Miliaria profunda.

Miliaria profunda is an uncommon but highly characteristic disorder that can be disabling. We describe a patient with miliaria profunda who responded to therapy with anhydrous lanolin and isotretinoin. Clinical features, pathogenesis, differential diagnosis, and therapy are reviewed.

Spontaneous improvement of a port wine stain.

A 3-year-old child had been born with an extensive vascular lesion that was clinically and histologically consistent with a port wine stain. Between the ages of six months and three years the lesion showed a remarkable degree of spontaneous resolution. This degree of improvement is exceptional after the age of six months and is documented photographically in this report.

Plantar warts in the athlete.

Plantar warts are thick, endophytic, hyperkeratotic lesions caused by human papilloma virus. Because they are frequently mistaken for calluses, they are often misdiagnosed in athletes. The diagnosis of a plantar wart is made by paring down the hypertrophic epithelium until multiple "seeds" are detected in the dermis representing the thrombosed vessels supplying the wart. A patch system containing 40% salicylic acid in the rubber-based vehicle is applied to the debrided site every 48 hours until healing occurs.

Multiple tumors of follicular infundibulum with sweat duct differentiation.

A 67-year-old man is reported with multiple tumors of follicular infundibulum containing ducts. Approximately 30 hypopigmented, scaling macules and minimally elevated papules were present on the face. Skin biopsy specimens from 5 representative lesions revealed similar findings. There was a proliferation of ramifying strands of pale-staining keratinocytes in the upper dermis showing connections with follicular infundibula of vellus follicles and epidermis. There was evidence of hair follicle differentiation with small follicular bulbs, papillary mesenchymal bodies, keratocysts, and occasional hair shafts in the tumor. These findings are characteristic of prior reports of TFI. Ducts were also present within the epithelial cords. Carcinoembryonic antigen, gross cystic disease fluid protein-15, epithelial membrane antigen, and S-100 protein were identified within the tumor. We theorize that the ductal elements within these TFI reflect the multipotential differentiating capacity of portions of infundibular epithelium.

Papular mucinosis associated with acquired immunodeficiency syndrome.

Papular mucinosis, also known as lichen myxedematosus, is a rare, idiopathic disorder in which focal deposits of mucin accumulate in the dermis. It has been previously reported in two patients with human immunodeficiency virus infection; we describe a third such case and note the presence of a granuloma adjacent to the mucin deposit. We also comment on other clinical manifestations of cutaneous mucin deposition and granuloma formation in human immunodeficiency virus infection.

Multiple perifollicular fibromas: report of a case and analysis of the literature.

Perifollicular fibroma is a cutaneous hamartomatous proliferation of the pilar connective tissue sheath. We describe a patient with multiple perifollicular fibromas and analyze the literature on this topic. Histologically, perifollicular fibroma is characterized by a concentric arrangement of collagen fibers surrounding a generally unaltered hair follicle. Clinically, it is usually multiple and occurs predominantly on the face and upper trunk. This clinical presentation is similar to that observed in patients with the Birt-Hogg-Dubé syndrome where, in addition to perifollicular fibromas, fibrofolliculomas, trichodiscomas, and acrochordons are found. Several reports of multiple perifollicular fibroma prior to the recognition of this syndrome may, in fact, represent cases of the Birt-Hogg-Dubé syndrome.

Idiopathic plantar hidradenitis: a neutrophilic eccrine hidradenitis occurring primarily in children.

Neutrophilic eccrine hidradenitis (NEH) has been described in a variety of clinical settings but is most often seen in leukemic patients receiving chemotherapy. We have recently encountered 6 healthy individuals, of whom 5 were children, who developed NEH localized to the feet. The patients were from 9 to 21 years of age. The presenting complaint was rapid development of tenderness of the feet with varying degrees of morbidity. At clinical evaluation, tender, erythematous papules and nodules were confined to the feet, primarily the plantar surfaces. The major differential diagnoses were erythema nodosum and vasculitis. Laboratory studies were non-contributory. Histologically, the findings were generally similar to those of NEH with certain exceptions, most notably the absence of syringosquamous metaplasia and the presence, in most cases, of neutrophilic abscesses in eccrine coils. Inflammatory and degenerative changes involved primarily the eccrine duct (coiled and dermal), and tended to spare the secretory apparatus. Stains for microorganisms were negative. There were brief recurrences in some of the patients, but those followed over time have remained well. We suggest the term idiopathic plantar hidradenitis for this condition.

