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Elvio Alessi

Publications and source records attributed to Elvio Alessi.

12 recordsLinked to original sources
PubMed2005

Primary cutaneous follicular center cell lymphoma of the nose with maxillary sinus involvement in a pediatric patient.

We report a primary cutaneous follicular center cell lymphoma (PCFCCL) that arose on the nose and the left naso-labial fold, spread to the left cheek, the left maxillary sinus, and the soft palate in a 16-year-old boy. Polychemotherapy was performed and the patient is disease-free after 41 months. This case is unusual because PCFCCL rarely arises on the nose, only occasionally disseminates to extracutaneous sites, and, to our knowledge, has never been described in pediatric patients.

Adolescent↗
PubMed2005

Fatal CD8+ epidermotropic cytotoxic primary cutaneous T-cell lymphoma with multiorgan involvement.

A 70-year-old woman presented with a 3-month history of two ulcerated erythematous-violaceous nodular lesions over the nose and forehead, respectively. The patient's history included a similar cutaneous nodule on the glabella diagnosed as pseudolymphoma 2 years ago. At that time, despite the diagnosis of a benign disease, an adequate staging was performed, ruling out any extracutaneous involvement. During hospitalization, multiple purpuric papules developed over the abdomen, and the disease spread to mediastinal lymph nodes, lungs and the central nervous system. Based on the histologic, immunophenotypic and molecular biology findings, a diagnosis of CD8+ epidermotropic cytotoxic primary cutaneous T-cell lymphoma was made. Secondary skin involvement by a CD8+ extracutaneous T-cell lymphoma could not be excluded with certainty, but seemed to be unlikely because of the negativity of the initial workup. The patient died from complications of right femoral artery thrombosis before starting specific polychemotherapy 21 months after onset of the disease. Among primary cutaneous T-cell lymphomas, the CD8+ epidermotropic cytotoxic subset comprises rare, highly aggressive forms characterized by metastatic spread to unusual sites such as the oral cavity, lungs, testis and the central nervous system but usually not to the lymph nodes. These cases seem to be distinct from mycosis fungoides with CD8+ phenotype, which shows a nonaggressive clinical behavior.

Aged↗
PubMed2004

Anetodermic lupus panniculitis and antiphospholipid antibodies: report of three cases.

Anetoderma is a rare cutaneous disease characterized by a loss of normal elastic tissue that is presented clinically as localized areas of wrinkled or flaccid skin. This form may be associated with several immunological abnormalities, most notably lupus erythematosus and antiphospholipid antibodies with or without clinical manifestations of the antiphospholipid syndrome. A retrospective study was conducted with the aim of summarizing the clinical characteristics, course and laboratory findings in three women with anetoderma-associated lupus erythematosus panniculitis, an unusual variant of cutaneous lupus erythematosus. The 3 patients (of the 12 patients with lupus erythematosus panniculitis seen by us since 1990) were all at a young age at onset of panniculitis (median, 22 years). None of the patients developed severe systemic involvement up to 9 years (median, 5 years) from onset of the disease. The most noteworthy laboratory finding was the presence of antiphospholipid antibodies. Anetodermic lupus erythematosus panniculitis may be regarded as an uncommon variant of cutaneous lupus erythematosus mainly affecting young females and showing a favourable clinical course, although the patients should be followed and screened for the emergence of antiphospholipid syndrome. Antiphospholipid antibodies could play a role in the elastolytic process, leading to anetoderma.

Adult↗
PubMed2004

Onycholemmal carcinoma.

We report on a slowly growing malignant tumor of the nail bed epithelium in a 69-year-old male. On light microscopic examination, the tumor was composed of: (1) some small cysts filled with eosinophilic, amorphous keratin and lined by an atypical squamous epithelium devoid of a granular layer and (2) solid nests and strands of atypical keratinocytes filling the dermis and penetrating the phalangeal bone. Because the nail bed epithelium is comparable to the outer root sheath, or trichilemma of the hair follicle, and since the reported tumor showed some analogies with trichilemmal carcinoma, we suggest that this entity be designated 'onycholemmal carcinoma'. Disarticulation of the involved phalanx was performed and neither local recurrence nor distant metastasis was observed during 4 years of follow up.

Aged↗
PubMed2004

Review of 120 biopsies performed on the balanopreputial sac. from zoon's balanitis to the concept of a wider spectrum of inflammatory non-cicatricial balanoposthitis.

