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A V Marzano

Publications and source records attributed to A V Marzano.

At least 19 recordsLinked to original sources
PubMed2005

Keratosis lichenoides chronica and eruptive keratoacanthoma-like lesions in a patient with multiple myeloma.

We describe a 72-year-old woman with a 13-year history of a lichenoid dermatitis, who developed multiple, papular keratoacanthoma (KA)-like lesions and few crater-like nodules on the extremities over a period of 6 months before our observation. Her medical history also recorded multiple myeloma diagnosed a few years before. The long-standing dermatosis was diagnosed, clinically, as keratosis lichenoides chronica (KLC), although, histologically, a lichenoid tissue reaction pattern was not evident. On the other hand, histology from papular and nodular lesions of recent onset was consistent with a possible early phase of KA and spinocellular carcinoma, respectively. Oral acitretin induced regression of KA-like lesions and improvement of KLC but had no effects on crater-like nodules, which required surgical excision. KLC is a chronic disorder of keratinization characterized by lichenoid hyperkeratotic papules arranged in a linear pattern, erythematosquamous plaques and seborrhoea-like dermatitis. We emphasize in our case the association between KLC and multiple possible KAs, never previously reported, and speculate that these two rare conditions may represent here a 'continuum' from a pathogenetic point of view.

Aged↗
PubMed2005

Sclerodermic linear lupus panniculitis: report of two cases.

Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. Unique clinical presentations, such as linear configuration or 'overlap' forms between lupus erythematosus panniculitis and localized scleroderma have been reported. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. The 2 patients (of the 14 patients with lupus erythematosus panniculitis seen by us since 1990) were females with a young age at the onset of disease (median, 25 years). In 1 case, evolution into systemic lupus erythematosus with severe renal involvement occurred whereas the other patient, who had a spontaneous abortion and exhibited anticardiolipin antibodies, should be followed and screened for the emergence of antiphospholipid syndrome. Thus, the clinical behavior of this variant seems to be more aggressive, as compared with the usual course of lupus erythematosus panniculitis, which is considered to be a benign disease, although some reports have suggested that its prognosis is not always favorable. The linear distribution could be the clinical hallmark of such a unique, 'sclerodermic' subset of lupus erythematosus panniculitis.

Adult↗
PubMed2004

Elephantoid oedema of the eyelids.

We describe a male patient with rosacea who had a 2-year history of persistent bilateral oedema of the eyelids, leading to an elephantoid condition with blepharoptosis. An upper eyelid blepharoplasty was performed, but swelling progressively recurred over a few months. Based on the case history, clinical appearance and histological findings, rosaceous lymphoedema was considered to be the diagnosis. The latter is a bilateral, solid oedema of the mid-third of the face, regarded as a rare complication of rosacea. It is thought to occur as a result of chronic inflammation and lymphatic stasis, but its exact aethiopathogenesis remains elusive. Predominant eyelid involvement, causing severe visual impairment as in our patient, is unique.

Aged↗
PubMed2003

Cutaneous infection caused by Salmonella typhi.

Salmonella typhi is a flagellated, gram-negative bacillus belonging to the family Enterobacteriaceae responsible for typhoid fever, which is a prolonged bacteraemic, systemic illness with minimal, at least initially, diarrhoea. Localized infection is a not uncommon complication of salmonella septicaemia, particularly occurring in immunocompromised patients. However, salmonella localization to the skin presenting as cutaneous ulceration is regarded as a rare event. We report a unique case of such a presentation as the sole clinical manifestation of infection with S. typhi and highlight the possible salmonella aetiology for unusual cutaneous lesions in individuals coming from endemic areas.

Adult↗
PubMed2002

Dermatomyositis in 132 patients with different clinical subtypes: cutaneous signs, constitutional symptoms and circulating antibodies.

We retrospectively studied 132 patients with dermatomyositis; 84 had idiopathic, 30 paraneoplastic, 5 juvenile and 13 amyopathic forms of the disease. The commonest features were macular erythema, heliotropic erythema and Gottron's papules. Flagellate erythema occurred in 5% of patients with idiopathic dermatomyositis and correlated with the disease activity. Necrotic lesions were also found in this group of patients but did not always signal malignancy. The prevalence of malignancy was high (23%). Raynaud's phenomenon occurred in 10.6% of patients, also in those with malignancy. Dysphagia, interstitial lung disease and arthralgias affected 20%, 8% and 40% of patients, respectively. Anti-Jo-1 antibodies were found in 5% of patients with idiopathic dermatomyositis and low titre ANA in 1/3 of patients. ANA did not correlate with the disease activity. We confirmed the data from the literature, but no cutaneous sign, constitutional symptom or circulating antibody was found marking a particular subtype of the disease.

