Chronic localized leukocytoclastic vasculitis: clinicopathological spectrum of granuloma faciale with and without extrafacial and mucosal involvement.

Granuloma faciale (GF) is a rare cutaneous condition of unknown origin, that usually presents as one or more brown-purple 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 clinico-pathological 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.

From erythema multiforme to toxic epidermal necrolysis. Same spectrum or different diseases?

Erythema 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.

Acute generalized exanthematous pustulosis: report of five cases and systematic review of clinical and histopathological findings.

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.