Coupled Plasma Filtration Adsorption for Treatment of Capillary Leak Syndrome Superimposed to Acute Generalized Exanthematous Pustolosis: A Case Report.

Coupled plasma filtration adsorption (CPFA) is an extracorporeal supportive therapy based on nonspecific adsorption of pro- and anti-inflammatory mediators combined with continuous renal replacement therapy. The main field of CPFA application is septic shock, and there are limited data about its efficacy in the treatment of other acute conditions characterized by a dysregulation in immune homeostasis. Capillary leak syndrome (CLS) defines a life-threatening condition sustained by hypercytokinemia and characterized by abrupt onset of increased capillary permeability leading to severe generalized edema and hypovolemic shock refractory to fluid administration. Therapy for CLS is not specific and, at present time, it consists in the use of steroids or intravenous immunoglobulins. We present the case of a 34-year-old woman who developed CLS superimposed to acute generalized exanthematous pustulosis after initiating therapy with hydroxychloroquine for undifferentiated connective tissue disease. CLS did not respond to steroids and intravenous immunoglobulins, while it was successfully treated with CPFA. This observation supports the possible role of CPFA in restoring a proper immunologic homeostasis not only in sepsis but also in other devastating conditions sustained by hypercytokinemia.

Acute generalized pustular bacterid concomitant with erythema nodosum, polyarthritis, and Achilles tendinitis.

We report a case of acute generalized pustular bacterid (AGPB) concomitant with erythema nodosum (EN), polyarthritis, and Achilles tendinitis. The patient was admitted with a complaint of fever, widespread plural pustules, erythema, and polyarthralgia. Histopathological examination of the skin lesions demonstrated features of AGBP and EN. Although arthralgia and AGPB can be recognized together, EN and Achilles tendinitis are rare manifestations seen in patients with AGPB. In this case report, we suggest arthralgia, EN, and Achilles tendinitis could coexist with AGPB.

Acute generalized exanthematous pustulosis and polyarthritis associated with a novel CARD14 mutation.

Acute generalised exanthematous pustulosis (AGEP) is a rare toxicoderma characterised by an acute onset rash, with many sterile pustules on the surface, high fever and increased acute phase reactants. We report the case of a patient who presented to the dermatology department with an AGEP and polyarthritis, in which a novel CARD14 mutation was identified. The pathophysiological mechanism of AGEP remains unclear, although mutations in the IL36RN gene have been identified in a small subset of AGEP patients. Similarly, mutations in the CARD14 gene have been linked to pustular types of psoriasis and familiar cases of pityriasis rubra pilaris; however, there are no reports associating mutations in the CARD14 gene with AGEP.

Acute generalized exanthematous pustulosis: atypical presentations and outcomes.

Acute generalized exanthematous pustulosis (AGEP) is an acute drug eruption characterized by erythematous plaques and papules studded with numerous, pinpoint pustules. Several atypical clinical presentations and triggers of AGEP have been described in the literature. These include systemic presentations similar to toxic epidermal necrolysis (TEN) and drug-induced hypersensitivity syndrome (DIHS) and localized presentations mimicking other medication reactions. We herein aim to review atypical presentations and medication triggers of AGEP to assist clinicians in recognizing this condition and making appropriate therapeutic interventions.

Systemic involvement of acute generalized exanthematous pustulosis: a retrospective study on 58 patients.

BACKGROUND: Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction characterized by rash with sterile pustules, high fever and elevated circulating neutrophil counts. OBJECTIVES: To investigate the frequency and clinical features of AGEP systemic involvement. METHODS: This retrospective study included all patients hospitalized in our department between 2000 and 2010 with a discharge diagnosis of AGEP. Patients had to fulfil the following criteria: (i) a specific EuroSCAR score > 4 and (ii) biological and radiological work-up available. RESULTS: Among the 58 patients enrolled, 10 had at least one systemic involvement: hepatic function test results were abnormal for seven; six had renal insufficiency; two developed acute respiratory distress, with one patient's bronchoalveolar lavage fluid containing many neutrophils but no microorganisms; one was agranulocytotic. Mean peripheral neutrophil counts and mean C-reactive protein levels were elevated significantly in patients with systemic involvement. Amoxicillin rechallenge and hospitalization duration were associated with systemic involvement. AGEP systemic involvement was observed in 17% of cases studied, including liver, kidney, bone-marrow and lung involvement. Outcomes were favourable after drug withdrawal, and symptomatic and topical steroid treatments. CONCLUSIONS: The neutrophil count-systemic involvement association may suggest a role for neutrophils in AGEP systemic involvement. Physicians should be aware of the possibility of systemic involvement in AGEP and should actively look for signs of extracutaneous reactions.

