1,720,967 research outputs found
Secondary erythromelalgia: A tryptophan dietary supplement-induced case associated with elevated 5-hydroxyindoleacetic acid (5HIAA) urinary levels
No full textPost-erysipela panniculitis ossificans of the lower leg treated with pentoxifylline
No full textWe report the case of a female patient who developed a firm, wooden-like, nonpitting edema of the left lower leg after a dermo-hypodermitis. The clinical picture was accompanied by intense pain, strongly impacting the patient's quality of life. A soft-tissue ultrasound demonstrated several millimetric hyperechoic linear lesions whose histopathological examination was conclusive for panniculitis ossificans. A conservative medical management with compression stockings associated with pentoxifylline 800 mg/day was prescribed with improvement of the edema and, in particular, a good pain control. To date, after a 2-year therapy with pentoxifylline, the leg wooden-like edema has substantially improved, despite the persistence of the well-known foci of ossification, and the pain has resolved, conditioning a substantial improvement of the patient's quality of life. No side effect has been observed during the routine follow up. Although there is no unanimous opinion in the literature about the effect of pentoxifylline on bone formation and osteogenic differentiation, pentoxifylline treatment proved to be beneficial in our patient both for the heterotopic ossification process and the pain control. We collected some of the data in literature about pentoxifylline effects and advanced some hypotheses to explain our results. Finally, we suggest that an anti-inflammatory and vasodilators drug such as pentoxifylline could be a possible alternative in heterotopic ossification disorders
Tumefazione orbito-temporale con enoftalmo e deformazione del volto: descrizione di un caso
Full text linkLa famiglia delle neurofibromatosi annovera differenti rare entità nosologiche, tra le quali, secondo la classificazione di Riccardi, si possono distinguere la neurofibromatosi di tipo 1 (NF-1) e la neurofibromatosi di tipo 2 (NF-2), aventi criteri diagnostici clinici codificati. Esistono tuttavia varianti cliniche che presentano un’espressività della malattia e delle manifestazioni fenotipiche non facilmente inquadrabili in una delle due forme codificate, risultando caratterizzate da aspetti e comportamenti clinici più sfumati o peculiari.
Si descrive un paziente, giunto all’osservazione per la presenza di una tumefazione peri-orbitale ed orbitale risultata poi essere, istologicamente, un neurofibroma; attraverso la RMN si evidenziava un’estensione della neoplasia molto oltre l’aspetto visibile, fino ai piani profondi, coinvolgente tutta la zona circostante, avendo margini mal definiti. In base al dato clinico, istologico, e strumentale si poneva il sospetto diagnostico di neurofibromatosi, ascrivibile allo spettro delle varianti rare di NF-1; la revisione dei dati bibliografici ha confermato, in parte, l’ipotesi diagnostica, come viene descritto nella discussione
Chronic localized leukocytoclastic vasculitis: Clinicopathological spectrum of granuloma faciale with and without extrafacial and mucosal involvement
No full textGranuloma faciale (GF) is a rare cutaneous condition of unknown origin, that usually presents as one or more brownpurple papules, plaques and/or nodules, localized mostly on the face, although extrafacial lesions can also occur. Eosinophilic angiocentric fibrosis (EAF) is regarded as the mucosal counterpart of GF. Histologically, it has been described as a persistent leukocytoclastic vasculitis, with a dense polymorphous inflammatory infiltrate in the superficial and mid dermis, typically sparing the subpapillary dermis, the so called grenz zone. The presence of eosinophils is considered a characteristic feature of the disease. All the cases of GF seen at the Dermatology Unit from 2002 to 2013 were considered and reviewed, both clinically and histopathologically. Only cases with consistent clinical findings of GF, and accurate patient's history were considered. Ten cases of GF were reviewed for both histological specificity and clinicopathological correlation. Two patients presented extrafacial lesions. One patient had involvement of nasal mucosa. Two patients suffered from associated rheumatological diseases. The most frequent histopathologic features were the presence of a grenz zone and eosinophils in the infiltrate, but also adnexal involvement was often present; vascular changes were constant, yet leukocytoclastic vasculitis could be recorded only in four cases. Fibrosis or sclerosis were always absent. Clinical pictures of the patients treated demonstrated a complete remission of the lesions, without scarring. However, a complete enduring healing was observed only in two patients, and relapse or incomplete remission of the disease was the rule. In conclusion a review of clinicopathological findings of ten patients affected by GF was made and new details of the disease presented
Acute generalized exanthematous pustulosis: report of five cases and systematic review of clinical and histopathological findings