BACKGROUND: Long-lasting erythematous lesions involving the balanopreputial sac may be clinically difficult to classify, and biopsies may be performed in order to clarify the nature of the disease. However, our previous experience led us to consider several cases as histopathologically unclassifiable. OBJECTIVE: To establish the causes of such unsatisfactory findings. METHODS: We reviewed 120 balanopreputial sac biopsies performed at the surgical unit of our institute from January 1999 to December 2002 examining also the patients' clinical records. RESULTS: We found that a small group of previously unclassified cases showed common clinical and histopathological features. Clinically, all patients were uncircumcised and had long-lasting asymptomatic erythematous plaques on the balanopreputial sac with no erythroplastic or lichenoid features and no correlation with sexual intercourse. Histologically, all specimens showed a thinned and spongiotic epithelium, a band-like infiltrate of lymphocytes and histiocytes with a variable number of plasma cells in the upper part of the chorion and further signs of acute, subacute or chronic inflammation. CONCLUSIONS: In our view, these cases fall within a spectrum of inflammatory non-cicatricial disorders, ranging from almost pure lymphohistiocytic forms to forms which fulfil all criteria to be classified as balanitis circumscripta plasmacellularis of Zoon. We propose to classify these cases histologically as inflammatory non-cicatricial balanoposthitis and clinically as idiopathic inflammatory non-cicatricial balanoposthitis.

Adult↗
PubMed2003

Frequent association between MEN 2A and cutaneous lichen amyloidosis.

OBJECTIVE: Multiple endocrine neoplasia type 2A (MEN 2A) and familial medullary thyroid carcinoma (FMTC) are genetic diseases due to activating mutations of the RET proto-oncogene. Affected patients develop medullary thyroid carcinoma (100%), in an isolated form (FMTC) or in association with phaeochromocytoma (30-50%), and primary hyperparathyroidism (10-20%) (MEN 2A). The presence of cutaneous lichen amyloidosis (CLA) has been anecdotally described in few families harbouring RET proto-oncogene mutation in codon 634. The aim of the study was to evaluate the incidence of CLA in MEN 2A/FMTC families. PATIENTS AND DESIGN: Ten MEN 2A/FMTC families were studied and RET gene mutations identified in all. Complete dermatological assessment was carried out in each family member. Skin biopsy for histological studies was performed in patients with CLA. RESULTS: Among 10 MEN 2A/FMTC families, the presence of CLA was found only in patients belonging to the three families with MEN 2A and RET mutation in codon 634. Nine of 25 patients (36%) with codon 634 mutation presented CLA, though two of them did not show CLA skin lesions but the typical neurological pruritus in the upper back. In all patients, neurological pruritus was present since infancy as a precocious marker of the disorder. The dermatological study of patients with CLA skin lesions added further evidence that pruritus has a pivotal role in the development of CLA, the amyloid deposition being the consequence of repeated scratching. Light microscopy revealed orthokeratotic hyperkeratosis, with elongation of the rete ridges, rare intramalpighian apoptic keratinocytes and deposits of amorphous material in the superficial dermis. Examination under ultraviolet light showed thioflavin T-positive staining, confirming the presence of amyloid in the papillary dermis. The use of Capsaicin at the dilution of 0.025% had a mild efficacy on the cutaneous symptoms. CONCLUSIONS: Among the members of the three families with MEN 2A and RET 634 mutation, the incidence of CLA was 36%, a figure similar to that reported in the literature for phaeochromocytoma (30-50%) and even higher than that for hyperparathyroidism (10-20%). The present data confirm that CLA is linked to codon 634 RET mutations and is a precocious marker of the disorder.

Adolescent↗
PubMed2003

Widespread livedoid vasculopathy.

A 37-year-old woman with a 13-year history of widespread livedo reticularis and recurrent, painful ulcerative skin lesions was referred to our department because of a relapse of cutaneous manifestations of the skin lesions involving almost the whole body surface; malar erythema and oedema, non-scarring alopecia and fever were also associated. Routine laboratory data, immunological investigations and coagulation parameters were normal or negative. Histology was consistent with livedoid vasculopathy. A good clinical response was obtained using intravenous methylprednisolone combined with pentoxifylline. Livedoid vasculopathy is a rare, distinctive dermatosis that can be associated with systemic autoimmune disorders or present in an "idiopathic" form. The latter is at present regarded as a non-inflammatory thrombotic disease that may occur in patients with coagulation abnormalities. It is noteworthy that, in the present case, despite long-standing and dramatic cutaneous features, serious systemic complications have not developed and the patient's seroimmunologic and coagulative profile has remained normal.

