Previous tuberculosis, hepatitis C virus and lichen planus. A report of 10 cases, a causal or casual link?

We report 10 cases of lichen planus (LP) and chronic liver disease linked to HCV. The mean age was 63.4 +/- 5.1 years (range 51-73), five were female; six patients had an established cirrhosis of the liver, as shown by either a liver biopsy or the ultrasonographic and biohumoral evidence. The remaining four patients had chronic hepatitis. Histological examination confirmed the presence of LP: the localization of the dermatosis was restricted to the skin in four patients, to the mucous membranes in five (4 atrophic erosive and one erosive) while the remaining had mucous-cutaneous localization. A type II cryoglobulinemia was demonstrated in two and a type III in one of the patients, while no one had otherwise circulating autoantibodies (anti-nuclear, anti-smooth muscle, anti-liver kidney microsomal type 1 and anti-mitochondrial antigens) such as other etiological factors of liver disease. In six of the patients the history was positive for previous Mycobacterium tuberculosis infection. In clinical practice the patients with chronic liver disease and HCV infection can also suffer from severe extrahepatic manifestations, including lichen planus.

Exhaled nitric oxide in systemic sclerosis: relationships with lung involvement and pulmonary hypertension.

OBJECTIVE: To measure nitric oxide (NO) concentration in exhaled air of patients with systemic sclerosis (SSc) and to investigate its relationships with lung involvement, complicated or not by pulmonary hypertension (PH). METHODS: Exhaled NO was measured by chemiluminescence in 47 patients with SSc (16 with PH) and in 30 controls. All the patients underwent Doppler echocardiography to assess pulmonary artery pressure (PAP), lung function tests, and thin section computed tomographic scans of the lung to quantify the extent of fibrosing alveolitis. RESULTS: Exhaled NO levels were higher in patients with SSc (16.6 +/- 9.1 ppb), particularly those with interstitial lung disease (ILD) (18.3 +/- 10.4 ppb), compared to controls (9.9 +/- 2.9 ppb; p < 0.0001). In patients with PH, exhaled NO was less than in patients without PH (10.7 +/- 5.9 vs 19.6 +/- 9 ppb, respectively; p < 0.001), and patients with PH without ILD had even lower exhaled NO than patients with PH and ILD (6.6 +/- 1.1 vs 12.6 +/- 6.3 ppb; p = 0.004). There was an inverse correlation between PAP and exhaled NO (r = 04).53, p = 0.004). Exhaled NO was not correlated to age, disease duration, current therapy, or form of disease (limited or diffuse). CONCLUSION: The increased concentration of exhaled NO in patients with SSc may reflect respiratory tract inflammation. The relatively low value of exhaled NO in patients with PH and the negative correlation between PAP and exhaled NO suggest the important role of NO in regulating pulmonary vascular resistance in patients with SSc.

Update on mucous membrane pemphigoid: a heterogeneous immune-mediated subepithelial blistering entity.

Most oral involvement in the skin diseases (dermatoses) is related to mucous membrane pemphigoid or lichen planus. Mucous membrane pemphigoid was the subject of a European Symposium held in Turin, Italy, in June 1997. This review is based on that symposium. Mucous membrane pemphigoid is a subepithelial vesiculobullous disorder mainly of late middle age; it has a slight predilection for women. Whereas mucous membrane pemphigoid was formerly considered a single entity, it is now quite evident that a number of subepithelial vesiculobullous disorders may produce similar clinical pictures and also that a range of variants of mucous membrane pemphigoid exists, with antibodies directed against various hemidesmosomal components or components of the epithelial basement membrane. The term immune-mediated subepithelial blistering diseases has therefore been used. Diagnosis and management of immune-mediated subepithelial blistering diseases on clinical grounds alone are impossible; a full history, general and oral examinations, and biopsy with immunostaining are now invariably required, sometimes supplemented with other investigations. Most patients with mucous membrane pemphigoid affecting the mouth manifest desquamative gingivitis, a fairly common complaint typically seen in women who are middle-aged or older. Oral vesicles and erosions may also occur, and there can be a positive Nikolsky sign. Some patients have lesions of other stratified squamous epithelia, presenting as conjunctival, nasal, oesophageal, laryngeal, vulval, penile, or anal involvement. Apart from improving oral hygiene, immunomodulatory-in particular, immunosuppressive-therapy is typically required to control oral lesions in mucous membrane pemphigoid. No single treatment regimen reliably controls all these disorders.

[Chronic HCV hepatopathy and cryoglobulinemia. The associated clinical spectrum]. / Epatopatia cronica da HCV e crioglobulinemia. Lo spettro clinico associato.