No full text: Acute generalized exanthematous pustulosis (AGEP) is a rare, drug-related pustular eruption usually starting from folds with edema and erythema and with subsequent spreading. Clinically AGEP is characterized by the sudden appearance of dozen of sterile, non follicular, small pustules on erythematous and edematous skin. Mild non erosive mucosal involvement, mostly oral, may sometimes occur. Fever, neutrophilia and peripheral blood eosinophilia (in a third of patients) are present. Other skin signs such as facial edema, purpura, target-like lesions and blisters have been described but are not typical for AGEP. Diagnostic criteria for AGEP were established by an international committee of experts, the European Study of Severe Cutaneous Adverse Reactions (EuroSCAR). The most relevant histopathological feature is represented by the detection of non-follicular subcorneal and/or intracorneal spongiform pustules that are usually large, contiguous and tend to coalesce. After elimination of the causative drug, pustules usually spontaneously disappear in a few days with desquamation and the reaction fully resolves within 15 days. Internal organs are not usually involved and no systemic treatment is required. Withdrawal of the culprit drug is mandatory. Although AGEP is a self-limiting disease with a favourable prognosis, secondary infections are a not infrequent complication in patients in poor general medical conditions. The reported mortality is about 5%. The most severe cases are associated with drug rechallenge
Intralesional vinblastine injections for treatment of Classic Kaposi sarcoma in diabetic patients
No full textClassic Kaposi sarcoma (KS) usually is a localized and slowly progressing disease that mainly affects elderly patients; therefore, local treatment generally is recommended. In this study, we evaluated the clinical efficacy of intralesional vinblastine (VNB) for the treatment of classic KS in 6 participants with type 2 diabetes mellitus. Results indicated that intralesional VNB injections may be an effective alternative treatment of classic KS in diabetic patients
From erythema multiforme to toxic epidermal necrolysis. Same spectrum or different diseases?
No full textErythema multiforme (EM), Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute bullous disorders associated to different prognosis, mainly due to infections and drugs. More in particular EM in more than 90% is caused by infections (especially Herpes virus infection), while, on the other hand SJS and TEN are referable in more than 95% of cases to drugs. Distinction among these three forms is often controversal and still debated. An attempt to distinguish these forms has been possible mainly according to anamnesis, clinical presentation (morphology, involved sites, extension of lesions) and pathogenetic mechanisms, being on the contrary more difficult from an histopathological point of view. Nowadays a clear diagnosis and a distinction from other life-threatening diseases is possible with the integration of all the mentioned aspects. Moreover, this recognition should be as early as possible in order to perform a prognostic evaluation of the case and to start supportive cares and therapies as soon as possible
Chronic localized leukocytoclastic vasculitis: Clinicopathological spectrum of granuloma faciale with and without extrafacial and mucosal involvement
No full textGranuloma faciale (GF) is a rare cutaneous condition of unknown origin, that usually presents as one or more brownpurple papules, plaques and/or nodules, localized mostly on the face, although extrafacial lesions can also occur. Eosinophilic angiocentric fibrosis (EAF) is regarded as the mucosal counterpart of GF. Histologically, it has been described as a persistent leukocytoclastic vasculitis, with a dense polymorphous inflammatory infiltrate in the superficial and mid dermis, typically sparing the subpapillary dermis, the so called grenz zone. The presence of eosinophils is considered a characteristic feature of the disease. All the cases of GF seen at the Dermatology Unit from 2002 to 2013 were considered and reviewed, both clinically and histopathologically. Only cases with consistent clinical findings of GF, and accurate patient's history were considered. Ten cases of GF were reviewed for both histological specificity and clinicopathological correlation. Two patients presented extrafacial lesions. One patient had involvement of nasal mucosa. Two patients suffered from associated rheumatological diseases. The most frequent histopathologic features were the presence of a grenz zone and eosinophils in the infiltrate, but also adnexal involvement was often present; vascular changes were constant, yet leukocytoclastic vasculitis could be recorded only in four cases. Fibrosis or sclerosis were always absent. Clinical pictures of the patients treated demonstrated a complete remission of the lesions, without scarring. However, a complete enduring healing was observed only in two patients, and relapse or incomplete remission of the disease was the rule. In conclusion a review of clinicopathological findings of ten patients affected by GF was made and new details of the disease presented
Intralesional vinblastine injections for treatment of Classic Kaposi sarcoma in diabetic patients
No full textClassic Kaposi sarcoma (KS) usually is a localized and slowly progressing disease that mainly affects elderly patients; therefore, local treatment generally is recommended. In this study, we evaluated the clinical efficacy of intralesional vinblastine (VNB) for the treatment of classic KS in 6 participants with type 2 diabetes mellitus. Results indicated that intralesional VNB injections may be an effective alternative treatment of classic KS in diabetic patients
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