Pure apocrine nevus: a report of 4 cases.

Apocrine nevus is a rare tumor composed of increased mature apocrine glands and ductal structures within a fibrous stroma, located predominantly in the reticular dermis. They have been reported in association with apocrine carcinoma, extramammary Paget disease, and syringocystadenoma papilliferum; less commonly a pure apocrine nevus is identified, unassociated with another apocrine proliferation. Clinically apocrine nevi may appear as solitary or multiple nodules or plaques on the scalp, presternal skin, though they are seen most commonly in the axillae. We describe 4 cases of pure apocrine nevus, all of which appeared clinically as painless or mildly tender skin-colored axillary masses, 2 of which were bilateral. In each case, the lesions appeared in adulthood, and patients denied knowledge of congenital or childhood presence. Patients denied pruritis, discharge, bleeding, or antecedent trauma. Grossly, the specimens consisted of subcutaneous, multicystic ill-defined nodules. Biopsy showed prominent apocrine glands composed of irregularly columnar luminal cells with eosinophilic cytoplasm arranged in a somewhat organoid pattern filling the reticular dermis and extending into the subcutaneous adipose tissue. The glandular luminal cells displayed decapitation secretion. There was a paucity of pilosebaceous units. In one case, the overlying epidermis was papillomatous. The deepest portion of one specimen had lactational change simulating a lactational adenoma. No atypia was seen in either the glandular structures or the stroma. The adjacent sebaceous and eccrine structures were normal. The histologic features and immunohistochemical profile in relation to other apocrine lesions will be reviewed.

Rapid onset of argyria induced by a silver-containing dietary supplement.

We describe a 53-year-old man in good general health who presented with an 8-month history of progressive gray hyperpigmentation of the face. He denied using any prescription medications; however, he admitted to taking a herbal supplement. Clinically, the differential diagnosis included hemochromatosis, Wilson's disease and hyperpigmentation secondary to supplement use. Punch biopsies from the left forehead and preauricular region showed heavily sun-damaged skin with a minimal inflammatory infiltrate. Closer inspection, however, revealed minute scattered black/brown particles distributed in the basement membrane zone of eccrine and sebaceous glands. Similar particles were also present in hair follicles, blood vessels and arrector pili muscles. The particles did not stain with Gomori methenamine silver, Fontana-Masson or iron stains. Electron microscopy with energy-dispersive x-ray analysis showed numerous particles, less than 1 µm in greatest dimension, which showed peaks for silver and sulfur. This analytical result confirmed the impression of argyria. Further history revealed that the patient had indeed been taking a silver supplement for several months under the premise that it would boost his immune system. This case is unique in that the patient's hyperpigmentation developed in a short period of time as compared with other reports in the medical literature.

Dermatomyositis: a clinicopathological study of 40 patients.

The histopathology of cutaneous lesions of dermatomyositis (DM) may be indistinguishable from acute cutaneous lesions of systemic lupus erythematosus (SLE). Misreported or incomplete clinical information may result in a clinicopathologic discrepancy and a delay in making a correct diagnosis of DM. The aim of this study was to systematically characterize the histopathologic findings of cutaneous lesions of DM and to determine if skin biopsy specimens of DM and SLE could be distinguished by light microscopic examination. Biopsies from 40 patients diagnosed with DM at the Wake Forest University School of Medicine from 1994 to 1999 were reviewed. The histological features by light microscopy were graded in a systematic fashion. We then assessed whether the cutaneous pathological changes of DM could be distinguished from those of SLE. Ten biopsy specimens each of DM and SLE (matched for anatomical site and lesion morphology) were randomized. Histological grading was performed in a blinded fashion, as was a histopathologic diagnosis (DM versus SLE). The most consistent histological findings of DM included increased dermal mucin, vacuolar alteration of the basal cell layer, and mild-to-moderate mononuclear cell inflammatory infiltrates. Our results show that the histological grading of SLE skin biopsies was nearly identical to that of DM. The correct histopathologic diagnosis of DM or SLE was made in 11 of the 20 skin biopsies without clinical information. Despite the limitations of our small sample size, these findings suggest that acute cutaneous lesions of SLE cannot be distinguished from DM. Clinicopathologic correlation is important for making a diagnosis of DM or SLE.

