Incidence of residual basal cell carcinoma in patients who appear tumor free after biopsy.

BACKGROUND: Basal cell carcinoma (BCC) biopsy sites often heal with no clinical evidence of residual tumor. OBJECTIVE: The purpose of our study is to determine whether such patients require further therapy. If biopsies can be curative, health care costs can be reduced by avoiding unnecessary surgery. METHODS: We prospectively evaluated 41 consecutive subjects with 42 biopsy-confirmed BCCs who appeared disease free. Each biopsy site was excised and processed by the Mohs micrographic technique. The tissue block was sectioned horizontally at 30-micrometer intervals until exhausted. Sections were stained and examined microscopically for residual tumor. RESULTS: Tumor was identified in 28 (66%) of 42 cases. No statistically significant relationship was found between the presence or absence of residual tumor and the following variables: age, sex, tumor location, biopsy technique, histopathologic subtype, scar size, time from biopsy to surgery, and extent of inflammation in histologic sections. CONCLUSION: Our data suggest that patients with small (< 1 cm) primary BCCs that appear to be completely removed after a biopsy procedure are at risk for recurrence without further treatment.

Exaggerated arthropod-bite lesions in patients with chronic lymphocytic leukemia: a clinical, histopathologic, and immunopathologic study of eight patients.

BACKGROUND: Unusual papulovesicular lesions resembling arthropod bites have been described in patients with chronic lymphocytic leukemia (CLL). OBJECTIVE: Our purpose was to describe and characterize further the clinical, histopathologic, and immunopathologic features of these lesions. METHODS: Eight patients were identified retrospectively who had CLL and characteristic skin lesions. Clinical and histologic features were recorded. Skin biopsy specimens were analyzed immunohistochemically for eosinophil granule major basic protein, eosinophil-derived neurotoxin, neutrophil elastase, and mast cell tryptase. RESULTS: The clinical features, including the lesional distribution, suggested arthropod bites, although most patients could not recall having been bitten. Mixed T- and B-cell lymphoid cell infiltrates were present within lesions, along with prominent eosinophil infiltration and eosinophil granule protein deposition. CONCLUSION: Exuberant papulovesicular lesions develop in patients with CLL apparently as an exaggerated response to arthropod bites. Prominent eosinophil infiltration and degranulation within these lesions likely contribute to the severity of symptoms.

Asteroid bodies and other cytoplasmic inclusions in necrobiotic xanthogranuloma with paraproteinemia.

BACKGROUND: Necrobiotic xanthogranuloma (NXG) with paraproteinemia is a distinctive palisading granuloma of the skin. Extracutaneous lesions are rarely present. OBJECTIVE: The purpose of this study was to confirm the presence and significance of giant cell asteroid bodies and other cytoplasmic inclusions in NXG with paraproteinemia. METHODS: Skin biopsy specimens from 24 patients with NXG with paraproteinemia were reviewed; autopsy and lung biopsy specimens from two patients were stained for iron, calcium, and polysaccharide. RESULTS: Giant cell asteroid bodies were observed in skin biopsy specimens of 8 (33%) of the 24 patients. In addition, large acidophilic polygonal cytoplasmic inclusions were observed in myocardial tissue of one of the autopsy cases. Iron and calcium were not found. CONCLUSION: Asteroid bodies and other inclusions can be present in the giant cells of NXG with paraproteinemia. They are as frequent as, or more frequent than, in other granulomatous diseases and should be considered in the diagnosis of NXG with paraproteinemia.

Thyroid carcinoma metastatic to the skin: a cutaneous manifestation of a widely disseminated malignancy.

BACKGROUND: Cutaneous metastases develop in 2% to 9% of patients with an internal malignancy. Thyroid carcinoma metastatic to the skin is a rare clinical entity. OBJECTIVE: Our purpose was to study the clinical and pathologic features and outcome in patients with cutaneous metastasis from thyroid carcinoma. METHODS: The study included a retrospective analysis of six patients with skin metastases from thyroid carcinoma and a review of the English-language literature since 1964. RESULTS: Including our six patients, 43 patients with skin metastases from thyroid carcinoma have been reported. Papillary carcinoma was the most common (41%), followed by follicular (28%), anaplastic (15%), and medullary carcinomas (15%). The scalp was the most common site of metastasis. For our patients, the average length of survival after diagnosis of cutaneous metastasis was 19 months. CONCLUSION: Cutaneous metastasis from thyroid carcinoma is rare and occurs in the setting of disseminated neoplastic disease.

Indeterminate cell histiocytosis treated successfully with 2-chlorodeoxyadenosine.

We report the case of a 54-year-old man with a ten-year history of a generalized papular eruption consistent with the diagnosis of indeterminate cell histiocytosis. The patient responded favorably to a course of treatment with 2-chlorodeoxyadenosine.

Localized involutional lipoatrophy: a clinicopathologic study of 16 patients.

