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Sylvia Hsu

Publications and source records attributed to Sylvia Hsu.

At least 19 recordsLinked to original sources

Cutaneous Rosai-Dorfman disease.

Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a benign idiopathic histiocytic proliferative disorder that commonly involves the lymph nodes but secondarily may involve the skin. However, purely cutaneous disease without lymphadenopathy or internal organ involvement rarely may occur. We present case reports of three patients who presented with asymptomatic nonspecific enlarging skin nodules without evidence of lymphadenopathy or internal disease. Histopathologic examination of skin lesions in all patients showed proliferation of large histiocytes with phagocytosed inflammatory cells characteristic of Rosai-Dorfman disease. However, the diagnoses of dermatofibroma, other spindle cell neoplasm, infectious granulomatous process, and other xanthohistiocytic proliferations were also considered due to the presence of storiform spindle cells and foamy cells in the first case. One patient experienced regression during a course of oral steroids, while another patient cleared spontaneously. In the absence of massive lymphadenopathy characteristic of Rosai-Dorfman disease, the diagnosis of purely cutaneous Rosai-Dorfman disease may be complicated by the rarity, non-specific clinical appearance of skin lesions, and broad histopathological differential diagnosis of this disorder. A high index of suspicion of the clinician and pathologist is often required.

Adult↗

Disseminated sporotrichosis mimicking sarcoidosis.

A 40-year-old Caucasian man presented to the dermatology clinic at Baylor College of Medicine, Houston, Texas, in February 2003, for the evaluation of three nonhealing ulcers. The patient's past medical history was significant for hypothyroidism and pulmonary sarcoidosis, the diagnosis of which was made in June 2000. In March 2000, the patient had complained of cough and shortness of breath. A purified protein derivative (PPD) (Mantoux text) was negative. Computed tomography (CT) scans of the chest revealed diffuse hilar and mediastinal adenopathy and bilateral interstitial and alveolar infiltrates. Although consistent with sarcoidosis, these findings were insufficient to exclude other etiologies, including disseminated fungal infection. Cultures and stains of subsequent bronchoscopy specimens failed to reveal any organisms, and histopathologic evaluation of the specimens was nondiagnostic. Based on the imaging studies and the negative cultures, a diagnosis of sarcoidosis was made, and the patient was started on therapy with prednisone. Before coming to our clinic, the patient had been on several courses of prednisone. In May 2002, the patient had presented to a private dermatologist with a 1-year history of a nonhealing 2.4 cm x 2.0 cm ulcer on the left medial forearm. Two biopsies were reported as nondiagnostic. The patient's presentation was interpreted as most consistent with Mycobacterium marinum infection, and so he was empirically treated with minocycline. This treatment was continued for almost 3 months without improvement in the ulcer. A few months after the minocycline had been discontinued, the patient was treated empirically for 2 months with ciprofloxacin. This treatment was also unsuccessful in ameliorating the ulcer. In between the two courses of antibiotics, specimens from the lesion were sent for bacterial and fungal cultures, which revealed normal skin flora. In January 2003, the patient returned to his private dermatologist with three ulcerations. In addition to the nonhealing ulcer on his left forearm, which he had acquired several months earlier, he had also developed a 3.0 cm ulcer on his right arm and a 3.0 cm ulcer on his central back. The patient refused biopsies at this visit. Given the patient's previous diagnosis of pulmonary sarcoidosis, it was thought that the skin lesions might represent ulcerative cutaneous sarcoidosis. Pyoderma gangrenosum was also considered to be a likely diagnosis. Therefore, the patient was started on a course of oral prednisone, an effective therapy for both sarcoidosis and pyoderma gangrenosum. Despite 1 month of treatment with 60 mg/day of prednisone, the ulcers increased, and the patient was subsequently referred to our clinic. Physical examination at the time of presentation revealed steroid acne on the trunk and upper extremities and three non-tender ulcers with erythematous, undermined borders (Figs 1-3). On the left arm, there was an adjacent nodule which the patient attributed to a scar from a previously healed ulcer. Histologic examination of biopsy specimens from all three sites showed similar findings. The lesion contained diffuse, suppurative, granulomatous, inflammatory infiltrates with extensive central necrosis. The infiltrates were composed of histiocytes, multinucleated foreign-body-type giant cells, plasma cells, lymphocytes, neutrophils, and neutrophil fragments. No organisms were seen in the initial, routinely stained sections. However, periodic acid-Schiff (PAS) staining demonstrated small fungal spores (Fig. 4) morphologically consistent with sporotrichosis, within the cytoplasm of multinucleated histiocytic giant cells (Fig. 5). Additional stains for bacteria and acid-fast organisms were negative. Cultures of the biopsy specimens from all three sites grew Sporothrix schenckii. Further questioning of the patient failed to reveal an obvious source of the infection. The patient denied any history of traumatic skin inoculation and did not engage in gardening or other outdoor activities that are classically associated with sporotrichosis. The patient did admit to blackberry picking on detailed retrospective questioning. Once the diagnosis of sporotrichosis was made, the patient was given 200 mg/day of itraconazole. After 2 months, the patient's ulcers were almost completely healed. The patient's pulmonary complaints were also much improved.