Neurothekeoma: an analysis of 178 tumors with detailed immunohistochemical data and long-term patient follow-up information.

This report describes the clinicopathologic findings in 176 patients who presented with 178 tumors currently referred to as neurothekeomas. Our study group included 64 males and 112 females, ranging from 20 months to 85 years old at the time of their first surgical procedure (median age: 17 y). Twenty-four percent of patients were or=30 years of age at initial diagnosis. The patients typically presented with a solitary, superficial, slow-growing, and relatively asymptomatic mass in the 0.3 to 2.0 cm size range. One patient had multiple tumors. More than 75% of the lesions involved the head (n=63), upper extremities (n=44), and shoulder girdle (n=27) regions. The tumors were evident a few weeks to 4 years (median duration: approximately 7 mo) before surgical resection was sought. Histologically, the lesions involved the dermis and/or subcutis, and they formed multinodular masses with varying amounts of myxoid matrix and peripheral fibrosis. On the basis of the amount of myxoid matrix, the tumors were subclassified as cellular (n=63), mixed (n=67), or myxoid (n=48). All cases had spindled and epithelioid mononuclear neoplastic cells with relatively abundant cytoplasm and indistinct cell borders. The majority of cases also had occasional multinucleated tumor cells. The lesional cells had a strong tendency for whorled growth, and oftentimes, focal fascicular growth was also present. Nuclear atypia was minimal in 62 cases, mild in 73 cases, at least focally moderate in 41 cases, and focally marked in 2 cases. Mitotic activity ranged from 0 to 124 mitotic figures/25 wide-field high power fields (WHPFs) (median mitotic count: 4 mitotic figures/25WHPFs). Twenty-five lesions had >10 mitotic figures/25WHPFs. A total of 16 cases (9%) had atypical mitotic figures. Osteoclastlike giant cells were detected in 39% of cases. Immunoreactivity was typically present for vimentin, NKI/C3, CD10, microphthalmia transcription factor, and PGP9.5, and focal reactivity was sometimes noted for smooth muscle actin and CD68. All tumors tested were negative for S100 protein, glial fibrillary acidic protein, and Melan A. The overwhelming majority of cases had involvement of the tissue margins. A complete follow-up record is available for 71 patients (40.3%) with follow-up intervals ranging from 3 years 2 months to 34 years 9 months (median: 17 y 9 mo). Limited or incomplete follow-up information is also available for an additional 14 patients with follow-up intervals ranging from weeks to approximately 10 years (median: 5 mo). Regrowth of tumor after biopsy or local excision was reported in 13 patients, one of whom had 2 recurrences. However, because of the nature of our consultation practice and a tendency for clinicians to specifically send us cases with a complex clinical course, this is believed an overestimation of the true recurrence rate. Neurothekeomas are morphologically and immunohistochemically distinct from true nerve sheath myxomas. An origin from fibroblastic cells with the ability to differentiate into myofibroblasts and a tendency to recruit histiocytic cells is postulated.

Lymphedematous fibroepithelial polyps of the glans penis and prepuce: a clinicopathologic study of 7 cases demonstrating a strong association with chronic condom catheter use.

This report describes an underrecognized entity of the penis that is associated with chronic condom catheter use and phimosis. Our study group consisted of 7 patients who presented with polypoid or cauliflower-like masses that involved the glans penis or prepuce and that ranged in size from 2 to 7.5 cm in greatest dimension (median size, 2.5 cm). The majority of lesions affected the ventral surface of the glans, near the urethral meatus. The patients ranged in age from 25 to 58 years (median age, 40 years) at the time of initial surgical resection. The preoperative duration of the lesions ranged from 6 months to 10 years. Five patients had a history of long-term condom catheter use (duration: 5 to 21 years), and 1 patient had paraphimosis. The background history for 1 patient is unknown. Histologically, all specimens had a polypoid configuration and a keratinizing squamous epithelial surface. The underlying stroma was notably edematous, and there was vascular dilation of preexisting vessels, and in many instances, a focal mild small vessel proliferation. The stroma had mildly to moderately increased cellularity with mononucleated and multinucleated mesenchymal cells. A mild inflammatory infiltrate was often present. Two cases were examined with immunohistochemistry, and the stromal cells had limited immunoreactivity for muscle-specific actin, alpha-smooth muscle actin, and desmin and had no reactivity for S100 protein or CD34. Surgical intervention was local in all instances. Follow-up information was available for 5 of the 7 patients (71%), with a mean follow-up interval of 11 years 4 months. Two patients developed a local recurrence of the process at intervals of less than 1 years and 3 years 7 months. Both recurrent lesions were also managed by local excision.

