A Newborn Female with a Diffuse Rash.

Langerhans cell histiocytosis is a rare and clinically heterogeneous group of dendritic histiocytic disorders with typical onset in the neonatal period or infancy, although it can present at any age. Histiocytes accumulate in one or more organs, leading to a variable clinical presentation of disease. We report a case of biopsy-proven Langerhans cell histiocytosis in a newborn and discuss the workup and management of this disease, along with reviewing its clinical variants.

Importance of universal mismatch repair protein immunohistochemistry in patients with sebaceous neoplasia as an initial screening tool for Muir-Torre syndrome.

Muir-Torre syndrome, a Lynch syndrome variant, is characterized by sebaceous neoplasia plus one or more malignancies, typically colon cancer. The significance of DNA mismatch repair (MMR) deficiency detection by immunohistochemistry (IHC) in colorectal carcinomas is well established and is recommended as a screening tool for Lynch syndrome in newly diagnosed colorectal carcinomas. In comparison, literature on IHC application to detect MMR proteins (MLH1, MSH2, MSH6, and PMS2) in sebaceous neoplasia has been less studied and has been derived almost exclusively from tertiary care centers.

Cutaneous clues to renal cell carcinoma: hereditary leiomyomatosis and renal cell carcinoma.

We present a case of a 33-year-old female who was incidentally found to have cutaneous leiomyomata during a routine skin examination. Further history revealed that she also suffered from uterine fibroids and that her mother had died at an early age from renal cell carcinoma. This case serves as a reminder of the often-subtle cutaneous clues, as well as the importance of a multidisciplinary approach, for early diagnosis of potentially fatal conditions.

Histopathology of vascular anomalies.

Vascular anomalies may be appropriately classified into two broad categories, vascular tumors and vascular malformations, which are distinguished by the presence of cellular proliferation in contrast to aberrations in morphogenesis, respectively. This system of classification is based upon histological features that may in large part be differentiating, but nevertheless, may show morphological overlap. Advances in immunophenotyping allow for more precise diagnoses as well as further delineation of cell origins.

Primary cutaneous amyloidosis of the external ear: a clinicopathological and immunohistochemical study of 17 cases.

Primary cutaneous amyloidosis includes several forms of localized amyloidosis characterized by superficial amyloid deposits occurring at or near the dermal-epidermal junction in the absence of systemic involvement. Primary cutaneous amyloidosis of the auricular concha and external ear represents a rarely described variant. There have been 27 cases reported in the English language literature, and herein we report 17 additional cases.

Incontinentia pigmenti: treatment of IP with topical tacrolimus.

Incontinentia pigmenti (IP), or Bloch-Sulzberger syndrome, is a rare X-linked dominant genodermatosis primarily affecting females. Although IP affects many organ systems, the hallmark feature of this disease is its characteristic cutaneous eruption along the lines of Blaschko that evolves through four distinct stages: inflammatory/vesiculobullous, verrucous, hyperpigmented and hypopigmented/ atrophic. We describe a case of IP in its vesicular stage that completely resolved with topical Protopic (tacrolimus) 0.

De novo intraepidermal epithelioid melanocytic dysplasia: a review of 263 cases.

Background: De novo intraepidermal epithelioid melanocytic dysplasia (DNIEMD) is a newly characterized lesion that is associated with a personal and/or family history of malignant melanoma (MM) and/or dysplastic nevi (DN). However, the biological significance is still uncertain and the persons predisposed to this lesion have not been adequately described.

Methods: Clinicopathologic data of 258 patients, from 263 biopsies diagnosed with DNIEMD, was obtained.