Acute localized exanthematous pustulosis (ALEP) is a rare disease characterized by the acute onset of multiple localized non‐follicular, pinhead‐sized pustules. ALEP is considered a localized form of acute generalized exanthematous pustulosis but its pathogeny is not well identified. We performed a systematic review of the literature of all publications regarding ALEP cases using the term “acute localized exanthematous pustulosis,” to provide an update on this disease and its management. Results and conclusion ALEP is an uncommon skin condition attributed primarily to a hypersensitivity reaction to a systemic drug (classical or herbal); though a contact mechanism has been reported. It may be misdiagnosed as infectious or inflammatory disease but the clinico‐pathological correlation in addition to the rapid response to withdrawal of the culprit agent supports this diagnosis. The pathogenesis of ALEP is still unclear, and there are no standardized treatment guidelines to manage this disease. Both AGEP and ALEP have a good prognosis if an early diagnosis is made.

The purpose of our study was to evaluate the frequency of leukocyte count abnormalities in a large cohort of SLE patients and its association with infection. To make this evaluation, we studied consecutive patients with SLE diagnosis and prospectively followed them in the Coimbra Lupus Cohort. Data about white blood cell count abnormalities and infection during one-year follow-up were obtained. The presence of leukopenia, lymphopenia, and neutropenia was registered for one-year period. Infections were classified as severe or mild. We found that of the 124 patients who were included (91.1% female, mean age 41.2, mean disease duration 11.1), mild infections occurred in 43.5%, and severe in 3.2% of the patients. Twelve percent, 41.1%, and 4.8% of the patients had persistent leukopenia, lymphopenia, and neutropenia, respectively. Fourteen percent received a pneumococcal vaccination. Patients with neutropenia had a significantly higher number of infections (P = 0.033). Thus, our study showed that neutropenia was associated with an increased risk of infection during this one-year follow-up. Infections were frequent, but most of them were mild.

Our series of LPPI seems that this disorder is underreported mainly among 3 dark-skinned persons as we have seen these ten cases within three years.

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