Adult↗

Sustained efficacy and safety of infliximab in psoriasis: a retrospective study of 73 patients.

BACKGROUND: Infliximab inhibits T-cell activation by binding tumor necrosis factor-alpha (TNF-alpha). This medication is widely used in the US for treatment of psoriasis as an off-label indication. The durability of its effect is largely unknown. OBJECTIVE: To assess the proportion of patients still on infliximab 12 months after initiation of therapy for psoriasis. METHODS: Retrospective chart review analysis of 73 patients with psoriasis treated with infliximab for at least 12 months or those who experienced treatment failure in less than 12 months. The point where infusions were deemed to no longer be efficacious was determined by physician's global assessment (PGA). RESULTS: Of 73 patients who started infliximab at least 12 months prior to this chart review, 22 (30.1%) had discontinued treatment secondary to loss of efficacy. Thirty seven patients (50.7%) had no loss of efficacy and continued to receive infusions. Two patients (2.7%) discontinued due to loss of efficacy after greater than 12 months. Of the 22 patients who discontinued treatment due to loss of efficacy, 2 were on concomitant methotrexate (5-7.5 mg/wk) therapy. Of the 37 patients still receiving treatment with no loss of efficacy at 12 months, 3 patients were on concomitant methotrexate therapy. Five patients (6.8%) discontinued secondary to minor adverse events: sinus infection (1), acne (1), fever (1), arthralgia (1), and transient rash (1). Three patients (4.1%) discontinued due to major adverse events: reactivation of tuberculosis (1), breast cancer (1), and gastrointestinal bleeding (1). One patient discontinued infliximab secondary to concerns of possible lymphoma risk (though there were no signs of symptoms of disease on examination), and 3 patients discontinued due to insurance concerns. CONCLUSION: Infliximab treatment resulted in significant improvement in psoriasis, with 37 out of 73 patients (50.7%) experiencing no loss of efficacy. This longitudinal retrospective chart review demonstrates continued benefit of infliximab infusions in about half of patients after 1 year, though a notable percentage (30.1%) experienced loss of efficacy as determined by physician's global assessment (PGA) and a number of others discontinued due to adverse events or insurance difficulty.

Adolescent↗

Hereditary papulotranslucent acrokeratoderma: a case report and literature review.

Hereditary papulotranslucent acrokeratoderma is a rare autosomal-dominant syndrome of the hands and feet characterized by persistent, asymptomatic, yellowish to white papules and plaques associated with fine-textured scalp hair and an atopic diathesis. Histopathologically, focal hyperkeratosis, hypergranulosis, and acanthosis of the epidermis are seen. We present a case of hereditary papulotranslucent acrokeratoderma in a young adult woman. The literature of this unusual condition is reviewed, and its relationship to acquired papulotranslucent acrokeratoderma is discussed.

Adult↗