Lichenoid eruption following penicillamine. A case report with some biochemical observations.

The phenomenon of lichenoid eruptions following medication with antimalarial, anti-arthritis and antibacterial medication is well documented in the literature. We report here the first case to be described as a result of anti-arthritis treatment with penicillamine, together with some biochemical studies of the effect of this compound on the enzyme glucose-6-phosphate dehydrogenase.     

Generalized morphea with blisters. A case report

A patient suffering from generalized morphea developed blisters in the morpheic plaques on her buttocks. The plaques had an increased concentration of serum aminoterminal propertice of type III procollagen, an echo response and thickened skin on ultrasound scanning, and compact bundles of collagen fibrils with bimodal distribution of the diameters. The blisters appeared as an echo-free band in the subepidermal zone by ultrasound scanning. Electron microscopy revealed blisters in the upper papillary dermis, surrounded by degraded collagen fibrils.     

Generalized eruptive histiocytoma. A case report with a review of the literature.

A case of generalized eruptive histiocytoma in a 52-year-old Indian male is described. This is probably the tenth case in the world and the first from this country. Histopathological features are discussed in detail and the literature is reviewed.     

Microcystic adnexal carcinoma: a case report with immunohistochemical and electron microscopical examinations.

Microcystic adnexal carcinoma (MAC) is an unusual cutaneous neoplasm comprised of pilar and sweat duct structures. We report here a 66-year-old Japanese woman with MAC on her left nasolabial fold with more prominent calcified keratinous cysts than ductal differentiated structures which stained positively for carcinoembryonic antigen (CEA). The clinical appearance and statistical characteristics of MAC are reported to be different from those of other adnexal tumors. Desmoplastic trichoepithelioma (DTE) is the most difficult tumor to differentiate from MAC, unless positive reactivity for CEA in differentiated ductal structures in MAC can be demonstrated. We compare the differences between the clinical, histological, immunohistochemical, and electron microscopical features of DTE and MAC. It seems probable that these differences could be explained by biological differences in the characteristics of the cells from which these tumors originate, hair follicle cells and eccrine sweat cells, respectively.     

Superoxide dismutase and myeloperoxidase activity in polymorphonuclear leukocytes, and serum ceruloplasmin and copper levels, in psoriasis

The levels of superoxide dismutase (SOD) and myeloperoxidase (MPO) in polymorphonuclear leukocytes (PMN), and serum ceruloplasmin activity and copper content, were measured in 60 patients with psoriasis and 33 healthy controls. There were no significant differences in the activity of MPO between the patients and the controls. However, SOD activity in PMN was significantly lower in the patients than in the controls. Serum ceruloplasmin activity and copper levels were significantly higher in the psoriatics than in the controls.     

Cutaneous macroglobulinosis. A case report with unique ultrastructural findings.

BACKGROUND--Cutaneous macroglobulinosis is a rare cutaneous manifestation of Waldenstr?m's macroglobulinemia. Lesions result from the direct deposition of macroglobulin in the skin and have been called IgM storage papules. A case of cutaneous macroglobulinosis with unique ultrastructural findings was studied. OBSERVATIONS--Cutaneous macroglobulinosis is characterized by multiple flesh-colored papules on extensor skin surfaces. Histologically, there are dermal collections of eosinophilic hyaline material, simulating amyloid. The material is positive on periodic acid-Schiff staining. Amyloid stains are negative or equivocal. Electron microscopy reveals thick, nonbranching, 56-nm-wide, linear material with cross striations at 12-nm intervals. These ultrastructural findings differ from the three previously reported cases. CONCLUSIONS--Cutaneous macroglobulinosis may be a rare presenting sign of Waldenstr?m's macroglobulinemia. Deposits of macroglobulin in the skin result in a histologic picture that greatly resembles amyloid. Histochemical stains, direct immunofluorescence microscopy, and electron microscopy are useful tools that enable accurate diagnosis and help to distinguish cutaneous macroglobulinosis from other deposition disorders.     

Xanthomatosis and other clinical findings in patients with elevated levels of very low density lipoproteins

Forty-six patients with xanthomatosis and elevated very low density lipoproteins (VLDL) levels (in different types of hyperlipoproteinaemia) were classified on the basis of the WHO criteria and the cholesterol/triglyceride ratio in VLDL. A large majority (31/46) of the patients referred to the Department of Dermatology could be classified as hyperlipoproteinaemia type III, only 8/46 as type IIB and 7/46 as type IV/V. This distinction seems to be relevant as the xanthomatous lesions differed distinctly between these three types of hyperlipoproteinaemia. Xanthochromia striata palmaris was present in 29/31 cases of hyperlipoproteinaemia type III and was not found in type IV/V patients, who had distinctive papuloeruptive xanthomas. During a follow-up in 35/46 patients all xanthomas disappeared within 2 years except the xanthelasma palpebrarum and tendinous xanthomas. All type IV/V patients (7/7) but only one type III patient (1/31) had abnormal glucose tolerance. Only 2/18 type III patients less than 45 years showed claudication and none of the young type III patients had angina pectoris. In contrast, all four type IIB patients less than 45 years had clinical signs of atherosclerosis. However, angina pectoris and/or claudication were present in 5/13 type III patients over 45 years old. The mean serum cholesterol level was equally elevated in both groups but the cholesterol was mainly present in VLDL in type III and in low density lipoproteins (LDL) in type IIB. In 9/31 type III patients the LDL level was also elevated but was easily normalized by a diet low in carbohydrate, whereas the elevated LDL level in type IIB was therapy-resistant. The recognition of xanthomatous lesions, specifically xanthochromia striata palmaris, as an early sign of type III hyperlipoproteinaemia, can lead to the early diagnosis and successful treatment of these patients, and thus possibly prevent the development of premature atherosclerosis.     

