RESUMEN
Erythromelalgia is a rare neurovascular pain condition characterized by erythematous, warm, and painful extremities. Symptoms are exacerbated by heat and relieved by cooling. Treatment is challenging and focuses on symptom control with various medications and therapies targeted toward eliminating destructive cooling behaviors. This pediatric case was notable because the patient's pain dramatically improved after a short-term, low-dose ketamine infusion, allowing her to finally wean off detrimental cooling practices of her extremities. Intravenous ketamine has rarely been described as an adjunctive analgesic strategy for erythromelalgia.
Asunto(s)
Eritromelalgia , Ketamina , Analgésicos/uso terapéutico , Niño , Eritromelalgia/complicaciones , Eritromelalgia/diagnóstico , Eritromelalgia/tratamiento farmacológico , Femenino , Humanos , Ketamina/uso terapéutico , Dolor/tratamiento farmacológico , Dolor/etiología , Manejo del DolorRESUMEN
A 70-year-old white housewife presented with a rare presentation of coexistent generalized morphea and lichen sclerosus et atrophicus with unusual clinical aspects. The patient had disseminated erythematous lesions that evolved into indurated large plaques. Hypopigmentation and hyperpigmentation developed later, in addition to ivory, white, and shiny plaques on the trunk (Figure 1). The skin of the arms and legs showed a wavy contour (Figure 2). Various areas were markedly sclerotic and some had edematous papules (Figure 3). Multiple indurated, ivory, white, shiny, large, and hypochromic plaques were seen on the trunk Laboratory examinations showed increased immunoglobulin A and antinuclear antibodies 1:200 speckled. Scl-70, anti-centromere, anti-ribonucleoprotein, and anti-DNA tests were negative. Esophageal manometry and abdominal and pelvic ultrasound findings were all normal. Complete blood cell count, blood profile, and urinalysis were also within normal limits. Skin biopsy of an arm lesion showed an atrophic epidermis with orthokeratotic hyperkeratosis and follicular plugging. A broad area of homogenization and edema was seen in the papillary dermis with dilated capillaries and a perivascular lymphocytic infiltration. There was also collagen sclerosis throughout the reticular dermis, and thickened, homogenized collagen bundles replaced the subcutaneous fat. Vessel walls showed proliferated intima with mucin deposition and sclerosis, as well as multiplied elastic layers. There was edema of the papillary dermis in some areas and incipient deposition of calcium (Figures 4-7).
Asunto(s)
Liquen Escleroso y Atrófico/complicaciones , Esclerodermia Localizada/complicaciones , Anciano , Biopsia , Femenino , Humanos , Hiperpigmentación/etiología , Hipopigmentación/etiología , Liquen Escleroso y Atrófico/diagnóstico , Liquen Escleroso y Atrófico/patología , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/patologíaRESUMEN
Although the majority of hemangiomas of infancy do not require treatment, a subset of patients have complications such as ulceration, functional impairment or severe disfigurement that necessitate intervention. It is often difficult to predict which hemangiomas will lead to permanent disfigurement and medical complications. We present three patients with segmental facial hemangiomas involving the nose which resulted in nasal cartilage destruction. All three patients had a linear gray atrophic crease in the inferior columella visualized on clinical examination prior to the onset of nasal collapse. We propose that this ''nasal crease sign'' in infants with segmental facial hemangioma involving the nose and philtrum may be a premonitory clinical finding of impending nasal cartilage destruction. Dermatologists should be aware of this clinical sign which may help identify patients at high risk of nasal destruction. Unfortunately, aggressive medical intervention may not always prevent devastating disfigurement.