Palisaded neutrophilic and granulomatous dermatitis in association with subcutaneous nodular and systemic sarcoidosis.

Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare entity that has been clearly defined neither clinically nor histopathologically. PNGD has been associated with some immune-mediated disorders such as rheumatoid arthritis, systemic lupus erythematosus, systemic vasculitis, Behçet's disease, as well as with lymphoproliferative conditions, bacterial endocarditis, sarcoidosis, and various drugs. We present a 44-year-old Caucasian woman with roundish erythematous-livid plaque and erythematous papules on the left calf that were present for three months. Histopathology of plaque lesion showed palisading neutrophilic and granulomatous dermatitis. Subsequently, she developed a firm and tender nodule on the right calf. Histopathology of the nodule showed typical naked sarcoid granulomas in the dermis and subcutis. Additionally, the patient developed non-tender subcutaneous nodules on the cheeks, submandibular region and left breast with normal overlying skin, which were histopathologically diagnosed as sarcoid granuloma. Red eyes and lower visual acuity on the right eye were diagnosed as anterior uveitis. Therefore, systemic sarcoidosis was established. This is a case of PNGD described in an adult patient with sarcoidosis with cutaneous, breast, eye and lung involvement established by clinical, radiographic, laboratory, and histopathologic criteria.

Diffuse sclerosing variant of thyroid carcinoma presenting as Hashimoto thyroiditis: a case report.

The aim of report is to present a case of a rare diffuse sclerosing variant of a papillary thyroid carcinoma. A 15-year old girl referred for ultrasound examination because of painless thyroid swelling lasting 10 days before. An ultrasound of the neck showed diffusely changed thyroid parenchyma, without nodes, looking as lymphocytic thyroiditis Hashimoto at first, but with snow-storm appearance, predominantly in the right lobe. Positive thyroid peroxidase antibodies (TPO-AT) also suggested Hashimoto thyroiditis. Repeated US-FNAB (fine needle-aspiration biopsy) of the right lobe revealed diffuse sclerosing variant of papillary thyroid carcinoma and patient underwent total thyreoidectomy. Patohistologic finding confirmed diffuse sclerosing variant of a papillary thyroid carcinoma in the both thyroid lobes and several metastatic lymph nodes. Two months later patient recived radioablative therapy with 3700 MBq (100 mCi) of 1-131 followed by levothyroxine replacement. At the moment, patient is without evidence of local or distant metastases and next regular control is scheduled in 6 months. In conclusion, a diffuse sclerosing variant is rare form of papillary thyroid carcinoma that echographically looks similar to Hashimoto thyroiditis and sometimes could be easily overlooked.

Neurofibromatosis type 1 associated with dysplastic nevus syndrome.

Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder that primarily affects the development and growth of neural cell tissues. It causes tumors to grow on nerves and produces other abnormalities such as skin changes and bone deformities. Dysplastic nevus syndrome (DNS) represents multiple atypical nevi associated with polygenetic inheritance pattern and may rarely occur together with NF-1. DNS type A is a marker of increased melanoma risk, while melanoma has been rarely reported in patients with NF-1. We describe a case of NF-1 type A with DNS presenting with multiple neural tumors, cafe-au-lait spots, hamartomas in globus pallidus and pigmented melanocytic iris hamartomas (Lisch nodules). The importance of close follow up of nevi in such patients with NF-1 and DNS for the development of melanoma as well as other NF-1 associated skin disorders and with multidisciplinary approach to other associated diseases is highlighted.