The recently published 2016 revision of the WHO classification of lymphoid neoplasms includes primary cutaneous acral CD8-positive T-cell lymphoma (PCATCL) as a provisional entity. This is a rare indolent lymphoma characterized by papules or nodules on the ear and a dermal infiltrate of CD8-positive T-lymphocytes with cytotoxic marker expression. A retrospective review of a single institutional experience with PCATCL identified 3 patients (mean age 54; range 49–62) with papules or nodules on the ear. Lesional biopsies demonstrated a dense diffuse dermal infiltrate of atypical lymphocytes with a Grenz zone in 2 cases and focal epidermotropism in 1 case. The atypical lymphocytes were predominantly CD3 and CD8 positive with expression of cytotoxic marker TIA1. Staging evaluation failed to reveal systemic disease. Two patients underwent local excision, and the third received local radiation therapy all with complete response and no disease recurrence at last follow-up 3 months (range 2–5 months). Our cases add to the existing limited literature on the clinical and histopathological features of PCATCL. We also performed an updated systematic literature view of the entity.
A 6-year-old girl presented with nightly fever, persistent joint pain of the knees, ankles, lower back, and hip. Her skin lesions were evanescent salmon-colored patches along with persistent pruritic light to dark pink papules and plaques on her face, postauricular scalp, trunk, thigh, and bilateral upper extremities. Skin biopsy supported the diagnosis of fixed papules and plaques of systemic juvenile idiopathic arthritis (sJIA).We report this case to highlight diagnostic features of this exceedingly rare cutaneous presentation of sJIA presenting with typical cutaneous salmon-colored evanescent eruptions.
We report a patient with severe recalcitrant erythromelalgia who had a partial and prolonged response to 3 weeks of transcranial magnetic stimulation (TMS), a novel management modality for this painful syndrome.A 24-year-old patient presented with an 8-year history of continuous severe, unrelenting, recalcitrant erythromelalgia (i.e., swelling, red, hot, and painful acral areas) affecting her feet, hands, and face. She presented with intermittent episodes that were mostly triggered by heat and exercise. Additional testing, including electromyography, thermoregulatory sweat testing, and autonomic reflex screen supported the diagnosis of primary (idiopathic) erythromelalgia with underlying small fiber neuropathy. Other differential diagnoses such as peripheral vascular disease and Raynaud's phenomenon were ruled out because of noncorrelating clinical course. The erythromelalgia impacted greatly on her daily functioning: Every activity revolved around her attempt to cool her feet, which only provided minimal improvement, and it was painful for her to walk more than 15 yards. The patient had to miss days of school and work because of the unbearable pain, which had been recalcitrant to multiple topical and systemic treatments. She rated her average pain as 7 of 10 in severity when self-rated on a scale from 0 to 10, with 10 being the worst imaginable pain.A 3-week trial was undertaken of TMS applied to the right dorsolateral prefrontal cortex. The patient underwent 15 sessions of 1-Hz TMS-5 treatments per week over 3 weeks-using 1,600 stimulations per session at 110% of motor threshold.At the time of the treatment, she was not taking concomitant other treatments. She tolerated the TMS well and had only minor headaches during treatment. Her baseline pain rating was 7 of 10 and reduced to 6.3 at the end of week 1, to 5.2 at the end of week 2, and to 5 at the end of week 3. During follow-up, her pain ratings were 4 at months 1 and 2 and further reduced to 3 by the 3month follow-up assessment. Thus, she continued to improve in her pain ratings even 3 months after the active treatment. The area of erythema was not reduced, however.Erythromelalgia is the clinical syndrome of red, hot feet and hands, and occasionally face, that can be intensely painful. The pain can be recalcitrant to treatment.TMS is a novel noninvasive treatment that has been shown to have an antidepressant effect in randomized controlled trials 1,2 for depression and is also being studied for a potential role in the treatment of chronic pain such as fibromyalgia pain. 3,4 During TMS, a magnetic field is induced by the generation of an electrical current in a coil, which is placed against the scalp and applied specifically to the target area of the dorsolateral prefrontal cortex. The magnetic field promotes stimulation of the brain areas that modulate mood control and depression 2 and suppress pain. The modality has been found to successfully decrease depression symptoms and improve mood. 2,5 Our patient has been reported previously in a separate publicati...
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