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2.
G Ital Dermatol Venereol ; 149(3): 301-9, 2014 Jun.
Article in English | MEDLINE | ID: mdl-24819757

ABSTRACT

Drug-induced lupus erythematosus (DI-LE) is defined as an entity characterized by clinical manifestations and immunopathological serum findings similar to those of idiopathic lupus but which is temporally related to drug exposure and resolves after withdrawal of the implicated drug. Similarly to idiopathic lupus, DI-LE can be divided into systemic LE, subacute cutaneous LE (SCLE), chronic cutaneous LE (CCLE) and cutaneous LE tumidus. DI-SCLE is the most frequent variant of drug-induced cutaneous LE and presents mainly with annular-polycyclic lesions; the clinical picture is often widespread, with involvement of the lower legs that are usually spared in idiopathic SCLE. ANA and anti-Ro/SSA antibodies are typically present, whereas antihistone antibodies are uncommonly found. We have recently addressed the question whether DI-SCLE differs significantly from its idiopathic counterpart by virtue of clinical features and, based on our findings, we have suggested that the frequent occurrence of malar rash and bullous, erythema multiforme-like and vasculitic manifestations can be regarded as the hallmark of DI-SCLE. In contrast, the histology is not a useful diagnostic criterion for DI-SCLE, considering that the typical pattern of lichenoid interface dermatitis is seen only in the early stage of disease and tissue eosinophilia does not represent a differentiating histopathological feature. DI-CCLE and DI-LE tumidus, albeit possibly misdiagnosed, are rarely observed and are characterized by classic discoid lesions and erythematous-oedematous plaques on sun exposed areas, respectively. Management of DI-LE is based on the discontinuation of the offending drug; topical and/or systemic corticosteroids and other immunomodulating/immunosuppressive agents should be reserved for resistant cases.


Subject(s)
Lupus Erythematosus, Cutaneous/etiology , Lupus Erythematosus, Cutaneous/pathology , Lupus Erythematosus, Systemic/etiology , Lupus Erythematosus, Systemic/pathology , Adult , Aged , Aged, 80 and over , Antibodies, Antinuclear/blood , Biomarkers/blood , Drug Therapy, Combination , Female , Glucocorticoids/therapeutic use , Humans , Immunologic Factors/blood , Immunosuppressive Agents/therapeutic use , Leg/pathology , Lupus Erythematosus, Cutaneous/diagnosis , Lupus Erythematosus, Cutaneous/drug therapy , Lupus Erythematosus, Cutaneous/immunology , Lupus Erythematosus, Discoid/etiology , Lupus Erythematosus, Discoid/pathology , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/immunology , Male , Middle Aged , Torso/pathology , Treatment Outcome
3.
J Plast Reconstr Aesthet Surg ; 66(11): e310-2, 2013 Nov.
Article in English | MEDLINE | ID: mdl-23668953

ABSTRACT

BACKGROUND: Primary cutaneous large B-cell lymphoma, leg type, is a rare and aggressive neoplasm as defined by the World Health Organization/European Organisation for Research and Treatment of Cancer classification of cutaneous lymphomas. In some cases this disease may simulate other forms of benign or malignant solid tumours. MATERIAL: We present a case of a 74-year-old man showing a quickly 'migrant' mass on his forehead. First skin biopsy, ultrasound and magnetic resonance images were not significant. A deeper biopsy revealed a pathology consistent with a primary cutaneous diffuse large B-cell lymphoma leg type. RESULTS: The patient was successfully treated with only local radiotherapy (total dose: 32.4 Gy). At 1-year follow-up there were no recurrences. DISCUSSION: To the best of our knowledge, this is the second case of a primary cutaneous diffuse large B-cell lymphoma leg type developed as a quickly 'migrant' lesion. In contrast with the first report, our case developed in a non-leg site. From these two cases, we should bear in mind that aggressive and quickly migrant cutaneous or subcutaneous masses might mask a lymphomatous disease.


Subject(s)
Forehead/pathology , Lymphoma, Large B-Cell, Diffuse/pathology , Skin Neoplasms/pathology , Aged , Biopsy , Humans , Lymphoma, Large B-Cell, Diffuse/radiotherapy , Male , Skin Neoplasms/radiotherapy
4.
G Ital Dermatol Venereol ; 148(2): 185-96, 2013 Apr.
Article in English | MEDLINE | ID: mdl-23588144

ABSTRACT

Pyoderma gangrenosum (PG) and Sweet's Syndrome (SS) are inflammatory skin diseases caused by the accumulation of neutrophils in the skin and, rarely, in internal organs, which led to coining the term of neutrophilic dermatoses (ND) to define these conditions. Recently, ND have been included among the autoinflammatory diseases, which are forms due to mutations of genes regulating the innate immune responses. Both PG and SS are frequently associated with inflammatory bowel diseases (IBD), a group of chronic intestinal disorders which comprises ulcerative colitis and Crohn's disease and whose pathogenesis involves both the innate and adaptive immunity in genetically prone individuals. Patients with IBD develop PG in 1-3% of cases, while SS is rarer. PG presents with deep erythematous-to-violaceous painful ulcers with undermined borders, but bullous, pustular, and vegetative variants can also occur. SS, also known as acute febrile neutrophilic dermatosis, is characterized by the abrupt onset of fever, peripheral neutrophilia, tender erythematous skin lesions and a diffuse neutrophilic dermal infiltrate. In this review that will be focused on PG and SS, we will describe also the aseptic abscesses syndrome, a new entity within the spectrum of ND which frequently occurs in association with IBD and is characterized by deep abscesses mainly involving the spleen and skin and by polymorphic cutaneous manifestations including PG- and SS-like lesions.


Subject(s)
Dermatitis/pathology , Inflammatory Bowel Diseases/pathology , Neutrophils/pathology , Pyoderma Gangrenosum/pathology , Sweet Syndrome/pathology , Abscess/complications , Antibodies, Monoclonal/therapeutic use , Colitis, Ulcerative/pathology , Crohn Disease/pathology , Dermatitis/complications , Dermatitis/drug therapy , Dermatologic Agents/therapeutic use , Gastrointestinal Agents/therapeutic use , Glucocorticoids/therapeutic use , Humans , Inflammatory Bowel Diseases/complications , Inflammatory Bowel Diseases/drug therapy , Infliximab , Pyoderma Gangrenosum/complications , Pyoderma Gangrenosum/drug therapy , Sweet Syndrome/complications , Sweet Syndrome/drug therapy , Treatment Outcome
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