Vulvar pseudoverrucous papules and nodules secondary to a urethral--vaginal fistula.

Irritant contact dermatitis is a common cause of genital eruptions representing at least one-fifth of presenting anogenital symptoms. A spectrum of inflammatory reactions have been identified, some of them with features severely mimicking more serious dermatoses, which may lead unnecessary workup and treatments. We report a case of a 10-year-old girl who presented at birth with cloacal atresia involving the rectum and the urethra. A diverting colostomy and a urethral-vaginal fistula were created to correct the deformity. Physical examination reveals numerous shiny, white-gray, pseudoverrucous papules and nodules coalescing into plaques over the vulva and its surrounding skin. Histological examination showed psoriasiform epidermal hyperplasia with a marked reactive acanthosis and altered cornification with parakeratosis, hypogranulosis, and pale keratinocytes in the upper reaches of the epidermis. The lesions regress when the irritating factor was removed. This case represents a peculiar form of presentation of perianal pseudoverrucous papules and nodules, usually secondary to urinary incontinence or encopresis (inability to control the elimination of stool) after surgery for Hirschsprung disease. Because similar findings have not been previously observed in patients with urethral-vaginal fistula, we attempt to extend the spectrum of presentations for a better knowledge of this condition.

Alpha-1-antitrypsin deficiency panniculitis.

Alpha-1-antitrypsin deficiency is a congenital error of metabolism linked to pulmonary (emphysema) and liver (cirrhosis) disease. Since 1972, panniculitis has been associated with this deficiency, initially related to Weber-Christian syndrome and finally as a differentiated entity. Clinical manifestations typically consist of wide nodular lesions on the trunk and proximal extremities that evolve to ulceration and drainage. Histopathologically it presents as a mixed septal-lobular panniculitis pattern with some typical findings referred. Differential diagnosis from other types of panniculitis and neutrophilic dermatosis must be established. Different treatments, including tetracyclines, dapsone, and alpha-1-antitrypsin repositioning, have shown variable efficacy in controlling this disease.

Superficial thrombophlebitis.

Superficial thrombophlebitis (STP) is a common disease usually characterized by an auto-resolving vasculitis of medium-sized veins of the upper subcutis or deep dermis that clinically manifests as a tender or painful palpable cord-like structure. It usually occurs in the setting of varicous veins, or hypercoagulable states, and may be the alarm signal for an underlining silent cancer. STP mainly involves the legs, but special locations, including the anterior chest wall or the penis, characterize specific clinical forms (Mondor's disease). The clinical signs and symptoms usually allow an easy diagnosis, but complementary techniques and biopsy are sometimes required. The main histopathologic differential diagnosis of STP is cutaneous polyarteritis nodosa.

[Treatment of mycosis fungoides with PUVA and bexarotene]. / Tratamiento de micosis fungoide con PUVA más bexaroteno.

INTRODUCTION: The combined use of bexarotene and PUVA is a treatment that is currently being investigated. In this paper, six patients treated with this combination are presented. OBJECTIVES: To assess the efficacy and safety of treatment with PUVA + bexarotene in patients with mycosis fungoides (MF). Patients, material and methods. Six patients diagnosed with MF in different stages, who received three sessions of PUVA treatment a week (initially 2.35 J/cm 2, with progressive increases to a maximum of 23.5 J/cm 2) + bexarotene (initial dose 300 mg/m 2/day, decreasing to 200, 150 or 75 mg/m 2 if signs of toxicity appeared). All received atorvastatin. RESULTS: Stage at the start of treatment: two patients IIb, one patient Ib with B2 blood involvement, two patients Ib, one patient Ia. Five of the six patients responded to the treatment (three full remissions [FR], two partial remissions [PR]). One patient did not respond. In those in whom FR was achieved, the time required for the response was 10, 20 and 24 weeks. All presented with hypertriglyceridemia (maximum 1194 mg/ dL). It was necessary to administer thyroid hormone supplements to four of the patients. Two of them had alterations in the hepatic biochemistry values, and two others presented with alterations in the muscle profile analysis. CONCLUSION: The combination of PUVA and bexarotene is a safe and effective treatment for MF. It will be necessary to await the results of the clinical trials currently underway to see if their combined use is better than treatment with PUVA alone. The response rate (RR) was 86 % (three FR and two PR out of six patients).