BACKGROUND AND AIM: The hepatitis C infection (HCV) has numerous extrahepatic manifestations owing to the systemic nature of the infection itself. HCV infects the cells that carry a CD 81 receptor and show a marked tropism for hepatocytes, bone marrow staminal cells and circulating lymphomonocytes. One consequence of this tropism is the activation of B lymphocyte clones with the consequent production of autoantibodies and cryoglobulins. The secondary event is the formation of circulating immune complexes which, having precipitated at an intravascular level, may cause part of the extrahepatic manifestations associated with these infections. METHODS: This retrospective study evaluated the manifestations correlated and/or associated with HCV hepatitis and mixed cryoglobulinaemia. RESULTS: This analysis showed that 75% of consecutively studied patients reveal clinically important extrahepatic manifestations. CONCLUSIONS: This underlines the "broad spectrum" action played by the hepatitis C virus in the host organism.

Multiple pyogenic granuloma of the penis.

A case of multiple pyogenic granuloma affecting the penis of a 28 year old man is reported. The lesions were arranged in a floret-like fashion around the inner aspect of the prepuce and developed after circumcision for congenital phimosis. Histopathological examination of sections from a biopsy specimen of the papillomatous growths revealed the findings of pyogenic granuloma. In this patient, the pathogenesis of the lesions is probably related to the failure in surgical wound repair that followed circumcision. Problems of clinical and histopathological differential diagnosis are discussed.

Dermoid cyst of the penis.

A dermoid cyst of the penis has not been documented previously in the literature. We report a young Caucasian who had an important swelling of the penis dating back several months. On examination a subcutaneous mass on the dorsal aspect of the shaft and prepuce was present. A cutaneous sinus discharging purulent material was also noted above the mass. Laboratory tests including serology for syphilis and bacteriologic examination were negative. An excisional biopsy revealed a nodular lesion in the subcutaneous tissue with tufts of hair emerging from the surface. Histopathologic examination disclosed granulation tissue containing fragments of hair surrounded by granulomatous infiltrate. Serial sections allowed identification of a subcutaneous cyst. The wall was epidermoid and connected with several pilosebaceous units. A sinus tract lined by stratified squamous epithelium contiguous with the epidermis was also noted. Clinical and histopathologic differential diagnoses are discussed. Evidence for a congenital origin of this condition is given.

Esophageal dysfunction in scleroderma: relationship with disease subsets.

OBJECTIVE: To investigate the relationship between esophageal function and the extent of disease in a nonselected group of scleroderma patients, and to study gastric and small bowel motility in a group of scleroderma patients with more severe clinical manifestations. METHODS: Esophageal function in 125 scleroderma patients was investigated by radiologic, endoscopic, manometric, and pH-metric techniques. Ten patients also underwent gastrointestinal (GI) manometric recording, both during fasting and after a standard meal. RESULTS: Radiologic abnormalities of the esophagus were found in 55 of 81 patients (68%) and esophagitis in 45 of 125 (36%). No significant relationship was disclosed between GI symptoms, radiologic abnormalities, esophagitis grade, and the various disease subsets. However, the overall incidence of endoscopic esophagitis (irrespective of the degree) was significantly (P < 0.05) correlated with the patient subgroups, with 100% incidence of esophagitis in those having the more severe cutaneous involvement (type III). Manometric abnormalities were documented in 80% of patients, and pathologic reflux in 78%. The severity of esophageal abnormalities on manometry significantly correlated with the severity of the disease, whereas no correlations were found with pH-metric data. Ninety percent of the 10 female patients undergoing antroduodenal manometry displayed abnormal findings; of these, 60% showed neuropathic, and 30% myopathic, patterns. The latter were recorded in patients with a more severe stage of the disease (type III). CONCLUSION: A direct relationship was observed between scleroderma subsets and the severity of esophageal (and, probably, more distal gut) motor involvement. Since no correlation was found between esophageal symptoms and the severity of manometric abnormalities, manometry should be considered the single most important GI test to document the severity of the "esophageal" disease. Gastric and small bowel manometry may also offer evidence of widespread gut involvement, and provide a rationale for a more targeted therapeutic approach.

Recurrent necroinflammatory disease of multiple organs and colon. Systemic presentation of inflammatory bowel disease or gut involvement during systemic disorder.

A 30-year-old man with a recurrent febrile illness resembling infection is described. Because he presented with an acute abdomen, he underwent a laparotomy, which showed the paraaortic and mesenteric lymph nodes to be changed into an abscess-like granulomatous tissue made up of necrotized granulocytes. During further flare-ups, the disease affected the spleen, skin, colon, peripheral nerve, and muscle. Histology on the biopsy materials of both the skin and colon, and on the surgically removed spleen showed the same invading pathologic tissue. Exhaustive investigation disclosed no pathogen, and the flare-ups responded repeatedly to high-dose steroids. This patient's picture has recently been defined as a syndrome of chronic granulomatosis based on several published cases. As a distinctive feature, in our patient the granulomas affected also the colon. For the present, and for another previously described similar case we analyzed the factors that might permit the differential diagnosis between the above-mentioned granulomatous syndrome and Crohn's colitis.

