Benign glandular schwannomas are rare and should be distinguished from malignant peripheral nerve sheath tumors with similar divergent tissue differentiation. The authors present a benign glandular schwannoma with ancient change that developed in the subcutis of a 46-year-old man's posterior calf. He lacked stigmata of neurofibromatosis type 1 (NF1). The glandular elements stained positively for epithelial membrane antigen and pancytokeratin. The spindled cells stained positively for SOX10 and S100 protein, supporting schwannian (neural crest) differentiation. The tumor's location and histopathology suggest that the pathogenesis stems from entrapment of sweat glands. Finally, it must be recognized that ancient change may mimic malignancy in these neoplasms as the malignant counterparts have a greater association with NF1 and a poorer prognosis.

Cutaneous meningiomas (CM) are a small subset of meningiomas, further classified into three subtypes. The authors present a 15-year-old male with a symptomatic congenital type I CM and describe the histopathological and immunohistochemical findings. To the authors' knowledge, this is the first report of an extraspinal lumbar type I CM with intradural attachment to the phylum terminale.

No abstract

No abstract

Relapsing linear acantholytic dermatosis (RLAD) is a rare disease that manifests as recurring episodes of crusted and vesicular lesions distributed in a Blaschkoid pattern with histology resembling Hailey-Hailey disease. RLAD, in the presence of generalized disease, has been shown to be a type 2 mosaic form of Hailey-Hailey disease. RLAD, without systemic disease, has been hypothesized to be type 1 mosaic Hailey-Hailey disease, but this assertion has lacked genetic conformation. To determine the genetic abnormalities causing RLAD, we performed exome sequencing of affected tissue and blood in one patient. Exome sequencing of a punch biopsy revealed a c.238A>T, p.(Lys80*) variant in ATP2C1 found in 26% of the reads from lesional skin but absent in germline DNA. This somatic variant causes a truncated protein that would likely result in loss of function. Our findings indicate that, in this patient, RLAD is a clinical presentation of type 1 segmental Hailey-Hailey disease.