Maria Stefanidou

Dermatological Manifestations in Primary Biliary Cirrhosis Patients: A Case Control Study

OBJECTIVES:Primary biliary cirrhosis (PBC), a disease of probable autoimmune etiology that affects the small intrahepatic bile ducts of mainly middle-aged women is commonly associated with pruritus, xanthomatous lesions, and melanosis. We conducted a prospective study to systematically describe the skin disorders of a group of PBC patients.METHODS:A prospective evaluation and analysis of dermatological manifestations including oral and genital lesions was carried out, in 49 PBC patients (45 females and 4 males). Median age 63 yr (range 35–87 yr). They were compared with 45 age and sex matched controls, selected among persons attending the dermatologic outpatient clinic.RESULTS:A total of 330 skin disorders were found in the 49 PBC patients versus 76 in the 45 controls; 31.5% of all lesions were skin fungal infections. Of all lesions analyzed with the Bonferonni rule of multiple comparisons significantly more common in PBC patients were plantar mycoses, onychomycoses, and interdigital mycoses. Pruritus was found in 69.3% of patients versus 22.2% of controls, xerosis in 69.3%versus 2.2%, dermographism in 57.1%versus 4.4%, and melanosis in 46.9%versus 0%. In 38.7% of the PBC patients the dermatologic lesion was the presenting symptom.CONCLUSIONS:PBC patients present with a wide variety of cutaneous manifestations varying in severity. Multiple skin fungal infections have been found even in the early stages. Since in more than one third of our PBC patients the dermatologic lesion was the presenting sign or symptom leading to diagnosis we believe that physicians should be aware so that a prompt and early diagnosis may be achieved.

Amicrobial Pustulosis of the Folds

Amicrobial pustulosis (AP) is a recently defined entity associated with connective tissue diseases. Few cases have appeared in the literature. We report a case of AP coexisting with a systemic lupus erythematosus-scleroderma overlap syndrome and marked photosensitivity. The patient presented prominent pustular skin lesions and a few discoid lupus ones. No significant differences in the inflammatory infiltrate were found between the two clinical variants. The infiltrate consisted mainly of CD4+ lymphocytes and many neutrophils. CD1a+ dendritic cells were few in both epidermis and dermis. AP introduces a potential source of diagnostic confusion, but increasing experience of this syndrome will improve the awareness and diagnostic potential among dermatologists.

Modular diffuse reflection and fluorescence emission imaging colorimeter for the in-vivo study of parameters related to the phototoxic effect in PDT

The biological response to PDT depends on the photosensitizer accumulation to the malignant cells, the tumor vasculature, the inherent cell sensitivity to the photodynamic effect, etc. The in-vivo measurement of physical parameters related with these factors can serve in the understanding of the mechanisms involved in the cell death, as well as in the optimization of the treatment procedure. In this paper we demonstrate a modular diffuse reflection and fluorescence emission imaging colorimeter. We have used it for the in-vivo on-line quantitative measurement and mapping the time course fluorescence intensity after ALA application and for the tissue color changes associated with the erythema developed during irradiation. Based on the obtained quantitative data, we investigate the correlation between the photosensitizer accumulation before light irradiation and the hemodynamic changes, revealed as erythema development, occurring during light irradiation. Their relative significance as predicting factors for the photodynamic treatment effectiveness is also evaluated.

Invasive cutaneous infection with Geotrichum candidum – sequential treatment with amphotericin B and voriconazole

A rare case of an invasive cutaneous infection by Geotrichum candidum in an 80-year-old male patient with diabetes mellitus is reported. The primary site of infection manifested after trauma as an ulcerative lesion on the distal phalanx of the midfinger and extended throughout the right hand. Histological examination showed fungal invasion in the deep dermis without vascular involvement and G. candidum was grown in cultures from the biopsy material. Angiography revealed severe obstructive disease of the right brachial artery and its branches. Treatment, after susceptibility testing of the isolated strain, consisted of sequential administration of intravenous liposomal amphotericin B with oral voriconazole followed by liposomal amphotericin B, resulting in substantial improvement of the infection.

Nevus lipomatosus cutaneous superficialis (Hoffmann-Zurhelle) with localized scleroderma like appearance.

An otherwise healthy 56‐year‐old woman presented with three symptomless unilateral plaques extending from the right lower lip to the submandibular region and the posterior lateral cervical area. Initially, one year previously, a solitary 2 × 3 cm plaque appeared with irregular distinctive borders that extended progressively. Two new plaques with the same characteristics developed slowly near the initial one within six months. Their color was ivory‐pale, with a hyperpigmented margin. The lesions were primarily soft but later became firm and indurated with smooth, shiny, mild scaling, and wrinkled surfaces. A unilateral distribution with no contraction of the skin over the affected area was observed ( Fig. 1 ).

Cutaneous alternariosis in a patient with idiopathic pulmonary fibrosis

A 78‐year‐old farmer presented with symptomless skin lesions for evaluation. Two years prior, he had developed idiopathic pulmonary fibrosis (IPF) and had been treated thereafter with oral prednisolone 20 mg/day and occasionally with colchicine 1 mg/day.

Primary cutaneous nocardiosis in 2 patients on immunosuppressants

Two female cases of primary cutaneous nocardiosis due to Nocardia brasiliensis are described. The first was associated with polymyositis and the second with chronic immune thrombocytopenic purpura. Both patients had received corticosteroids. In both cases the responsible actinomycetes were sensitive to trimethoprim/sulfamethoxazole. This drug was administered to both patients with excellent results. Treatment was continued for 3 months to prevent recurrence, a common consequence of short-term therapy. N. brasiliensis should be included in the differential diagnosis of any case of nodular lymphangitis, especially in immunocompromized patients.

Photodynamic treatment of skin malignancies with aminolevulinic acid: Emphasis on anatomical observations and in vivo erythema visual assessment

background Photodynamic therapy with &dgr;‐aminolevulinic acid is a promising alternative treatment for superficial skin Malignancies. objective Further clinical experience, study of tissue alterations leading to recovery, and correlation/prediction of the therapeutic response through in vivo skin color changes as represented by erythema development. methods The therapeutic procedure, sequential histology and histochemistry, and the development of a remote machine vision system to measure, map, and monitor the erythema development. results/conclusions A high cure response rate with adequate follow‐up was shown. A significant correlation of the clinical‐histologic response of tumors subjected to treatment with the erythema measurements implies that erythema inspection and quantitative analysis offer a reliable predictor of the therapeutic outcome and a clue for optimization of this treatment Sociality.

A rare case of leishmaniasis recidiva cutis evolving for 31 years caused by Leishmania tropica

A 64‐year‐old woman presented with erythematous, infiltrative plaques with a central atrophic area on both zygomatic regions. Several yellow‐reddish papules were seen at the periphery of the plaques and showed an “apple‐jelly” color on diascopy ( Fig. 1 ). No visceral involvement was detected.

Chronic lymphocytic leukemia presenting as cutaneous and bone involvement

An 84‐year‐old man had a 3‐year history of a progressive, painless, papulonodular eruption, that was particularly prominent on the face and extremities. Physical examination revealed firm, bluish‐red nodules and plaques, located on the tip of the nose, the cheeks, ears, and distal digits. Skin lesions produced a leonine facies ( Fig. 1 ), deformities of the fingers and toes, finger clubbing, and onyxis. An identical lesion was seen on a postoperational scar on the left cheek. The mucous membranes were spared. The patient had anterior and posterior cervical and bilateral axillary lymphadenopathy and splenomegaly.