Tratamiento de micosis fungoide con PUVA más bexaroteno / Treatment of mycosis fungoides with PUVA and bexarotene

Introducción. La utilización conjunta de bexaroteno y psoraleno y radiación ultravioleta A (PUVA) en la actualidad es un tratamiento en investigación. En el presente trabajo se presentan 6 pacientes tratados con esta combinación. Objetivos. Valorar eficacia y seguridad del tratamiento con PUVA más bexaroteno en pacientes con micosis fungoide. Pacientes, material y métodos. Seis pacientes diagnosticados de micosis fungoide en distintos estadios que han recibido PUVA, tres sesiones semanales (inicio 2,35 J/cm 2, con aumentos progresivos hasta llegar a un máximo de 23,5 J/cm 2) más bexaroteno (dosis inicial, 300 mg/m 2/día, disminuyendo a 200, 150 o 75 mg/m 2 si aparecía toxicidad). Todos los pacientes recibieron atorvastatina. Resultados. Al inicio de tratamiento, 2 pacientes se encontraban en estadio IIb, un paciente en Ib con afectación hemática B2, 2 pacientes en Ib y un paciente en estadio Ia. Cinco de los 6 pacientes respondieron al tratamiento (tres remisiones completas [RC], dos remisiones parciales [RP]). Un paciente no respondió. De los que obtuvieron RC, el tiempo hasta la respuesta fue de 10, 20 y 24 semanas, respectivamente. Todos los pacientes presentaron hipertrigliceridemia (máximo de 1.194 mg/dl). En cuatro de los pacientes fue necesario administrar suplementos de hormona tiroidea. Dos de ellos tuvieron alteraciones de la bioquímica hepática y dos más presentaron alteraciones analíticas del perfil muscular. Conclusión. La combinación de PUVA y bexaroteno es un tratamiento eficaz y seguro para la micosis fungoide. Habrá que esperar a los resultados de los ensayos clínicos en curso para ver si es mejor su uso combinado que el tratamiento con PUVA sola. El índice de respuesta fue del 86 % (3 RC y 2 RP de 6 pacientes)

Introduction. The combined use of bexarotene and PUVA is a treatment that is currently being investigated. In this paper, six patients treated with this combination are presented. Objectives. To assess the efficacy and safety of treatment with PUVA + bexarotene in patients with mycosis fungoides (MF). Patients, material and methods. Six patients diagnosed with MF in different stages, who received three sessions of PUVA treatment a week (initially 2.35 J/cm 2, with progressive increases to a maximum of 23.5 J/cm 2) + bexarotene (initial dose 300 mg/m 2/day, decreasing to 200, 150 or 75 mg/m 2 if signs of toxicity appeared). All received atorvastatin. Results. Stage at the start of treatment: two patients IIb, one patient Ib with B2 blood involvement, two patients Ib, one patient Ia. Five of the six patients responded to the treatment (three full remissions [FR], two partial remissions [PR]). One patient did not respond. In those in whom FR was achieved, the time required for the response was 10, 20 and 24 weeks. All presented with hypertriglyceridemia (maximum 1194 mg/ dL). It was necessary to administer thyroid hormone supplements to four of the patients. Two of them had alterations in the hepatic biochemistry values, and two others presented with alterations in the muscle profile analysis. Conclusion. The combination of PUVA and bexarotene is a safe and effective treatment for MF. It will be necessary to await the results of the clinical trials currently underway to see if their combined use is better than treatment with PUVA alone. The response rate (RR) was 86 % (three FR and two PR out of six patients)