Adolescent↗
PubMed2001

Paraneoplastic pemphigus. A report of two cases associated with chronic B-cell lymphocytic leukaemia.

Paraneoplastic pemphigus (PNP) is an autoimmune blistering and erosive mucocutaneous disease associated with neoplasia. Clinical manifestations are polymorphous, and include erythema, bullae, erythema multiforme-like lesions and severe mucous membrane involvement. PNP manifesting as lichenoid dermatitis has recently been observed. We describe two Italian men with fatal PNP featuring typical PNP autoantigens associated with chronic B-cell lymphocytic leukaemia. The first patient presented with an extensive blistering eruption, several erythema multiforme-like lesions and severe mucosal involvement. The second patient presented with a lichenoid dermatitis, then developed bullae, and died with an erythrodermic and exfoliative dermatosis resembling pemphigus foliaceus. Our patients represent two Italian cases of well-documented PNP. In patient 2, the sequence of clinical presentations was unique, and strongly supports the hypothesis of epitope spreading through chronic lichenoid inflammation of the dermo-epidermal junction exposing new self antigens, leading to the humoral response characteristic of PNP.

Fatal Outcome↗
PubMed2001

Clonal CD8 infiltration of the skin in common variable immunodeficiency: a prelymphomatous stage?

Common variable immunodeficiency is a heterogeneous syndrome characterized by hypogammaglobulinemia and recurrent bacterial infections. Patients with this syndrome also have an increased incidence of autoimmune disease and malignancy, most notably lymphoproliferative disorders. The treatment for common variable immunodeficiency is supported by the use of intravenous infusion of immunoglobulins that allows for control of the disease and avoidance of recurrent opportunistic infections. This report describes a young patient having common variable immunodeficiency who presented with a clonal CD8(+) lymphocytic infiltration of the skin clinically manifesting as a widespread papulonodular eruption and resolving with intravenous immunoglobulin therapy. We speculate that such a unique cutaneous presentation may represent a prelymphomatous condition because of the development of an autonomous T-suppressor/cytotoxic cell clone, possibly also responsible for the impaired immunoglobulin production.

Adult↗
PubMed2001

Bilateral laryngeal pseudoparalysis in xanthoma disseminatum treated by endoscopic laser medial arytenoidectomy.

Xanthoma disseminatum is a rare non-Langerhans' cell histiocytosis, characterized by papular cutaneous eruption, possible mucosal involvement, and frequent association with vasopressin-sensitive diabetes insipidus. Herein we report a case of xanthoma disseminatum with pharyngolaryngeal involvement. In this patient, mucosal xanthomas involving the arytenoid cartilages and the interarytenoid area resulted in laryngeal stenosis and severe impairment of both cricoarytenoid joints' motility. Endoscopic CO2 laser medial arytenoidectomy, according to the technique described by Crumley (1993), and vaporization of interarytenoid xanthomas were successfully performed, thus reestablishing bilateral cordal motility and the laryngeal airway. Four years later, a CO2 laser revision was necessary because of recurrence of xanthomas in the posterior larynx. Two years after the latter operation, the patient has no signs of laryngeal obstruction and has a normal voice quality. This case report suggests that endoscopic medial arytenoidectomy may be successfully used in the treatment of bilateral laryngeal pseudoparalysis secondary to xanthoma disseminatum.

Adult↗
PubMed2000

Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases.

BACKGROUND: Cytophagic histiocytic panniculitis (CHP) is a rare subtype of panniculitis that usually follows a fatal course, with a terminal hemophagocytic syndrome. Recent reports on a subset of peripheral T-cell lymphoma named subcutaneous panniculitis-like T-cell lymphoma (SPTL) raised the question about the relationship between these entities. OBSERVATIONS: We describe 7 patients in the study: 1 with fatal CHP, 4 with SPTL, and 2 with long-term CHP. The 5 patients with fatal CHP and SPTL died of complications of hemophagocytic syndrome, with a disease duration ranging from 8 to 74 months. The other 2 patients were still alive 6 and 41 years after disease onset. Immunohistochemical results proved that 2 of the SPTL cases were type alpha/beta and expressed the cytotoxic/suppressor antigen CD8, while the other 2 were type gamma/delta and were positive for the natural killer-associated antigen CD56. In these 4 cases, molecular biology studies by polymerase chain reaction detected T-cell receptor gamma gene rearrangement, indicating a clonal process. In contrast, in the 2 patients who had long-term CHP, the polymerase chain reaction results failed to disclose clonality. In the subject with fatal CHP, genotypic analysis was not performed. CONCLUSION: Our observations suggest that CHP and SPTL may span a clinicopathologic spectrum in which there is a natural disease progression from CHP to SPTL.

