Cutaneous bacillary angiomatosis in a patient with chronic lymphocytic leukemia.

BACKGROUND: Bacillary angiomatosis is a recently described vascular disorder that is associated with infection by Bartonella henselae (formerly known as Rochalimaea henselae) and Bartonella quintana (formerly known as Rochalimaea quintana); this disorder usually occurs in patients with human immunodeficiency virus infection. We report a case of cutaneous bacillary angiomatosis that occurred in a patient with chronic lymphocytic leukemia. OBSERVATIONS: A 55-year-old man with chronic lymphocytic B-cell leukemia, Rai stage IV, presented with multiple angiomatous papules that clinically resembled pyogenic granulomas. Histopathologic examination revealed circumscribed lobules of small vessels with plump endothelial cells, numerous neutrophils, and abundant nuclear dust; these features were diagnostic for bacillary angiomatosis. The diagnosis was confirmed by the Grocott-Gomori methenamine-silver nitrate stain that revealed argyrophilic bacteria and by ultrastructural demonstration of bacillary structures with trilaminar walls. Treatment with clarithromycin led to complete resolution of the lesions within 4 weeks. CONCLUSIONS: This case emphasizes that (1) bacillary angiomatosis must be considered in the differential diagnosis of vascular lesions in immunocompromised patients without human immunodeficiency virus infection, (2) Grocott-Gomori methenamine-silver nitrate stain is a simple and satisfactory alternative to the Warthin-Starry stain for the demonstration of bacilli in this condition, and (3) clarithromycin is an effective oral antibiotic for the treatment of this disease.

Ex vivo application of delta-aminolevulinic acid induces high and specific porphyrin levels in human skin tumors: possible basis for selective photodynamic therapy.

In photodynamic therapy with topically applied delta-aminolevulinic acid porphyrins are acting as photosensitizers. The profile of porphyrin metabolites in normal or in neoplastic skin after administration of delta-aminolevulinic acid has not been determined in detail yet. Thus, to study porphyrin biosynthesis in human skin an organ culture model was developed. Explant pieces of normal skin, keratoacanthoma, and basal cell carcinoma were incubated with 1 mM delta-aminolevulinic acid for 36 h. Levels of delta-aminolevulinic acid, porphyrins and porphyrin metabolites were measured in tissues and supernatants. After incubation with delta-aminolevulinic acid, higher porphyrin levels were demonstrated in tumors as compared to normal skin. In supernatants, most of formed porphyrins, preferentially highly carboxylated porphyrin metabolites, were measured. The pattern of synthesized porphyrins differed between normal and neoplastic skin explants. In tissues of basal cell carcinomas protoporphyrin was preferentially shown and tissues of keratoacanthomas were characterized by a predominance of coproporphyrin as compared to normal skin. The results show that explant cultures offer an easy approach to examine the porphyrin biosynthesis of various tissues. The tumor-specific delta-aminolevulinic acid metabolism indicates additional porphyrin metabolites such as coproporphyrin apart from protoporphyrin as effective photosensitizers and may offer a novel approach to tumor-selective photodynamic damage.

Barium oxoaluminate hydride.

The crystal structure of Ba(3)(AlO(4))H is isotypic with Ba(3)SiS(5) and contains AlO(4)(5-) and H(-) anions. The hydride and oxide anions are coordinated by six Ba and five Ba/one Al atoms in an octahedral geometry. The hydrogen content was examined by MAS-NMR experiments of the deuterated compound.

[Hyperpigmentation in primary adrenal cortex insufficiency: Addison disease]. / Hyperpigmentierung bei primärer Nebennierenrindeninsuffizienz: Morbus Addison.

Diffuse hyperpigmentation of the skin may develop without preceding inflammatory skin disease or be associated with various inflammatory disorders. The differential diagnosis of the diffuse hyperpigmentation is complex and difficult. We present a 36-year-old woman with diffuse hyperpigmentation caused by adrenal insufficiency, with special reference to the diagnosis and differential diagnosis of hyperpigmentation associated with endocrine disorders. In addition, metabolic, toxic, nutritional and internal factors and the skin-associated diseases leading to hyperpigmentation are categorized. A classification of diffuse hyperpigmentation is presented.

Supravenous hyperpigmentation in association with CHOP chemotherapy of a CD30 (Ki-1)-positive anaplastic large-cell lymphoma.

Pigmentary aberrations are well known side effects of cytostatic chemotherapeutic agents. We report an unusual pattern of supravenous hyperpigmentation occurring after CHOP chemotherapy.

[Cyclosporin A therapy in pyoderma gangraenosum]. / Cyclosporin-A-Therapie bei Pyoderma gangraenosum.

A 33-year-old woman with pyoderma gangrenosum was treated with cyclosporine A. Cyclosporine A is a valuable drug for the treatment of recalcitrant pyoderma gangrenosum. Its mechanism of action is discussed. The numerous side-effects require close monitoring of cyclosporine A blood levels and several other parameters.

[Cutaneous apocrine mixed tumor with follicular differentiation]. / Apokriner kutaner Mischtumor mit follikulärer Differenzierung.

An 85-year-old man presented with a firm, papillomatous nodule on the upper lip, which had been growing slowly for 30 years. Histopathologic examination revealed an apocrine mixed tumour with follicular differentiation. Mixed tumours of the skin are rare benign neoplasms, which are composed of different tissue components, e.g. epithelial and glandular elements and myxoid or chondroid components. Mixed tumours with apocrine differentiation can be discriminated from mixed tumours with eccrine differentiation. Criteria for apocrine differentiation are: decapitation secretion, elongated gland-like and duct-like structures lined with two rows of epithelial cells and branching of tubular structures. Follicular structures are another clue to the apocrine differentiation of neoplasms. The designation mixed tumour is preferable to chondroid syringoma, because most mixed tumours show apocrine differentiation. The differential diagnosis of apocrine mixed tumour includes fibroadenoma, hidradenoma, mucinous adenocarcinoma, adenoid cystic carcinoma, and rare soft tissue tumours like the myxoid chondrosarcoma.

