The cutaneous manifestations of atypical complete DiGeorge syndrome: a histopathologic and immunohistochemical study.

DiGeorge syndrome is a congenital anomaly with a constellation of findings that includes thymic hypoplasia. Only a small subset of patients with DiGeorge syndrome has complete athymia, classified as complete DiGeorge anomaly; one third of these patients show an eczematous dermatitis, oligoclonal T-cells and lymphadenopathy, known as atypical complete DiGeorge anomaly. Six biopsies from six patients with the distinctive clinical phenotype of atypical complete DiGeorge anomaly were studied. Every biopsy showed exocytosis (100%), parakeratosis, often confluent and spongiosis (100%). Neutrophilic abscesses (50%), dyskeratosis (67%) and satellite cell necrosis (50%) were seen. Perieccrine and perivascular inflammation were seen in half of the cases. Eosinophils were identified (83%); most commonly in both the epidermis and dermis. All of lymphocytes were CD3 positive. Most (83%) of cases contained T-cell intracellular antigen 1 (TIA-1) positive cells. Special testing of the selected patients using spectratyping identified oligoclonal T-cell populations. The presence of dyskeratotic keratinocytes, satellite cell necrosis and parakeratotic scale with neutrophils characterizes the cutaneous rash seen in this subset of complete DiGeorge syndrome patients. Such skin lesions from patients with DiGeorge anomaly should alert the pathologist to the potential diagnosis of atypical complete DiGeorge anomaly. The pathophysiologic role of the oligoclonal T-cells in this entity requires additional study.

Melanocytic nevi with nonsurgical trauma: a histopathologic study.

There is a belief among dermatopathologists that benign melanocytic nevi (BMN) may display atypical histologic characteristics when traumatized. However, to our knowledge, a systematic study of nonsurgically traumatized melanocytic nevi (TMN) has not been published. We studied a series of 92 TMN. Cases were analyzed for histologic evidence of architectural and cytologic criteria associated with atypia. Of the patients, 54 were female and 37 were male. The mean age was 38 years old (range 8-74 years old). Nevi were present, in order of frequency, on the extremities, trunk, and head/neck, but there were no acral sites. Histologic findings of trauma were as follows: parakeratosis (92%), dermal telangiectasias (61%), ulceration (51%), dermal inflammation (49%), melanin within stratum corneum (24%), and dermal fibrosis (25%). Pagetoid spread of melanocytes was limited to the site of trauma in 20% of cases and was identified away from areas of trauma in 8% of cases. Melanocytic atypia was seen in three cases. Dermal mitoses were rare (one mitotic figure in three cases). Pagetoid spread under a traumatized epidermis was relatively frequent and, in isolation, is compatible with a benign TMN. Any traumatized melanocytic lesion that displays cytologic atypia, pagetoid spread outside of the area of the traumatized epidermis, or dermal mitoses should be treated with caution because these findings were rarely seen in TMN.

A physician's perspective on the dining experience in long-term care.

Unfortunately, weight loss is frequently an expected part of a patient's normal nursing home residential trajectory. However, the clinical team should determine if the weight loss is reversible. The patient's clinical condition is often such that weight loss cannot be reversed or improved. Currently, life expectancy for a patient who has been admitted to a nursing home is approximately 2.2 years. These patients have been suffering from multiple medical conditions that have ravaged their body and mind and left them in a frail condition. Food has many personal meanings to each resident that can improve the quality of a person's few remaining years. While many specialty diets are available to patients in a hospital, many of these diets may not be appropriate for patients who reside in a nursing home. Careful attention should be given to the prescription and preparation of meals in long-term care facilities. A focus on liberalizing diets in long-term care facilities can lead to improved quality of life for many patients.

Fine-needle aspiration cytology of endometrioid adenofibroma of the ovary.

We report the fine-needle aspiration (FNA) cytology findings of endometrioid adenofibroma arising in the ovary of a 60-year-old woman who presented with vaginal bleeding. Imaging studies revealed a large pelvic mass, which was sampled by computed tomography-guided FNA and core biopsy. The FNA yielded cellular smears composed of bland endometrioid cells and fragments of ovarian-type stroma. The core biopsy showed a biphasic process comprising bland endometrioid glands in a spindle-cell stroma. Immunohistochemical studies performed on the core showed the stroma to be CD10-negative and smooth muscle actin-positive. Subsequent resection of the tumor confirmed the diagnosis and revealed an adenocarcinoma arising in the tumor that was not sampled by FNA. To our knowledge, the cytologic features of ovarian endometrioid adenofibroma have not been previously described.

Myelolipoma associated with adrenal ganglioneuroma.

Adrenal myelolipomas are rare, benign mesenchymal tumors composed of mature adipose tissue and hematopoietic cells in varying proportions. Although the majority of cases occur as isolated adrenal lesions, myelolipomas have been described in association with various adrenal pathologic conditions. These conditions include enzyme deficiencies and hyperplastic and neoplastic lesions of the adrenal cortex, with perhaps endocrine dysfunction as a common feature. Ganglioneuroma is a benign tumor of the sympathetic nervous system that rarely produces symptoms of endocrine dysfunction. We report an unusual case of myelolipoma associated with ganglioneuroma of the adrenal medulla. The histogenesis of myelolipoma remains speculative. However, the close proximity to adrenal cortical cells within the stroma of ganglioneuroma suggests that the hormonal microenvironment may have played a role in the development of the myelolipoma.