Purnima Sau

Cutaneous deposition diseases. Part II

Part II of the cutaneous deposition disorders focuses on cutaneous calcification and ossification, alkaptonuria and ochronosis, and gout. These disorders have in common the deposition of materials in the dermis or subcutis and often involve metabolic defects in hormonal and enzymatic regulation. The pathogenesis, clinical findings, and treatment of these diseases are discussed. Both the histologic and ultrastructural findings are reviewed.

Proliferating epithelial cysts : clinicopathological analysis of 96 cases

Ninety‐six proliferating cutaneous epithelial cysts were classified into two subtypes, proliferating trichilemmal cysts (PTC) and proliferating epidermoid cysts (PEC), depending on the mode of keratinization or the origin of the tumors. The clinicopathological features and the biological behavior of these two subtypes were compared. Among 63 patients with PTC, 45 (71%) were women and 18 (29%) were men. The most common site was the scalp (78%), followed by the trunk (13%). These tumors were well circumscribed subepidermal lesions and demonstrated uniform histologic pattern with varying degrees of cytologic atypia. A few tumors extended into the epidermis and occasionally became ulcerated. Follow‐up of 59 (94%) PTC for an average of 4 years revealed recurrence in one. Ten tumors demonstrated carcinomatous changes including one with anaplastic carcinoma and regional lymph node metastasis. None of these tumors recurred or developed further metastasis following wide excision. Of 33 PECs, 12 (36%) occurred in women and 21 (64%) in men. These tumors were widely distributed in the pelvic and anogenital areas (36%), followed by the scalp (21%), upper extremities (18%), and trunk (15%). Seventy‐nine percent of the PECs were located in areas outside the scalp. The PECs were subepidermal tumors but often communicated to the surface. The histologic pattern of PEC was more variable than that of PTC. Seven tumors exhibited carcinomatous changes. Follow‐up of 30 (91 %) PEC revealed local recurrences in 6, with multiple recurrences in 3, and extensive local invasion in 2, resulting in death in one. Greater anaplasia, high mitotic rate and deeper invasion were associated with increased incidence of recurrence and aggressive behavior. Although both PTC and PEC were locally aggressive tumors and potentially malignant, distant metastasis was unusual. These tumors should be treated with wide local excision, especially those showing cytologic atypia and carcinomatous changes.

Pigmented spindle cell nevus: A clinicopathologic analysis of ninety-five cases†

BACKGROUND Pigmented spindle cell nevus (PSCN) is often interpreted as a Spitz nevus or misdiagnosed as malignant melanoma. OBJECTIVE The purpose of this study was to analyze the clinical and histologic features and to determine the biologic behavior of 95 cases of PSCN. METHODS We reviewed clinical data, follow-up information, and microscopic features of all 95 cases of PSCN. RESULTS PSCNs are dark brown to black, 3 to 6 mm in diameter, and occur most commonly on the extremities (75%) and back (16%) with a predilection for the legs. These lesions are more common in women in the third decade of life. Microscopically, PSCNs are characterized by uniform, spindle-shaped, pigmented melanocytes. Although some histologic features overlap with those in spindle and epithelioid cell nevus, PSCN is a separate entity. In addition, PSCN must be differentiated from malignant melanoma. Fifty-seven patients (60%) observed for an average of 6 years did not develop local recurrence or metastasis. CONCLUSION PSCN is a distinctive, acquired, benign melanocytic lesion, that should not be confused with spindle and epithelioid cell nevus or malignant melanoma. Complete excision is recommended for treatment.

Confluent and reticulated papillomatosis: Response to minocycline

BACKGROUND Confluent and reticulated papillomatosis (CRP) of Gougerot and Carteaud is an uncommon disorder of unknown cause for which a variety of treatments have been proposed. OBJECTIVE We attempted to evaluate the effectiveness of oral minocycline. METHODS Nine patients with CRP were treated with oral minocycline, 50 mg twice a day, for 6 weeks. The average follow-up period was 11 months. Recurrence rate, side effects, and effectiveness of therapy were assessed. RESULTS All patients except two had a 90% to 100% response to therapy. Recurrences were noted in three patients, all of whom responded to re-treatment with minocycline. None of the nine patients had an adverse reaction. CONCLUSION Minocycline, 50 mg twice a day, is safe and effective for CRP.

Intravenous pyogenic granuloma

A 43‐year‐old man presented with a 1‐month history of a nodule on the left side of the neck. There were no subjective symptoms. He denied any history of trauma.

Trichogerminoma. Report of 14 cases.

We report 14 cases of trichogerminoma, a rare form of cutaneous adnexal neoplasm, derived from hair germ epithelium. The neoplasm occurred in 9 men and 5 women. Their ages ranged from 16 to 73 years (median 53 years). The tumors were slow growing, asymptomatic dermal or subcutaneous nodules, located on the head and neck (6), trunk (4), extremities (2) and hip (1), with no distinguishing clinical features. Histologically, trichogerminomas were characterized by sharply circumscribed, pseudo‐encapsulated dermal and subcutaneous nodules, ranging in size from 0.4 to 4.0 cm in diameter (mean 1.9 cm). The nodules were subdivided into lobules separated by variable amounts of stroma that demonstrated varying cellularity and mucin content. The lobules were composed of basaloid cells that formed densely packed, round nests or “cell balls” resembling hair bulbs. The basaloid cells demonstrated peripheral palisading, keratinization and differentiation towards various pilosebaceous structures. Retraction spaces, well developed hair follicles and hair shafts were not observed. These distinctive histologic features separated these neoplasms from other tumors of pilar origin and from basal cell carcinoma. The trichogerminomas behaved in a benign fashion with one exception. Complete excision of the lesions is the treatment of choice.

Hypersensitivity to paclitaxel manifested as a bullous fixed drugeruption

Paclitaxel is the first of a new class of microtubule-stabilizing antitumor agents, with demonstrated activity against advanced and refractory ovarian, breast, lung, and head and neck cancers. It was first isolated from the bark of the endangered Pacific yew Taxus brevifolia. 1 Neutropenia is the dose-limiting toxicity of paclitaxel. A high incidence of major hypersensitivity reactions (HSRs) (including those with cutaneous manifestations) occurred in phase I studies, but they were minimized by premedication schemes and prolonged administration times. 2 We describe a previously unrecognized hypersensitivity to paclitaxel that we classified as a bullous fixed drug eruption.

Basal cell carcinoma of the nipple: Report of two cases

Two cases of basal cell carcinoma of the nipple are presented, bringing the total number of reported cases to 15. The majority, including our two patients, are elderly men. This finding suggests a causal role of exposure to ultraviolet radiation. In our cases excision was curative.

Epithelioid hemangioendothelioma associated with reflex sympathetic dystrophy

A patient with long-standing reflex sympathetic dystrophy of the foot developed an aggressive epithelioid hemangioendothelioma of the involved extremity. The tumor metastasized to a regional lymph node and later to the lungs. The features of epithelioid hemangioendothelioma and reflex sympathetic dystrophy are reviewed.

Phialophora verrucosa: A new cause of mycetoma

A 29-year-old Thai woman had draining sinus tracts, tumefaction, and granules on the plantar aspect of the foot. Phialophora verrucosa was isolated from the lesion. P. verrucosa is a major agent of chromoblastomycosis, which is known to rarely cause subcutaneous phaeohyphomycosis. This dematiaceous fungus has not been previously reported to cause mycetoma. This case illustrates the clinical spectrum of disease of this fungus. The salient features of mycetoma and management options are presented.