Jordi Mollet

Clinical patterns of cutaneous nontuberculous mycobacterial infections.

Background  Cutaneous nontuberculous mycobacterial infections result from external inoculation, spread of a deeper infection, or haematogenous spread of a disseminated infection. There are two species‐specific infections (fish‐tank or swimming‐pool granuloma, due to Mycobacterium marinum, and Buruli ulcer, caused by M. ulcerans). Most infections, however, produce a nonspecific clinical picture.

Eruptive juvenile xanthogranuloma associated with relapsing acute lymphoblastic leukemia.

Abstract:  Juvenile xanthogranuloma is a benign, self‐healing disorder with characteristic lesions mainly involving the skin. Although most patients with juvenile xanthogranuloma have only cutaneous symptoms, recent articles have documented extracutaneous manifestations: systemic involvement of many organs has been reported and there is a known association between juvenile xanthogranuloma and childhood leukemia, most commonly juvenile chronic myelogenous leukemia. This case provides further corroboration, that in rare instances, juvenile xanthogranuloma may be associated with hematologic malignancies.

Pitiriasis rubra pilaris aguda postinfecciosa: una dermatosis mediada por superantígenos

Resumen La pitiriasis rubra pilaris (PRP) aguda postinfecciosa es una variante de la forma juvenil de PRP (tipo III de Griffiths) caracterizada por la ausencia de antecedentes familiares, curso agudo relacionado con un episodio febril previo y buen pronostico. Clinicamente puede simular otras enfermedades mediadas por superantigenos, como los exantemas escarlatiniformes o el sindrome de la escaldadura estafilococica; sin embargo, su histologia y tratamiento son distintos. Presentamos 4 casos de PRP aguda postinfecciosa que ilustran las caracteristicas clinicas de este proceso infrecuente y revisamos los posibles mecanismos fisiopatogenicos subyacentes.

Tufted Angioma Associated with Kasabach-Merritt Phenomenon: A Therapeutic Challenge

Fig. 1. (A) Tufted angioma involving the chin in a one-month-old child. (B) The patient at 2 months of age showing a subtle increase in tumour size and decrease in platelet count

Cervico‐Thoracic Lipoma Associated with Occult Syringohydromyelia

Abstract:  Cutaneous lesions in the midline may be hallmarks of an occult spinal dysraphism. They rarely may occur in the cervical and thoracic regions. We report a 9‐year‐old girl with a cervico‐thoracic lipoma associated with an underlying syringohydromyelia. Early detection of potentially complicated conditions can prevent the occurrence of irreversible neurologic damage.

Síndrome esclerodermiforme por paclitaxel

Resumen —Los sindromes esclerodermiformes se han relacionado con una larga lista de medicamentos, a la que se han anadido en los ultimos anos los taxanos. Se presenta el caso de una mujer con una neoplasia de mama que recibio tratamiento ciclico con paclitaxel. Tras 17 sesiones presento edema y eritrodisestesia en ambos pies que, a pesar del tratamiento sintomatico y de reducir la dosis de paclitaxel, progresaron hacia un endurecimiento cutaneo en estas localizaciones. Diez meses despues de suspender el taxano las lesiones cutaneas se mantenian estables y no existian otras manifestaciones de esclerosis sistemica. Los taxanos pueden producir un sindrome esclerodermiforme que suele afectar a las extremidades inferiores y esta precedido por edema y/o eritrodisestesia; no se acompana de otras manifestaciones de esclerosis sistemica y tiende a estabilizarse o a involucionar al suspender el tratamiento.

Acute Postinfectious Pityriasis Rubra Pilaris: A Superantigenmediated Dermatosis

Acute postinfectious pityriasis rubra pilaris (PRP) is a variant of juvenile PRP (Griffiths type III) characterized by no family history, an acute course associated with a prior fever, and good prognosis. Clinical features may resemble other superantigen-mediated diseases, such as scarlatiniform rash or staphylococcal scalded skin syndrome, but its histology and treatment are different. We present 4 cases of acute postinfectious PRP that illustrate the clinical features of this uncommon disease and we review possible underlying pathogenic mechanisms.

Utility of Ultrasound in a Rapidly Growing Cutaneous Nodule in an Infant

A healthy 8-year-old girl consulted for a rapidly growing nodule on her left arm, which had appeared after a contusion 6 weeks earlier. Physical examination results revealed an indurated nodule, 2.5 cm in diameter, violaceous, with a pseudoblisterous surface (Figure 1; available at www.jpeds.com). Ultrasonography showed a well-defined subcutaneous nodule with central hyperechoic points, peripheral hypoechoic rim, and a posterior acoustic shadowing; echo-Doppler scanning revealed increased peripheral vascularity. All of this is compatible with pilomatrixoma (Figure 2). Surgical excision was performed and histopathologic examination showed a well-demarcated subcutaneous tumor, with a peripheral proliferation of basaloid cells, central eosinophilic shadow cells, and areas of calcification. The overlying dermis showed a lax tissue with dilated lymphatic vessels and without elastic fibers, consistent with anetodermic pilomatrixoma (Figure 3; available at www.jpeds.com). Pilomatrixoma, a benign appendage tumor derived from the matrix cells of the hair follicle, appears mainly in children or young adults and is normally located in the head, neck, and upper extremities. It is usually solitary, asymptomatic, with a slow-growing pattern and with a characteristic histology. Ultrasonography is an accessible, quick, and noninvasive imaging tool; it is gaining an important role in the diagnosis of cutaneous lesions, especially in pediatric patients. It has been seen that the combination of central internal heterogeneous echo texture by scattered pattern calcifications,peripheral hypoechoic rim,and subsequentposterior shadowing could be discriminative ultrasonographic criteria for differentiating pilomatrixoma from other subcutaneous tumours. Anetodermic pilomatrixoma is rare. Clinically, it is characterized by atrophic, wrinkled, pseudoblisterous, or bulla-like changes of the skin overlying the tumor. Preceding mechanical trauma at the site of occurrence of the tumor has been reported, as is the case in our patient. It would lead to the disruption of the dermal integrity and cutaneous microcirculation responsible of the clinical appearance of the tumor. ■