Barium antimonide oxide, Ba3Sb2O.

The antimonide oxide Ba(3)Sb(2)O consists of discrete [Sb(2)](4-) and O(2-) anions, and crystallizes with a new structure type. The Sb-Sb distances are comparable to those known from electron-precise zintl phases and the tetrahedral coordination of the O(2-) anion is also observed in some other Ba-rich metallide oxides.

[Purpura fulminans with extensive skin necroses]. / Purpura fulminans mit ausgedehnten Hautnekrosen.

Purpura fulminans must be treated as an emergency in internal medicine and dermatology. Its characteristic features are the sudden development of progressively enlarging haemorrhagic skin necrosis, severe disseminated intravascular coagulation with consumption of anticoagulant factors, and signs of shock. Purpura fulminans can be classified into a neonatal form with inherited protein C deficiency and an acquired type for which multiple causes are known. Clinically it is characterized by massive ecchymosis with haemorrhagic blebs and acral necrosis. Histologically the lesions show widespread extravasation of erythrocytes and thrombosis of small vessels. Thrombocytopenia, decrease of coagulation factors, the presence of fibrinogen split products and fragmented erythrocytes in the blood smear help to confirm the diagnosis. The therapy includes fresh-frozen plasma, heparin, antibiotics and surgical debridement of necrotic areas. It is important to recognize the disease promptly because the mortality rate is about 30-40% and only quick intervention helps to save the life of the patient.

Porphyrin concentrations in various human tissues.

We measured the concentrations of total porphyrins and their metabolites (uro-, hepta-, hexa-, penta-, copro- and protoporphyrin) in various human tissues: liver, erythrocytes, skin, adipose tissue, and mammary gland. The porphyrin concentrations varied within major limits, e.g., 3.1 +/- 2.3 nmol porphyrins/g liver and 0.50 +/- 0.10 nmol/g erythrocytes. No significant differences were detectable in other tissues in comparison with liver. In all tissues, the predominant metabolite was protoporphyrin, followed by coproporphyrin, whereas only low concentrations of higher carboxylated porphyrins such as uroporphyrin were detectable. It is concluded that porphyrin metabolism and its regulation is similar in all human tissues, perhaps with some small differences in the erythrocytes.

Neutrophilic dermal infiltrates in granulocytopenic patients with acute leukemia.

Acute febrile neutrophilic dermatosis (AFND, Sweet's syndrome) is clinically characterized by fever, neutrophilic leukocytosis, and tender dermal plaques. Histological examination typically reveals infiltration of the dermis by neutrophils. In three patients (2 female, 1 male, 54-59 years) with acute leukemia (2 myelogenous, 1 lymphoblastic) dermal plaques developed during febrile episodes in chemotherapy-induced pancytopenia. The clinical appearance was compatible with AFND. The diagnosis was substantiated by skin biopsies which showed dense neutrophilic dermal infiltrates without leukemic cells. Leukocytoclastic vasculitis was considered as differential diagnosis. Plasma levels of soluble adhesion molecules ICAM-1, VCAM-1, and E-selectin regulating leukocyte transendothelial migration were in the normal range. Systemic glucocorticoids were avoided because of the high risk of infection during prolonged bone marrow aplasia. The lesions were treated with topical steroids and resolved without scarring within 1-5 weeks. AFND has been reported in association with acute leukemia at normal or elevated white blood cell counts. Although implausible from a pathophysiological point of view, similar neutrophilic dermal infiltrates were found in three patients during chemotherapy-induced pancytopenia with white blood cell counts distinctly below 1 x 10(9)/l.

[Low dosage cyclosporin A therapy in pyoderma gangrenosum. Experiences with 6 patients]. / Niedrig dosierte Cyclosporin-A-Therapie bei Pyoderma gangraenosum. Erfahrungen bei 6 Patienten.

Pyoderma gangraenosum can cause great therapeutic problems. High dosed corticosteroids are the treatment of choice. However, recalcitrant pyoderma gangraenosum or side effects from corticosteroid treatment may require therapeutic alternatives. Pyoderma gangraenosum responds excellently to treatment with cyclosporine A. Because of side effects and drug interactions, the patients must be selected and carefully and closely monitored. Six patients with pyoderma gangraenosum, unresponsive to various topical and systemic therapies, were treated with oral cyclosporine A at mean daily doses of approximately 3 mg/kg. Marked improvement of the skin lesions and complete healing occurred in all patients over a period of 3-6 months. Only one patient suffered a relapse after discontinuation of the treatment. No severe irreversible side effects occurred. The results show that low-dose cyclosporine A treatment can be considered a first-line treatment of pyoderma gangraenosum.

[Angioimmunoblastic lymphadenopathy accompanied by Duhring disease-like lesions ]. / Angioimmunoblastische Lymphadenopathie begleitet von M.-Duhring-ähnlichen Effloreszenzen.

A 62-year-old female patient presented with bullous, intensely itching cutaneous lesions, which clinically and histopathologically resembled dermatitis herpetiformis (Duhring's disease). Therapy with DADPS was unsuccessful. Because of associated cervical lymph node enlargement and splenomegaly, a lymph node biopsy was taken. Histopathology of a lymph node and of the bone marrow confirmed the diagnosis: angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma. Intensely pruritic associated skin eruptions are typical for this peculiar kind of lymphoma. These skin lesions are due to inflammatory cells and not to neoplastic infiltrations. This case report is the first report of AILD with bullous skin lesions to appear in the literature.