Low-grade fibromyxoid sarcoma: case report and immunohistochemical study.

A case is presented of low-grade fibromyxoid sarcoma involving the arm of a 52-year-old man. Low-grade fibromyxoid sarcoma is a recently described neoplasm of the deep and subcutaneous soft tissue which demonstrates a spectrum of histologic images. The current case demonstrated the typical patterns of intermixed, sweeping bands of fibrous and myxoid tissue, homogeneous foci of fibrous and myxoid tissue, focal areas of storiforming, and concentric perivascular cuffs of slender spindle cells, all lacking the nuclear anaplasia, mitotic activity, and necrosis generally associated with sarcoma. Immunohistochemical analysis performed on paraffin-embedded sections demonstrated strong labeling of the tumor cells by anti-CD34 antibody, moderate labeling for vimentin, and rare, focal positivity for muscle-specific actin. Tumor cells were negative for markers of epithelial, muscular, neural, histiocytic, melanocytic, and vascular differentiation. The constellation of histopathologic features described in this and previous reports is characteristic of low-grade fibromyxoid sarcoma. Based on this case, it appears that the immunohistochemical features of low-grade fibromyxoid sarcoma can help to exclude many cutaneous and deep soft tissue tumors from the differential diagnosis. The findings support the interpretation that the neoplasm is essentially fibroblastic in nature.

Generalized eruptive keratoacanthoma of Grzybowski.

A patient with generalized eruptive keratoacanthoma of Grzybowski is described. Our findings, along with those of the 21 reported cases from the world literature, are summarized. This rare variant of keratoacanthoma most commonly affects patients in the fifth to seventh decades of life and appears as a generalized eruption of hundreds to thousands of follicular papules. These small papules often have a keratotic center and demonstrate the microscopic features of keratoacanthoma. Marked facial involvement is characteristic and can lead to masked facies with ectropion. Severe pruritus, mucosal lesions, and koebnerization are features of the disease. The course is chronic and the response to therapy is poor, although a few patients have improved with the use of systemic retinoids.

Plexiform and other unusual variants of palisaded encapsulated neuroma.

Palisaded, encapsulated neuroma (PEN) has been described as a predominantly solitary, nodular tumor; plexiform or multinodular growth patterns have not been reported in detail. We reviewed 55 PEN in order to evaluate: 1) the predominant growth patterns; 2) features that may indicate previous trauma; and 3) association with acne-like changes. Sixteen cases (29%) had growth patterns other than the solitary, nodular type. These patterns were: a) plexiform (7/16), b) multinodular (5/16), and c) fungating (4/16). Other unusual features included evidence of trauma, i.e., fibrosis, myxoid change, chronic inflammation (5/16), partially incomplete capsule (6/16), and association with acne-like features (13/16). These results suggest that: 1) plexiform or multinodular growth patterns occur relatively often in PEN; 2) some changes indicate a possible histogenetic relation to traumatic neuroma, probably via secondary traumatization; 3) a high frequency of association with acne-like changes which could imply, but does not prove, an acne-induced histogenesis; and 4) the differential diagnosis of cutaneous plexiform lesions should include PEN.

Proliferating cell nuclear antigen (PCNA) in common epidermal lesions. An immunohistochemical study of proliferating cell populations.