Recurrent Sweet's syndrome in reactivated Crohn's disease.

A 50-year-old woman developed an acute febrile dermatosis on two occasions concurrently with recurrent Crohn's disease of the colon. Based on the presence of painful erythematous plaques on both hands and forearms, on the leukocytosis with excess bands in peripheral blood, on the histology showing dermal infiltration by mature granulocytes, and on the prompt response to steroids, the diagnosis was made of Sweet's syndrome associated with Crohn's disease. Sweet's syndrome is thought to be a hypersensitivity reaction that leads to parainflammatory (e.g., infections, autoimmune disorders, vaccinations) and paraneoplastic (myeloproliferative disorders, solid malignancy) associations, with a frequency of 10-30%. The association of Sweet's syndrome with Crohn's disease is very rare, but the gastroenterologist should readily differentiate it; it is important to be aware that such patients may have a nonspecific elevated activity index owing to the underlying dermatosis.

What is the role of sensitization in uremic pruritus? An allergologic study.

Patch tests were carried out to evaluate the presence of a sensitization to some components of dialytic circuits in 17 uremic patients complaining of pruritus of unknown origin. Fragments of different dialyzer membranes, of tubing sets, of dialyzer membranes recently resterilized with ethylene oxide and the International Contact Dermatitis Research Group standard series substances were tested. Neither patients nor healthy subjects reacted positively to patch tests, which leads us to question the role of contact allergy in the determination of uremic pruritus.

Serological profiles as prognostic clues for progressive systemic scleroderma: the Italian experience.

Ninety-one patients with progressive systemic sclerosis have been examined both clinically and serologically in order to have a better prognostic insight. Three main serological profiles have been isolated. The patients with anticentromere antibodies (ACA) represented one third of the cases, developed skin sclerosis rather later and rarely exhibited ankyloses and ulcerations. The esophagus was commonly involved while the lung, heart and kidneys were not. ACA-positive patients were not identified with the CREST syndrome, as the latter disclosed other profiles with the same frequency. Patients with anti-Scl-70 antibody represented one fourth of the cases and had the fastest progression, developing sclerosis in less than 5 years after the onset of Raynaud's phenomenon. Ankyloses and lung fibrosis, as well as joint, heart and kidney involvement, were found in most of them. Patients with anti-SSA/Ro antibodies were uncommon, but corresponded to a severe subset, having a fast progression and a constant involvement of the lung. Probably due to the rougher definition of their serology, patients with antinuclear, antispeckle-patterned and anti-Ku antibodies or without any detectable antibody could be defined less easily and corresponded to an intermediate position between ACA- and anti-Scl-70-positive patients. Though it is probably premature to trust it completely, a serological classification may provide the prognostic clues clinical classifications cannot.

Cutaneous tuberculosis: atypical skin lesions in immunodepressed patients.

We report a case of secondary skin tuberculosis due to endogenous secondary infection in a 27-year-old subject affected by ulcerative colitis. The clinical appearance the lesion was atypical and its classification uncertain. The morphology of the lesion and the fact that the primary tubercular complex, at pulmonary level, was masked by a simultaneous candidiasis infection were probably due to cell-mediated immunodeficiency consequent to the ulcerative colitis and on-going therapy (Salazopyrin and prednisone). Rapid remission of cutaneous and pulmonary lesions was achieved following specific therapy (rifampicin, isoniazid, ethambutol).

Sporotrichoid infection. Two cases.

The Authors describe two cases of Sporotrichoid infection characterized by the onset, above previous skin accidental injuries, of a papulo-pustular lesion, with further development of centripetal satellite lesions similar to the first one. A precise etiological definition was possible only after the cultural,l mycological and bacteriological examination of biopsy material. The very similar clinical features of the two cases were due to different etiological pathogens: Sporothrix schenckii in one case and Mycobacterium marinum in the other case. A complete healing of the skin lesion was obtained with Itraconazole in the first case and Rifampin in the second case.

[Paresthetic notalgia. Report of 2 cases]. / Notalgia parestesica. Osservazione di due casi.

The Authors describe two cases of notalgia paresthesica, a disorder with localized pruritus of the back and hypesthesia for touch possibly due to a sensory neuropathy involving the posterior primary rami of thoracic nerve T2 through T6. The condition is not rare, but underdiagnosed for the poor clinical relevance. The possible X-linked hereditary transmission and other pathogenetic hypotheses are discussed.