A case of Schnitzler syndrome with unusual immunoglobulin A gammopathy exacerbated by COVID-19 infection.

Schnitzler syndrome (SchS) is a rare autoinflammatory disease characterized by chronic urticarial rash and monoclonal immunoglobulin M (IgM) or IgG gammopathy. Viruses, including COVID-19, activate the innate immune system, therefore SchS, in which the innate immune system is improperly activated, is hypothesized to be exacerbated by viral infection. However, there were no reported SchS cases exacerbated by any viral infection. Here, we report a SchS case with an unusual IgA gammopathy manifested and exacerbated by COVID-19 infection. This report advocates the need for recognizing unusual cases of SchS with monoclonal IgA, and following up on paraprotein like IgA even when it is initially undetectable in cases with SchS symptoms. We also hypothesize that existing autoinflammatory diseases may be exacerbated by COVID-19 infection in the case of a combination of these diseases.

Exploratory study on quantitative assessment of skin hardness in patients with systemic sclerosis using SOFTGRAM.

OBJECTIVES: There is a lack of quantitative and objective methods for measuring skin hardness. This study aimed to verify whether SOFTGRAM, a device that can measure elastic modulus using the Hertz elastic contact theory, could be used to evaluate skin hardness in systemic sclerosis (SSc). METHODS: Skin score according to the modified Rodnan total skin thickness score and elastic modulus of the skin using SOFTGRAM were measured for 20 patients with SSc and 20 healthy controls on 8 parts of the body, both of the cheeks, forearms, fingers, and hands. Five observers shared to measure skin score 320 times (40 participants × 8 parts). Elastic modulus was measured 1600 times (40 participants × 8 parts × 5 times each). As an additional examination to compare differences between observers, the skin score of another healthy control was measured 40 times (5 observers × 8 parts). Elastic modulus was measured 200 times (5 observers × 8 parts × 5 times each). RESULTS: There was a significant correlation between elastic modulus and skin score (correlation coefficient=0.67, p<0.001) and a significant difference in elastic modulus (8 parts: healthy controls vs. limited cutaneous SSc vs. diffuse cutaneous SSc: 22.6±15.7 vs. 32.0±27.7 vs. 44.8±39.8, p<0.001). Intraobserver reliabilities were sufficient in 6 out of 7 observers; however, interobserver was less satisfactory. CONCLUSIONS: This study showed the practicality of SOFTGRAM as an accurate measurement method of skin hardness but also revealed points to be improved. More studies are needed to find an accurate measurement method of skin hardness.

Mapping biopsy for Buruli ulcer self-medicated with occlusive dressing.

Buruli ulcer is the third most common mycobacterial infection next to tuberculosis and leprosy caused by Mycobacterium ulcerans. Although it affects the skin, subcutaneous tissues, muscles and sometimes bones, there is no reliable evidence to determine the extent of debridement. We present here a case of Buruli ulcer treated successfully with a preoperative mapping biopsy procedure, which had been self-medicated with occlusive dressing. Because Buruli ulcer is accompanied by subtle pain, patients and clinicians tend to initially treat the ulcer with occlusive dressing therapy, which leads to the misdiagnosis of Buruli ulcer as a common bacterial infection only judging from bacterial culture of the surface of the ulcer. We propose the efficacy of mapping biopsy for treating Buruli ulcer.