Relationship between cutaneous and pulmonary involvement in systemic sclerosis.

OBJECTIVE: Pulmonary disease may shorten survival in patients affected by systemic sclerosis (SSc). However, pulmonary involvement may commonly be silent, whereas skin fibrosis is usually the clinical feature drawing most attention. We investigated the relationship between cutaneous and pulmonary involvement during SSc. METHODS: We studied 52 patients (mean age 50.2 +/- 13.7 years) affected by SSc (mean duration of disease 13.8 +/- 9.5 years). Twenty-eight had the diffuse form of the disease (dSSc) and 24 the limited form (lSSc). All patients underwent pulmonary function studies, high resolution computed tomography (HRCT) of the lungs, and complete echocardiographic examination. Pulmonary artery systolic pressure was measured by Doppler echocardiography. Pulmonary interstitial fibrosis and skin fibrosis were evaluated using a point system. RESULTS: Mean percentages of predicted values of forced vital capacity and total lung capacity were significantly reduced in patients with dSSc compared to lSSc (80.0 +/- 18.9 vs 98.4 +/- 16.8%, p < 0.001; and 81.3 +/- 13.9 vs 92.1 +/- 14.2%, p < 0.01, respectively). The overall HRCT score was 6.1 +/- 4.9, with no significant differences between disease subgroups. However, a HRCT score of 10 or more was present in 10 patients with dSSc vs 2 patients with lSSc (p = 0.02). Pulmonary hypertension was present in 27 patients, 15 with lSSC and 12 with dSSc (p = NS). No significant correlation was observed between skin score and lung volumes, carbon monoxide diffusing capacity, HRCT score, or pulmonary artery systolic pressure for all patients and subgroups. CONCLUSION: Extent and severity of cutaneous and pulmonary involvement in SSc are not directly correlated. Nevertheless, different patterns of pulmonary involvement between SSc subgroups were observed. Restrictive lung disease was more frequent in patients with dSSc, while a trend to higher prevalence of pulmonary hypertension was observed in patients with lSSc.

Bacillary epithelioid angiomatosis in acquired immunodeficiency syndrome (AIDS)--clinicopathological and ultrastructural study of a case with a review of the literature.

Bacillary epithelioid angiomatosis (BEA) is a rare cutaneous disorder usually affecting patients with human immunodeficiency virus (HIV) infection often misdiagnosed as a vascular tumour. We describe a 51-year-old, HIV-positive, Caucasian, homosexual male who developed scattered papulo-nodular lesions with clinicopathological and ultrastructural features of BEA. He had a dramatic therapeutic response to systemic antibiotics. There has been a lack of such reports in the European literature. The differential diagnosis is discussed and a brief review of the English literature to date is included.

Kaposi's sarcoma: a clinico-pathologic overview.

A careful overview of the classical appearances of Kaposi's sarcoma (KS) as well as of its variants were reviewed from the clinical and pathological point of view. The growth phases (stages) and the cellular patterns were histopathologically compared with emphasis on the developmental progression of disease as well as mitotic activity. Other morphological aspects were also assessed such as the features of the early phases and the incidence of hyaline bodies. One hundred and forty-three lesions from 96 patients mostly of the Italian sporadic type were investigated. A complete list of those entities which should be considered in differential diagnosis is shown and the dilemma of whether KS is a neoplasia or a hyperplasia is discussed.

Juvenile colloid milium. Immunohistochemical and ultrastructural studies.

A 7-year-old Italian girl with juvenile colloid milium was studied with histological, immunohistochemical, and electron microscopic methods. This patient had a well-documented history of severe sunburn and developed the lesions on the face shortly afterward. Numerous apoptotic keratinocytes were observed in the lower epidermis. These cells began their degeneration with filamentous whorl formation (or filamentous degeneration) of tonofilaments. In the papillary dermis the colloid substance was resolved by the electron microscopy into either wavy, thin filaments derived from the epidermal keratinocytes or typical amyloid filaments. Many desmosomes and gap junctions were found in the colloid substance. Polyclonal antikeratin antibody (DAKO) was positive in the colloid substance, particularly in the parts close to the epidermis. These findings suggested that juvenile colloid milium is different from adult colloid milium despite clinical similarities and that the former belongs to the group of actinic amyloid K, i.e. amyloidoses due to actinic degeneration of keratinocyte and its keratin.

Unusual dermatologic toxicity of long-term therapy with hydroxyurea in chronic myelogenous leukemia.

The unusual appearance of extensive skin ulcerations was observed in 17 patients with chronic myelogenous leukemia on continuous chemotherapy with hydroxyurea. The strict relationship between ulcers and therapy was proved by the complete (14 cases) or almost complete (3 cases) healing of lesions after therapy was discontinued. The possible pathogenetic mechanisms responsible for skin alterations are considered. The particular importance of the continuous hydroxyurea administration modality clearly emerges, suggesting the use of different administration modalities to reduce such serious side effects.