Adolescent↗
PubMed2000

Cutaneous infection caused by Serratia marcescens.

An 86-year-old woman presented with a chronic granulomatous skin lesion on the dorsal aspect of her left hand. Histologic examination showed pseudoepitheliomatous hyperplasia and a dense dermal infiltrate largely composed of lymphocytes and histiocytes. Abscess formation and fibroblastic proliferation were also present. Use of Fite, Giemsa, and periodic acid-Schiff stains did not show specific organisms. The gram-negative bacillus Serratia marcescens was the only microorganism isolated from all cultures performed. Trimethoprim-sulfamethoxazole, 960 mg every 12 hours for 20 days (orally), was given and resulted in complete disappearance of the lesion and negative culture findings. Cutaneous infection by S marcescens may represent a distinctive entity, whose clinical and possible pathogenic features are presented here.

Aged↗
PubMed1999

Primary cutaneous B-cell lymphoma with a dermatomal distribution.

Primary cutaneous B-cell lymphomas (CBCLs) are indolent lymphoproliferative skin disorders with the only exception of the variant arising on the legs, which is characterized by a more aggressive clinical course. We report a case of CBCL unusual in that it presented with a dermatome-like distribution and showed early peripheral lymph node involvement. The other noteworthy finding is that rearrangement of the bcl-2 gene was detected by polymerase chain reaction in skin tissue samples. The bcl-2 rearrangement, which is usually absent in the classic CBCLs, is likely to play a role in the aggressive clinical behavior of the tumor, although further studies are necessary to confirm it. The hypothesis of a virus causing the dermatomal presentation is intriguing but there is no proof for such speculation at this point.

Female↗
PubMed1999

Lupus erythematosus with antiphospholipid syndrome and erythema multiforme-like lesions.

The occurrence of erythema multiforme (EM) in patients with lupus erythematosus (LE) has been described previously as a coincidental association. In contrast, LE with EM-like lesions and a peculiar immunological pattern, including positive rheumatoid factor, antinuclear antibodies and a serum antibody against an extract of human tissues recently recognized as similar to Ro (SSA), constitutes an established entity named Rowell's syndrome. We describe a woman with LE and long-standing widespread vesiculobullous and necrotic haemorrhagic EM-like lesions in combination with Ro (SSA) and scl-70 antibodies and the typical laboratory findings of the antiphospholipid syndrome (APS), namely lupus anticoagulant, anticardiolipin antibodies and prolonged activated partial thromboplastin time. This case could conceivably be consistent with a diagnosis of Rowell's syndrome, if the latter is regarded as a clinicopathological spectrum. However, the coexistence of LE, persistent EM-like disease and incomplete APS may also fulfil the diagnostic criteria for the 'multiple autoimmune syndromes'. We speculate that the laboratory markers of APS play a pivotal part in such an unusual clinical presentation.

Adult↗
PubMed1999

Dystrophic calcinosis cutis in subacute lupus.

Dystrophic calcinosis cutis is known to be associated with various connective tissue disorders but to the best of our knowledge has never been reported in subacute cutaneous lupus erythematosus (SCLE), a distinctive cutaneous subset in the spectrum of lupus erythematosus. It occurs without calcium and phosphorus metabolic abnormalities and may be localized or generalized. We report a patient with SCLE who developed calcinosis cutis and had normal serum calcium and phosphorus levels and, interestingly, a normal concentration of blood ionized calcium. This latter, which represents the active form in the total amount of blood calcium, is a parameter only rarely assessed in patients with dystrophic calcinosis cutis. Thus, other pathogenic factors should be investigated to clarify the pathophysiology of the dystrophic type of calcification.

Calcinosis↗
PubMed1999

Unilesional follicular mycosis fungoides.

Follicular mycosis fungoides (FMF) is an unusual clinical and histological variant of MF, characterized by selective involvement of hair follicles by atypical lymphocytes. We describe a female patient who had follicular papules located only on the medial aspect of her right thigh. To the best of our knowledge, this patient represents the first reported case of unilesional FMF.

Diagnosis, Differential↗