Adult↗
PubMed2003

Poikilodermatous subacute cutaneous lupus erythematosus.

BACKGROUND: Subacute cutaneous lupus erythematosus (SCLE) is a distinct subset of lupus erythematosus with unique clinical, immunological and genetic features. Among the unusual variants of SCLE, there is a poikilodermic presentation. However, to date, only 1 case of poikilodermatous SCLE has been reported. OBJECTIVE: Our goal was to summarize the clinical characteristics and course as well as the pathological, laboratory and immunofluorescence findings of 4 patients with poikilodermatous SCLE. METHODS: A retrospective study was conducted including 54 patients diagnosed as having SCLE between 1980 and 2002. RESULTS: Four patients (7.4%) had SCLE. All patients were alive, and none developed severe systemic involvement in up to 36 years (median, 24 years) after the onset of disease. The most noteworthy laboratory finding was the cutaneous deposition of amyloid. CONCLUSION: Poikilodermatous SCLE represents an uncommon variant within the clinicopathological spectrum of SCLE following a favorable course, in spite of extensive cutaneous involvement. Photosensitivity is the pathomechanism explaining, theoretically, the development of both poikiloderma and cutaneous amyloidosis in such cases.

Adult↗
PubMed2002

Primary cutaneous large B-cell lymphoma of the leg: histogenetic analysis of a controversial clinicopathologic entity.

This study analyzes the pathologic and molecular features of 5 cases of primary cutaneous large B-cell lymphoma of the leg (PCLBCL-leg), recently included in the European Organization for Research and Treatment of Cancer (EORTC) classification of primary cutaneous lymphoma. PCLBCL-leg accounts for 5% to 10% of all primary cutaneous B-cell lymphoma (PCBCL), usually affects elderly patients and carries a worse prognosis than other forms of PCBCL. It has been proposed that the malignant cells of PCLBCL-leg originate from germinal center (GC)-related cells, but their effective normal counterpart is unclear, and the rationale behind the inclusion of this lymphoma as a separate entity is based on its prognosis rather than on its proved histogenesis. All of our cases of PCLBCL-leg morphologically resembled diffuse large B-cell lymphoma (DLBCL), but to better define their histogenesis, we also analyzed various phenotypic and genotypic markers, including mutations of the Ig and of BCL-6 genes, as well as expression of the bcl-6, MUM1, and CD138/syndecan-1 proteins. Immunohistochemically, all of our cases stained for the L-26/CD20cy and CD79a antigens and expressed the bcl-2, bcl-6, and MUM-1 proteins but were negative for both the CD10/CALLA and CD138 antigens. With respect to molecular analysis, the lymphoma population of all PCLBCL-leg carried hypermutation of Ig genes, and all but 1 case also harbored mutations of the BCL-6 gene. Our results indicate that PCLBCL-leg are similar both under the morphofunctional and molecular profiles to most DLBCL of other sites. Thus, caution seems justified before definitely considering PCLBCL of the leg as a distinct entity.

Aged↗
PubMed2002

Ichthyosiform mycosis fungoides.

BACKGROUND: Mycosis fungoides (MF) is a skin malignancy of T helper lymphocytes with a wide clinical spectrum. Among the atypical variants of MF, there is an ichthyosis-like presentation. However, to date, only 1 case of ichthyosiform MF has been reported. OBJECTIVE: Our goal was to summarize the clinical characteristics and course, and the pathological, immunohistochemical and molecular genetic findings on 4 patients with ichthyosiform MF. METHODS: A retrospective study was conducted. RESULTS: The 4 patients represented 1.8% of the 221 patients with MF seen by us since 1975. None progressed to systemic disease in up to 12 years (median, 10 years) after the onset of the cutaneous manifestations. Interestingly, skin lesions typical of so-called follicular MF (FMF) were associated in 3 of 4 cases, whereas cutaneous manifestations of classic MF were absent in all 4 patients. CONCLUSION: Ichthyosiform MF represents a rare variant within the clinicopathologic spectrum of MF usually featuring a benign course and a tendency to be associated with lesions of FMF but not with lesions of classic MF.

Adult↗