Acute generalized erythematous pustulosis occurring with Hailey-Hailey disease.

A 70-year-old woman urgently presented with severe eruptive skin dermatitis associated with fever and malaise 7 days after taking clindamycin for an unknown skin eruption. She had a 40-year-long history of well-controlled Hailey-Hailey disease. Physical examination revealed erythrodermic skin changes covering more than 80% of the patient's body surface, with hundreds of nonfollicular pustules. Many of the pustules fused into large bullae, involving the intertriginous as well as the extensor areas, sparing the mucosa. Her body temperature was 103 degrees F. Laboratory workup was significant for neutrophilia with a white cell count > 10,000/mm3.

Acute generalised exanthematous pustulosis: an unusual cause of carpal tunnel syndrome.

A 65-year-old man presented with generalised erythematous pustular rash following an insect bite. He was initially treated with antibiotics for presumed cellulitis. He subsequently developed bilateral carpal tunnel syndrome. Skin biopsy confirmed a diagnosis of acute generalised exanthematous pustulosis, which resolved with oral prednisolone. He also had steroid injections for his bilateral carpal tunnel syndrome and this recovered uneventfully. Clinical presentation, histological assessment and EuroSCAR criteria were key to the diagnosis and management of this case.

Antibiotic-Impregnated Intramedullary Cement Nail-Induced Acute Generalized Exanthematous Pustulosis and Septic Shock: A Case Report.

CASE: A 28-year-old woman with an infected proximal femur nonunion was treated with an antibiotic-coated intramedullary nail. Shortly after discharge, the patient presented to the emergency department and was readmitted with a morbilliform rash sparing the left lower extremity. She became hemodynamically unstable, despite cessation of intravenous antibiotics, requiring transfer to the intensive care unit and urgent removal of her antibiotic nail. She improved after surgery, and biopsy results from the rash confirmed acute generalized examthematous pustulosis. CONCLUSION: Acute generalized exanthematous pustulosis is a rare, dermatologic crisis that can be precipitated by antibiotics, even in the form of antibiotic cement.

Pustulosis acuta generalisata following chemotherapy in an HIV-positive patient.

Pustulosis acuta generalisata is an uncommon condition characterized by scattered symmetrical eruption of sterile pustules associated with elevated inflammatory markers, leukocytosis, fever and arthropathy caused by previous infection by group A streptococci (GAS). We reported here a case of pustulosis acuta generalisata in an HIV-positive patient recently treated with chemotherapy for a seminoma.

Acute generalized exanthematous pustulosis with multiple organ dysfunction syndrome.

Acute generalized exanthematous pustulosis is a rare condition characterized by sterile pustules on erythematous and edematous tissue. Mostly drug induced, this condition can also be caused by other factors. Cases due to vancomycin are rare. A 67-year-old woman with cellulitis of the left lower extremity was admitted with marked bilateral lymphedema of the lower extremities and diffuse erythema of the left lower extremity from foot to knee. She was given clindamycin and then vancomycin. On day 5, her condition worsened, with erythema involving the entire back. Although treatment with clindamycin and vancomycin was discontinued, acute generalized exanthematous pustulosis developed. After successful treatment of other complications, the skin condition improved. Because vancomycin is frequently used, clinicians should be aware of the possibility of acute generalized exanthematous pustulosis. Because the pustulosis decreases after withdrawal of the causative drug, being able to diagnose and differentiate the abnormality from other conditions is prudent.