Patients with neurofibromatosis 1 (NF1) have various clinical symptoms. In particular, diffuse plexiform NF (dNF) negatively influences the quality of life in NF1 patients due to pain, disfigurement and other problems. An epidemiological study showed that dNF accounted for approximately 30% of NF1 patients with skin lesions. This tumor was found mainly in the trunk. Furthermore, a survey of NF1 patients who underwent surgical treatment with cNF or dNF showed that there was no significant difference between the two groups in terms of medical costs related to surgical procedures. DNF is a special tumor, its clinical characteristics need to be well recognized and appropriate treatment should be given.
A continuous glucose monitoring system, FreeStyle Libre, that measures interstitial glucose level, has been introduced in Japan since 2017. Two cases of allergic contact dermatitis related to the sensor devise has been reported to date. Here we report three cases that developed erythema where the sensor was applied. Three patients exhibited erythema when a patch test was performed with the adhesive part of the sensor, and were diagnosed with allergic contact dermatitis. Two out of three were further tested with the components of adhesive tape, and isobornyl acrylate was identified as a culprit allergen. Out of 15 diabetic patients who used FreeStyle Libre in our hospital, three patients were diagnosed by patch test and two additional patients also had erythema and pruritus, although the latter were not diagnosed by the patch test. Due to the increasing use of the devise, the allergen should be considered in future diagnosis.
Case 1: A 35-year-old man who had been treated for five years with prednisolone and cyclosporin for eosinophilia developed purpura and edema suddenly in his legs without any trigger. Case 2: A 36-year-old woman developed purpura suddenly in her left leg and left ankle joint, also without a trigger. In both cases, a prolongation of activated partial thromboplastin time (APTT), a decrease in Factor VIII (FVIII) activity, and an elevated activity of FVIII inhibitors were contributing factors. The patients were diagnosed with acquired hemophilia A and treated with systemic prednisolone. After treatment, the titer of FVIII inhibitors became negative. Because acquired hemophilia A can be lethal due to unexpected bleeding, it is important to consider this disease when treating cases of extensive, sudden eccymosis without trigger.
Lichen planus pigmentosus (LPP) is an uncommon variant of lichen planus characterized by brown to gray-brown macules preferentially arise in sun-exposed areas. The distribution is usually bilateral and symmetric, although a unilateral blaschkoid variant has also been described. We report a 53-year old Peruvian woman with gray-brown macules distributed along the lines of Blaschko on the face. Histological examination showed a lichenoid dermatitis with prominent dermal melanophages, leading to the diagnosis of lichen planus pigmentosus. The lesions persisted after topical corticosteroid therapy, but almost disappeared with subsequent Q-switched alexandrite laser treatment. We review the literature published since 2004 on LPP and summarized 664 cases, including 13 blaschkoid variants and 26 laser-treated patients. LPP should always be considered when we encounter a patient with pigmented macules.