar de pelo asociado a aplasia cutis congénita del cuero cabelludo

Servicio de Dermatologia. Hospital General Universitario. Valencia. Espana. naunay y malformacion de ArnoldChiari es excepcional. Solo hemos encontrado un caso publicado en la literatura revisada (MEDLINE 1966-2007)7. La baja frecuencia de ambos procesos descarta en cierta medida que dicha asociacion sea de caracter casual, por lo que creemos importante que ante todo paciente con lesiones cutaneas de sindrome de Klippel-Trenaunay se investigue la presencia de clinica neurologica que haga sospechar una malformacion de Arnold-Chiari, debiendo plantearse la realizacion de una resonancia magnetica a todos estos pacientes.