Exantema intertriginosoflexural y simétrico (SDRIFE) por piroxicam: a propósito de un caso clínico / Symmetrical drug related intertriginous and flexural exanthema secondary to piroxicam: a case report

Resumen El exantema intertriginoso y flexural simétrico por fármacos (SDRIFE) es una toxicodermia que se presenta con exantema limitado a pliegues, sobretodo en región glútea y genital, y representa una reacción a la exposición sistémica a un medicamento sin sensibilización previa. Presentamos el caso de una paciente mujer adulta previamente sana, que consulta por este tipo de reacción medicamentosa asociada al uso de piroxicam intramuscular, manejada de forma exitosa con antihistamínicos, esteroides tópicos y sistémicos.

Abstract Symmetrical drug related intertriginous and flexural exanthema is a cutaneous drug eruption that is characterized by exanthema limited to creases, buttocks and the upper inner thighs. It represents a reaction to systemic exposure to a drug, without previous sensitization. We present the case of a woman, previously healthy, who consulted due to this drug reaction after being injected with piroxicam, who was successfully treated with antihistamines, topical and systemic corticosteroids.

Papulonodular mucinosis in the primary onset of juvenile systemic lupus erythematosus: An unusual association / Mucinosis papulonodular en el inicio de lupus eritematoso sistêmico juvenil: asociación inusual

ABSTRACT Cutaneous mucinosis is a group of conditions characterized by the abnormal deposition of mucin in the skin. They can be primary, which in turn can be inflammatory-degenerative, and hamartomatous-neoplastic; or secondary. Papulonodular mucinosis is part of the group of dermal inflammatory-degenerative primary mucinosis. Its association with autoimmune connective tissue diseases has been described, especially with systemic lupus erythematosus, but it is considered an unusual manifestation of this disease. The clinical case is presented of an 11 year-old girl who, at the onset of systemic lupus erythematosus, presented with skin lesions for which the histopathological diagnosis corresponded to mucinosis.

RESUMEN Las mucinosis cutáneas son un grupo de condiciones caracterizadas por el depósito anormal de mucina en la piel. Pueden ser primarias, que a su vez pueden ser inflamatorias-degenerativas (dérmicas o foliculares) y hamartomatosas-neoplásicas; o secundarias. La mucinosis papulonodular forma parte de las mucinosis primarias inflamatorias-degenerativas dérmicas. Se ha descrito su asociación con enfermedades autoinmunes del tejido conectivo, especialmente con el lupus eritematoso sistémico, pero se considera una manifestación inusual de esta enfermedad. Se presenta el caso clínico de una niña de 11 años, quien al inicio del lupus eritematoso sistémico presentaba lesiones en la piel cuyo diagnóstico histopatológico correspondió a mucinosis.

Early-Onset Invasive Infection Due to Corynespora cassiicola Associated with Compound Heterozygous CARD9 Mutations in a Colombian Patient.

PURPOSE: CARD9 deficiency is an inborn error of immunity that predisposes otherwise healthy humans to mucocutaneous and invasive fungal infections, mostly caused by Candida, but also by dermatophytes, Aspergillus, and other fungi. Phaeohyphomycosis are an emerging group of fungal infections caused by dematiaceous fungi (phaeohyphomycetes) and are being increasingly identified in patients with CARD9 deficiency. The Corynespora genus belongs to phaeohyphomycetes and only one adult patient with CARD9 deficiency has been reported to suffer from invasive disease caused by C. cassiicola. We identified a Colombian child with an early-onset, deep, and destructive mucocutaneous infection due to C. cassiicola and we searched for mutations in CARD9. METHODS: We reviewed the medical records and immunological findings in the patient. Microbiologic tests and biopsies were performed. Whole-exome sequencing (WES) was made and Sanger sequencing was used to confirm the CARD9 mutations in the patient and her family. Finally, CARD9 protein expression was evaluated in peripheral blood mononuclear cells (PBMC) by western blotting. RESULTS: The patient was affected by a large, indurated, foul-smelling, and verrucous ulcerated lesion on the left side of the face with extensive necrosis and crusting, due to a C. cassiicola infectious disease. WES led to the identification of compound heterozygous mutations in the patient consisting of the previously reported p.Q289* nonsense (c.865C > T, exon 6) mutation, and a novel deletion (c.23_29del; p.Asp8Alafs10*) leading to a frameshift and a premature stop codon in exon 2. CARD9 protein expression was absent in peripheral blood mononuclear cells from the patient. CONCLUSION: We describe here compound heterozygous loss-of-expression mutations in CARD9 leading to severe deep and destructive mucocutaneous phaeohyphomycosis due to C. cassiicola in a Colombian child.

Feohifomicosis, una infección fúngica oportunista emergente / Phaeohyphomycosis, an emerging opportunistic fungal infection

Las feohifomicosis comprenden un grupo de infecciones causadas por hongos pigmentados, negros o dematiáceos. En las últimas dos décadas se ha incrementado la frecuencia de reportes y la diversidad de los agentes etiológicos implicados, especialmente en los individuos inmunosuprimidos. Los principales géneros involucrados incluyen Alternaria, Bipolaris, Cladophialophora y Exophiala. Estos hongos típicamente se encuentran en el suelo y son introducidos al cuerpo a través de la inhalación o el trauma. El espectro de las enfermedades asociadas también se ha ampliado e incluye infecciones cutáneas superficiales y profundas, enfermedad alérgica, neumonía, abscesos cerebrales e infección diseminada. El diagnóstico de laboratorio está basado en las características morfológicas de los agentes según lo observado en el examen microscópico directo y la histopatología. El tratamiento es a menudo difícil y depende del síndrome clínico. No hay terapias estandarizadas, pero voriconazol, posaconazol e itraconazol han demostrado la actividad in vitro más consistente contra este grupo de hongos. La rareza de estas micosis justifica describir las características clínicas, epidemiológicas y diagnósticas para ayudar a un reconocimiento inmediato y un tratamiento oportuno

Phaeohyphomycosis comprises a group of infections caused by black pigmented or dematiaceous fungi. In the last two decades the frequency of reporting and diversity of etiologic agents involved have increased, especially in immunosuppressed individuals. The main genera involved include Alternaria, Bipolaris, Cladophialophora and Exophiala. These fungi are typically found in the soil and introduced through inhalation or trauma. The spectrum of associated diseases also has broadened and includes superficial and deep cutaneous infections, allergic disease, pneumonia, brain abscess and disseminated infection. The laboratory diagnosis is based on the morphological characteristics of the agents as observed by direct microscopic examination and histopathology. Treatment is often challenging and depends uponthe clinical syndrome. There are no standardized therapies but voriconazole, posaconazole and itraconazole demonstrate the most consistent in vitro activity against this group of fungi. The rarity of this mycosis justifies describe the clinical, epidemiological and diagnostic characteristics to aid in its immediate recognition and early treatment