BACKGROUND: Localized loss of adipose tissue without antecedent clinical or histologic inflammation is termed idiopathic lipoatrophy. OBJECTIVE: Our purpose was to study the clinical and pathologic features in 16 patients with clinically focal lipoatrophy and a distinct pathologic pattern of fat lobule involution. METHODS: A retrospective study of 16 patients was performed. RESULTS: The buttocks and proximal extremities were involved most frequently. Lesions were solitary in 10 patients and multiple in six. Nine patients had received intramuscular or intraarticular corticosteroid or antibiotic injections in the affected areas before the development of lipoatrophy. Histologic examination showed that individual fat cells were decreased in size and separated by hyaline material. Progressive reduction in the size and number of adipocytes resulted in diminutive fat lobules with prominent vessels resembling embryonic fat lobules. Some adipocyte masses were acidophilic. Scattered macrophages, confirmed by immunoperoxidase staining for CD68 (KP-1), were identified within the fat lobules and surrounding connective tissue. Yellow-gray granules were recognized within the cytoplasm of macrophages in nine cases. Macrophages becoming lipophages were observed by electron microscopy in one case. Other inflammatory cells were not prominent. CONCLUSION: This is a common pattern of postinjury response to fat tissue characterized by macrophage infiltration of the fat lobules in variable numbers. The term involutional lipoatrophy is justified by the resemblance of the distinctive pathologic changes to embryonic fat lobules.

Pancreatic panniculitis.

BACKGROUND: Pancreatic panniculitis is a rare disease in which necrosis of fat in the panniculus and other distant foci occurs in the setting of pancreatic disease. OBJECTIVE: Our purpose was to study the clinical and histopathologic features of this disease. METHODS: We conducted a retrospective review of 11 patients. RESULTS: In five patients subcutaneous fat necrosis preceded the diagnosis of pancreatic disease by an average of 13 weeks. Five of our patients had underlying pancreatitis, and the remainder had carcinoma of the pancreas. Joint manifestations were prominent in six patients. Intestinal submucosal fat necrosis developed in two cases and caused massive gastrointestinal bleeding in one. Acute lesions demonstrated focal fat necrosis with "ghost cells," pathognomonic of this condition. Chronic lesions showed granulomatous and lipophagic panniculitis, with rare areas of basophilic fat necrosis. Cholecystectomy for gallstone pancreatitis was curative in two patients. CONCLUSION: Pancreatic panniculitis has distinctive clinical and pathognomonic histopathologic findings that can be the presenting features of pancreatic disease.

The vascular calcification-cutaneous necrosis syndrome.

BACKGROUND: Although medial calcification of larger elastic arteries in chronic kidney failure and with advancing age is relatively common, calcification of the cutaneous vascular system is rare. OBJECTIVE: Our purpose was to describe three patients with the vascular calcification-cutaneous necrosis syndrome and review the cause, clinical and pathologic features, and treatment of this syndrome. METHODS: We describe three patients with ischemic necrotic ulcers and underlying cutaneous vascular calcification. The clinical setting was abnormal calcium metabolism from either chronic kidney failure or excessive vitamin D intake. RESULTS: The clinical findings in all patients consisted of multiple tender livedoid nodules and ulcerative plaques on the thighs and legs, which developed in the setting of abnormal calcium metabolism from either chronic kidney failure or excessive vitamin D intake. Histologic study demonstrated vascular calcification. Although this syndrome usually has a chronic course with significant morbidity and mortality, subtotal parathyroidectomy followed by kidney transplantation resulted in complete resolution in one of our patients. CONCLUSION: The clinical and histopathologic findings in the vascular calcification-cutaneous necrosis syndrome are unique. The pathogenesis is likely multifactorial. Treatment for the skin lesions is largely supportive.

Jadassohn-Lewandowski syndrome (pachyonychia congenita).

Pachyonychia congenita is an uncommon autosomal dominant disorder with variable expression. Symmetrical nail hypertrophy, present in nearly all cases, is accompanied by dyskeratosis and dysplasia of other ectodermal tissues. This article reviews the genetics, clinical manifestations, histopathology, and treatment of pachyonychia congenita. Many clinical features have been reported in association with this syndrome. From a review of the literature, we propose criteria for the diagnosis of pachyonychia congenita using the more important of these clinical manifestations.

Noonan syndrome.

The Noonan syndrome is a rare disease characterized by dysmorphic facies, short stature, ear abnormalities, cryptorchidism, ocular abnormalities, cardiovascular anomalies, cubitus valgus, webbed neck, and cutaneous and hair abnormalities. Some 25% to 40% of patients have dermatologic abnormalities. Diagnosis is purely clinical, and intrauterine diagnosis is very important based on the presence of cystic hygroma and evidence of myocardial abnormalities. Treatment is symptomatic. Genetic counseling is necessary.