Chemical and traumatic alopecia from thioglycolate in a black woman: a case report with unusual clinical and histologic findings.

We report an unusual case of verrucal alopecia occurring on the vertex scalp of a black woman, which was presumed to be secondary to use of a thioglycolate permanent wave preparation. We describe the clinical and histologic features of this chemical and traumatic alopecia as well as that of frontotemporal chronic traction alopecia resulting from use of sponge rollers. Common hair care practices are a frequent cause of alopecia in black women. Familiarity with these hair practices will aid in the accurate diagnosis, prevention, and proper management of this problem. The evaluation, causes, and differential diagnosis of alopecia in a black woman are discussed.     

Familial sea-blue histiocytosis with cutaneous involvement. A case report with ultrastructural findings

A 25-year-old man presented with the sea-blue histiocyte syndrome. In addition to hepato-splenomegaly, lung infiltrate and bone marrow involvement, he had cutaneous nodules and plaques over his face, and thickened eyelids. Typical sea-blue histiocytes were found in the skin biopsy. Electron microscopy showed cytoplasmic bodies revealing a peculiar pleomorphic structure. A sister suffered from the same disease. This is the first familial case of sea-blue histiocyte syndrome showing cutaneous involvement.     

Generalized perforating granuloma annulare: a case report and review of the literature.

A nineteen year old girl with mongolism and generalized perforating granuloma annulare of the lower legs is presented herein. Skin biopsy specimens showed dermal necrobiosis and epidermal perforation. The literature is reviewed and summarized. The lesions have been characterized as small, often umbilicated papules occurring in large numbers on the extremities.     

Congenital granular-cell neoplasms. An unusual case report with ultrastructural findings and a review of the literature

Congenital granular-cell tumors are uncommon benign neoplasms of unknown cause that develop primarily in gingival tissue and usually do not recur after surgical excision. Untreated, these neoplasms either cease to grow or "spontaneously" regress after birth. In this report, we describe an unusual case of a child with several congenital granular-cell neoplasms that arose on the lips and continued to increase in size as the child grew. Histologic examination of the neoplasms revealed them to be composed of granular and mesenchymal cells associated with abundant collagen fibers and prominent vascular structures. Ultrastructural study disclosed typical granular cells and a preponderance of immature mesenchymal cells, some of which appeared to be transitional or early granular cells. We interpreted these findings to mean that primitive mesenchymal cells are the proliferative elements and precursors of granular cells in congenital granular-cell neoplasms. The presence of numerous immature mesenchymal cells corroborates the clinical impression that the lesions were growing. Based upon the histological and ultrastructural findings reported here, and a review of the literature, we favor a mesenchymal or endothelial-cell origin for congenital granular cell neoplasms.     

A case of argyria: multiple forms of silver ingestion in a patient with comorbid schizoaffective disorder

Argyria is a rare cutaneous manifestation of silver deposits in the skin, characterized by a grayish blue discoloration, particularly in sun-exposed areas. We report the case of a patient with a history of schizoaffective disorder and type 2 diabetes mellitus who presented with argyria of the face and neck. The patient had a history of ingesting colloidal silver proteins (CSPs) for approximately 10 years as a self-prescribed remedy for his medical conditions. Colloidal silver protein has gained popularity among patients who seek alternative medical therapies. Argyria is the most predominant manifestation of silver toxicity. It is unclear if our patient began taking CSP because of his schizoaffective disorder or if silver toxicity may have induced somatic delusions; however, it is important for physicians to have a thorough understanding of alternative therapies on the market. We present a detailed background on silver ingestion and its systemic effects.     

Aquagenic syringeal acrokeratoderma: report of a case with histologic findings

Aquagenic syringeal acrokeratoderma is a rare acquired condition characterized by painful symmetric swelling and hypopigmentation of the palms and lateral fingers, which develops after brief exposure to water. Histopathologic examination suggests that an aberration in the eccrine sweat gland apparatus may be the underlying cause of this condition. The "hand-in-the-bucket sign," in which patients arrive in their physician's office with their hand in a bucket of water to more readily demonstrate their lesions, is such a common presentation that it almost can be regarded as pathognomonic. All 12 cases reported to date have been in young females. We report a case of aquagenic syringeal acrokeratoderma in a male with unique histologic findings.     

Calcifying aponeurotic fibroma of the knee: A case report with radiological findings

Calcifying aponeurotic fibroma is a rare type of benign tumor that occurs most commonly in the distal extremities of young children. Due to its infiltrative growth, it has a high tendency of recurrence. Although the clinicopathological features of over 100 cases of this rare disease have been reported, its clinical and radiological features have yet to be described in detail. We present a case of calcifying aponeurotic fibroma of the knee from birth with radiological images, that demonstrate the peculiar features of this uncommon benign tumor and discuss its clinicopathological features based on computed tomography and magnetic resonance images.     

Generalized essential telangiectasia: a case report and review of the literature

Generalized essential telangiectasia (GET) is a well-established but seldom reported disorder. Patients with this condition develop widespread telangiectasias for no known reason. Although the condition can be cosmetically and psychologically devastating, it fortunately is not associated with other diseases or complications. Treatment can be difficult and expensive, but the therapeutic benefit of providing patients with a name and prognosis for their disease cannot be overemphasized.