Kenneth E. Greer

Acute febrile neutrophilic dermatosis (Sweet‐s syndrome) and myeloproliferative disorders

Acute febrile neutrophilic dermatosis (ND) consists of the abrupt onset of red, tender, cutaneous plaques on the face, extremities, and upper trunk, accompanied by fever, malaise, and neutrophilic leukocytosis. Histologically, there are distinctive, dense, dermal infiltrates of neutrophils. Response to systemic steroids is dramatic. This report describes four patients with leukemia or preleukemia and ND (LND), reviews reports of 12 similar patients, and compares LND with ND in otherwise healthy individuals (idiopathic ND, IND). Although lesions of LND more frequently had vesiculobullous appearances or location on mucous membranes, this study showed no consistent difference between LND and IND with regard to cutaneous signs, symptoms, histologic findings, and response to therapy. The first episode of LND either preceded or followed documentation of the myeloproliferative disorder, and the most common associated hematologic conditions were acute myeloid or myelomonocytic leukemia. Moderate to severe anemia was present in nine of ten patients whose first episode of LND preceded the discovery of the hematologic condition by eights months or less. The presence of anemia is the most obvious and readily detectable difference between LND and IND. The possibility of an underlying myeloproliferative disorder should be considered in all patients with ND, and LND should not be confused with infectious complications in patients known to have myeloproliferative disorders.

Squamous cell carcinoma in disseminated superficial actinic porokeratosis

We describe the clinical and pathologic observations of a 59-year-old woman with disseminated superficial actinic porokeratosis (DSAP) who developed squamous cell carcinoma on her leg. The tumor was surrounded by lesions of DSAP and appeared to develop from dysplastic epidermal cells located under cornoid lamellae.

The spectrum of normolipemic plane xanthoma

Normolipemic plane xanthoma (NPX) is a well-characterized clinicopathologic entity distinct from necrobiotic xanthogranuloma (NXG). We present the case of a patient with the classic clinical course of NPX but with many histologic features in common with NXG, namely, multinucleated lipophages, cholesterol clefts, and degeneration of collagen with associated cellular necrosis (necrobiosis). We suggest that NPX and NXG represent part of a spectrum of xanthomatous dermal reactions associated with paraproteinemias and may be more closely related than previously recognized.

Eruptive disseminated lobular capillary hemangioma (pyogenic granuloma)

A 43-year-old man reported a 2-month history of numerous widespread vascular lesions that had developed suddenly on his scalp, trunk, and extremities. The lesions were 2 to 6 mm in diameter, firm, dark red papules that bled easily with minor trauma. Histopathologic examination of four lesions revealed lobular capillary hemangioma, also known as pyogenic granuloma. Treated lesions did not recur after surgical excision or electrocauterization. Five months after the onset of the eruption, no new lesions were developing, and some were resolving spontaneously after bleeding. Eruptive, disseminated lobular capillary hemangioma is a rare, self-limited cutaneous disorder that occurs in otherwise healthy persons.

Papillon-Lefèvre syndrome: Report of two patients treated successfully with isotretinoin

The Papillon-Lefevre syndrome is an autosomal recessive disorder characterized by diffuse palmoplantar keratoderma and premature loss of both the deciduous and the permanent teeth. We report two female patients with PapillonLefevre syndrome in whom treatment with oral isotretinoin produced remarkable improvement of the keratoderma within 2 weeks. (J Am Acad Dermatol 15:46-49, 1986.)

Lipedematous alopecia : a clinicopathologic histologic and ultrastructural study

Lipedematous alopecia is a rare condition of unknown etiology characterized by a thick, boggy scalp with varying degrees of hair loss that occurs in adult black females, with no clearly associated medical or physiologic conditions. The fundamental pathologic finding consists of an approximate doubling in scalp thickness resulting from expansion of the subcutaneous fat layer in the absence of adipose tissue hypertrophy or hyperplasia. Observations by light and electron microscopy detailed in this report suggest that this alteration principally manifests by localized edema with disruption and degeneration of adipose tissue. Some diminution in the number of follicles as well as focal bulb atrophy is noted. Aberrant mucin deposition such as that seen in myxedema or other cutaneous mucinoses is not a feature. The histologic findings bear some resemblance to those seen in lipedema of the legs, a relatively common but infrequently diagnosed condition. We present a case of lipedematous alopecia with emphasis on histologic and ultrastructural features. The etiology is unknown.

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.

The treatment of keratosis palmaris et plantaris with isotretinoin: A multicenter study

Five of six patients with keratoderma palmaris et plantaris were safely and effectively treated with isotretinoin with dramatic and definite clearing of the keratoderma within the first 4 weeks of therapy. The mean dose was 1.95 mg/kg/day with a mean duration of 113 days.

Metastatic cutaneous breast carcinoma: A case report and review of the literature

Cutaneous metastatic carcinoma, also known as carcinoma erysipeloides, is an unusual clinical finding. In women, it is most frequently found in association with breast cancer rather than other visceral malignancies. Breast cancer patients can present with cutaneous manifestations of breast disease at the time of their initial diagnosis; however, cutaneous metastases more often present well after the initial diagnosis and treatment of the breast disease. Prompt recognition of skin metastases by the plastic surgeon, especially during the delayed setting of reconstructive surgery following mastectomy, allows for earlier intervention in treating the systemic spread of the disease. A clinical case of carcinoma erysipeloides presenting in this delayed setting is outlined in the present report, along with a review of the literature.

HEREDITARY SCLEROSING POIKILODERMA

ABSTRACT: Three persons with hereditary sclerosing poikiloderma were studied to find any clue to explain the mechanism involved in producing the cutaneous lesions which are so striking clinically and also evident histologically. Investigational studies included a blood chemistry screen, chromosome analyses, and skin biopsies evaluated by routine stains as well as by electron microscopy and direct immunofluorescence. No mechanism for the production of the clinical and histological changes in the dominantly inherited disorder was found.