A commercially available antibody to proliferating cell nuclear antigen was used to characterize and compare proliferating cell populations in paraffin sections of benign, premalignant, and malignant lesions of human epidermis using routine immunohistochemical techniques. Three patterns emerged. An ordered pattern was found in prurigo nodularis and keratoacanthoma, wherein moderately and strongly positive nuclei were distributed in a continuous, basal-suprabasal layer of relatively uniform thickness. There was graded loss and ultimate extinction of PCNA staining in progressively more superficial epidermal cells. A basal dysplastic pattern was found in actinic keratosis and squamous cell carcinoma. Nuclei of essentially all dysplastic cells of both categories expressed PCNA, with a preponderance of strongly positive nuclei. These were localized to basal-suprabasal zones that were often expanded. Loss of PCNA reactivity toward the surface was often abrupt. Bowen's disease exhibited a diffuse dysplastic pattern, wherein large numbers of moderately and strongly positive nuclei, in random array, were present in essentially full thickness distribution. In many fields, however, a layer of cytologically bland basal cells, with faint or no nuclear staining, was interposed between dysplastic epithelium and dermis. This study has demonstrated that proliferating cell populations in epidermal lesions can be assessed with simple, inexpensive methods. There were consistent differences between the proliferating cell populations of the various entities studied, differences that can be reasonably correlated with other known clinical, microscopic, and biologic features of the lesions. This technique should provide an interesting new avenue for study of diverse cutaneous diseases.

Eruptive xanthomas during pregnancy.

A case of eruptive xanthomas during two successive pregnancies is reported. These xanthomas developed in association with marked hypertriglyceridemia; complications included severe pancreatitis and acute respiratory distress syndrome. This patient most likely had combined familial hyperlipidemia which usually causes only a modest elevation in plasma lipid levels. However, with the added stimulus of estrogens during pregnancy, hypertriglyceridemia and secondary complications developed.

Deep penetrating (plexiform spindle cell) nevus. A frequent participant in combined nevus.

This report describes 41 patients with lesions similar to those previously termed "deep penetrating" or "plexiform spindle cell" nevus (DPN). DPN occurs primarily during the first four decades, is somewhat more common in females, and has a predilection for the face, trunk, and proximal extremities. It is usually less than 1 cm in diameter and often shows variegation in color, including shades of brown, blue, and black, that create clinical concern regarding malignant melanoma. None of the present tumors nor those from the literature recurred following excision. Microscopically, DPN usually has a wedge shape, invariably involves reticular dermis, and may penetrate subcutis. Involvement of neurovascular structures and adnexae and spread between fibers of the reticular dermis create a fascicular-plexiform architecture. The melanocytes are fusiform or epithelioid, lightly to moderately pigmented, and exhibit mild to focally prominent nuclear atypia. Sparse to abundant melanophages are characteristic. Mitotic figures are few and present in only a small minority of lesions. The present study of a consecutive series also indicates that DPN is a frequent participant in combined nevus, as it was associated with ordinary nevus in two-thirds of the lesions.

Shave excision and dermabrasion for facial angiofibroma in tuberous sclerosis.

Tuberous sclerosis is an inheritable disease of varied manifestations. Hallmarks of the disease have historically been identified as infantile seizures, severe mental retardation, and facial growths. The facial lesions were formerly termed adenoma sebaceum, but are now known to be angiofibroma. We present a patient who was referred for management of large facial lesions complicated by intermittent hemorrhage. A combination of shave excision and dermabrasion led to a symptomatic and cosmetic improvement.

Spectrophotometric method to quantify and discriminate urokinase and tissue-type plasminogen activators.

Plasminogen activator and urokinase are often used as biological markers of cell activation. However, the methods currently used are cumbersome, make no discrimination between tissue-type plasminogen activator and urokinase, and do not allow expression of the results of the overall reaction in International Units. The one-step method described in this paper lacks these drawbacks. Moreover, we propose use of H-D-Val-Phe-Lys-4-nitroanilide as substrate which has a lower Km than the standard H-D-Val-Leu-Lys-4-nitroanilide which is commercially available. Low concentrations of sodium dodecyl sulfate in the reaction mixture dramatically and preferentially accelerate the reaction catalyzed by tissue-type plasminogen activators. Identical results are obtained under kinetic or fixed-time assay conditions using either a photometer or 96-well plate reader. The corresponding formulae are provided.

Late-onset nevus of Ota.

We report a patient with a type IB nevus of Ota whose lesion did not appear until she was thirty years old. To our knowledge, this represents the oldest age of a patient at clinical onset of nevus of Ota. The diagnosis, implications, and treatment of nevus of Ota are reviewed.