Telangiectasia Macularis Eruptiva Perstans of the Scalp Mimicking Angiosarcoma
We present a case of a 74-year-old man with marked photodamage who was ultimately diagnosed with telangiectasia macularis eruptiva perstans (TMEP) of the scalp. The diagnosis was made more difficult because of the clinical and histological similarity of this case with an early angiosarcoma. TMEP is a benign and indolent rare subtype of cutaneous mastocytosis presenting clinically with red–brown telangiectatic macules, usually symmetrically distributed over the trunk and extremities. Although most cases are limited to the skin, systemic involvement can occur, and this can be a potentially life-threatening disease. Althoug...
Source: The American Journal of Dermatopathology - November 26, 2021 Category: Pathology Tags: Extraordinary Case Report Source Type: research

Pediatric Extraspinal Subcutaneous Sacrococcygeal Myxopapillary Ependymoma: Case Report and Minireview
Abstract: A 9-year-old girl presented with a slow-growing and painless mass for 7 months in the soft tissue of the sacrococcygeal region. Magnetic resonance imaging revealed a well-circumscribed solid mass located in the subcutaneous soft tissue of the sacrococcygeal area, but not affecting bone structures. The mass was completely removed, and the disorder was diagnosed as myxopapillary ependymoma. In addition, the MYCN gene amplification status of the tumor was evaluated. Extra-axial ependymomas are very rare tumors with a tendency to metastasis, but they are usually regarded as low-grade ependymomas. Long-time survei...
Source: The American Journal of Dermatopathology - November 26, 2021 Category: Pathology Tags: Extraordinary Case Report Source Type: research

A Young Boy With Hemophagocytic Lymphohistiocytosis Presenting With Vaccine-Related Granulomatous Dermatitis: A Case Report and Literature Review
We report the case of a 4-year-old boy who initially developed a widespread popular–pustular rash 2 weeks after his 12-month measles, mumps, and rubella vaccinations. These resolved with scarring then recurred following his 24-month vaccinations. Multiple skin biopsies were negative for infectious organisms and showed a granulomatous infiltrate with perforation and necrobiosis. The differential diagnosis included perforating granuloma annulare, infection, or rheumatoid nodules. At the age of 4, he developed fever, hepatosplenomegaly, pancytopenia and other laboratory abnormalities, requiring hospitalization. A number of ...
Source: The American Journal of Dermatopathology - November 26, 2021 Category: Pathology Tags: Extraordinary Case Report Source Type: research

Orbital TFE3-Rearranged Perivascular Epithelioid Cell Tumor: A Case Report and Review of the Literature
This report presents a 28-year-old man who consulted for a painless left orbital mass. With the presumptive diagnosis of cavernous hemangioma, a surgical excisional biopsy was performed. Histopathological examination showed a well-delimited tumor composed of epithelioid cells with an eosinophilic cytoplasm and oval nucleus. The tumor cells stained diffusely for HMB-45 and transcription factor E3 (TFE3) and were focally positive for actin. There was no reactivity to S100 or desmin. Genetic testing revealed a TFE3 rearrangement. Based on these results, an extremely rare orbital TFE3-rearranged PEComa was diagnosed. Although ...
Source: The American Journal of Dermatopathology - November 26, 2021 Category: Pathology Tags: Extraordinary Case Report Source Type: research

Mycosis Fungoides Preceding Pityriasis Lichenoides et Varioliformis Acuta by Twelve Years in a Pediatric Patient
Abstract: A 15-year-old boy presented to the pediatric dermatology department with long-standing patch stage CD8+ mycosis fungoides and subsequent development of recurrent pityriasis lichenoides et varioliformis acuta eruptions. There have been rare reports of patients with chronic, recalcitrant pityriasis lichenoides developing mycosis fungoides, but we believe this to be the second case of mycosis fungoides preceding a diagnosis of pityriasis lichenoides, and the first case reported in the pediatric population. (Source: The American Journal of Dermatopathology)
Source: The American Journal of Dermatopathology - November 26, 2021 Category: Pathology Tags: Extraordinary Case Report Source Type: research