Primary cutaneous ganglioneuroma: a report of two cases and literature review.

Primary cutaneous ganglioneuroma is a rare neoplasm first described by Collins et al. in 1972. Eight cases have been reported in the English literature to date. We report two cases of solitary cutaneous ganglioneuroma, one constituting, to our knowledge, the first reported case of this entity occurring on the face. Clinically, both lesions were firm flesh-colored papules and asymptomatic, being removed primarily for cosmesis. Neither patient had any significant past medical history. Histologically, both neoplasms consisted of relatively well-circumscribed spindle cell proliferations of axons and Schwann cells with interspersed mature ganglion cells. There was also focal myxoid change. The spindle cell component stained positively for S-100 protein, and the ganglion cells stained positively for glial fibrillary acidic protein and neurofilaments by routine immunohistochemistry.

Microtubule associated protein (MAP-2) expression defines the companion layer of the anagen hair follicle and an analogous zone in the nail unit.

BACKGROUND: Microtubule associated protein 2 (MAP-2) is one of a group of polypeptides that are an integral component of the microtubular cytoskeletal structure of the central and peripheral nervous system. During the course of another investigation that utilized immunohistochemistry, MAP-2 expression was observed in the hair follicle, almost exclusively in the innermost layer of the outer root sheath of the anagen follicle. This innermost layer, the so-called companion layer, has unique properties that clearly distinguish it from the rest of the outer root sheath. Among these are diminished glycogenation and an intimate association with the Henle's layer, which it directly apposes. Numerous intercellular connections exist between the companion layer and Henle's layer and, in fact, the companion layer accompanies Henle's layer during its vertical ascent in the follicle. Circumferentially oriented keratin filaments have also been demonstrated between the companion layer and Henle's layer, apparently providing structural support to the inner root sheath. Experimentally, disturbances in the keratin filaments of the companion layer in animals ultimately results in destruction of the hair follicle and an alopecia. METHODS: Immunohistochemical studies for MAP-2 were performed on 25 additional paraffin-embedded scalp specimens using standard techniques. Because of the parallels between the follicle and nail, MAP-2 expression in the nail unit was also investigated in three specimens. RESULTS: The presence of MAP-2 in the companion layer was confirmed in all cases. Intense MAP-2 expression in the companion layer begins at the B-fringe (the start of the keratogenous zone with cornification of the Henle's layer of the inner sheath) and extends to the level of the isthmus where the inner root sheath exfoliates. MAP-2 is also expressed in the upper layers of the nail matrix. CONCLUSION: The expression of MAP-2 almost exclusively in the companion layer is probably related to the unique cytoskeletal structure of this microanatomic layer. Since experimental evidence has shown that the cell cytoskeleton is important to the integrity of the hair follicle, it is probable that MAP-2 expression is also important and disturbances in its expression could play a role in the pathogenesis of some alopecias. MAP-2 may play a similar role in the nail matrix.

Multiple herpetic whitlow lesions in a patient with chronic lymphocytic leukemia.

Herpetic whitlow, a herpes simplex virus infection involving the digits, most commonly presents as a vesicular eruption involving a single digit. Diagnosis of herpetic whitlow can usually be made with the history of exposure, the characteristic vesicular eruption, and a positive Tzank smear and/or viral culture. We describe a case of herpetic whitlow in a patient finishing 6 cycles of chemotherapy for refractory chronic lymphocytic leukemia that presented with a bilateral, multi-digit, crusted eruption of the hands. This is an illustrative case of an immunocompromised host status altering appearance and course of cutaneous disease such that the history and physical exam alone may not help in diagnosing atypical presentations of herpetic infections. This case underscores the necessity for clinico-histopathologic correlation.