Cutaneous Richter Syndrome mimicking a lower limb cellulitis infection - a case report and review of the literature.

Richter syndrome (RS) is characterized by the development of a high-grade lymphoma in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Herein, we present the case of an 85-year-old woman with a 3-year history of stable asymptomatic CLL that developed a cutaneous RS. The patient presented with painless inflammation in the left leg and foot that was initially diagnosed as a cellulitis infection. She was treated accordingly with ceftriaxone and clindamycin. However, after completing the antibiotic regimen, not only did the inflammation persist, but also superimposed painless nodules gradually appeared on the left leg and foot over the course of four months. The histopathological examination of the nodules revealed a large B-cell cutaneous lymphoma. The patient underwent chemotherapy with CVP, followed by R-CHOP, resulting in a reduction of size of the nodules and remission of the inflammation. The patient died five months after the diagnosis owing to a bacterial pneumonia. We identified in previous reports a total of fifteen cases of cutaneous RS. Most cases presented with rapidly growing tumors or multiple erythematous nodules, similar to our case. This case of a cutaneous RS mimicking a cellulitis infection underlines the importance of a low threshold for performing biopsies of suspicious skin lesions in patients with CLL/SLL.

Acute generalized exanthematous pustulosis to amoxicillin associated with parvovirus B19 reactivation.

We report the case of a 22-year-old male patient with 2 episodes, 4 months apart, of acute generalized exanthematous pustulosis (AGEP) associated with oral intake of amoxicillin and simultaneous reactivation of parvovirus B19 infection proven by positive polymerase chain reaction test in the skin fragment and blood sample and elevation of the IgG antibodies titer. To our knowledge, this is the first report of AGEP resulting from the interaction between drug hypersensitivity and the reactivation of parvovirus B19. A combination of an immunological reaction to the drug and virus infection could be responsible for the clinical picture.

Etoricoxib-induced fixed drug eruption with positive lesional patch tests.

Fixed drug eruption (FDE) is most commonly associated with antibiotics, anticonvulsants, and nonnarcotic analgens, including nonsteroidal anti-inflammatory drugs (NSAIDs). However, the newer cyclooxygenase 2 (COX-2) inhibitors have been rarely reported to cause FDE. We report the case of a 52-year-old Caucasian woman with erythematous pruritic plaques on the neck, left forearm, and second finger of the right hand, healing with hyperpigmentation and recurring in the same locations. The patient was sporadically taking oral etoricoxib 90 mg for her back pain and noticed the relation between administration of the drug and skin lesions, the time interval decreasing progressively from 1 week to 30 minutes. No other signs, symptoms, or drug intake was mentioned. The patch tests with etoricoxib 1% and 5% in petrolatum were positive at the location of the lesions and negative on the back (nonlesional skin). Standard European and NSAID series were negative. Patch tests of 10 healthy controls with etoricoxib 1% and 5% in petrolatum were negative. After the avoidance of the drug, no relapse was mentioned. The patch test was reliable for the diagnosis of FDE, avoiding the need for subsequent oral provocation testing and therefore preventing the possible adverse effects. Despite being regarded as a safe drug, the occurrence of cutaneous adverse reactions to etoricoxib should be considered, especially in the setting of its increasing use in pain control.

Hypocomplementemic urticarial vasculitis in mixed connective tissue disease.

Urticarial vasculitis is characterized clinically by urticaria-like skin lesions and histologically by leukocytoclastic vasculitis. It may be idiopathic or associated with various conditions such as infections, hematologic disorders, drugs, and connective tissue diseases, primarily systemic lupus erythematosus; an association with mixed connective tissue disease (MCTD) has rarely been reported. We present a case of hypocomplementemic urticarial vasculitis in a patient with MCTD that responded to hydroxychloroquine after a period of corticosteroid dependence.

Dermatoscopy of flat pigmented facial lesions-evolution of lentigo maligna diagnostic criteria.

Recognition of facial lentigo maligna (LM) is often difficult, particularly at early stages. Algorithms and multivariate diagnostic models have recently been elaborated on the attempt to improve the diagnostic accuracy. We conducted a cross-sectional and retrospective study to evaluate dermatoscopic criteria aiding in diagnosis of flat pigmented facial lesions (FPFL). We examined 46 FPFL in 42 Caucasian patients and found that 4 of 20 dermatoscopic criteria reached the significance level required for features indicating malignancy namely, hyperpigmented follicular openings, obliterated follicular opening, annular-granular structures, and pigment rhomboids. Concomitant presence of at least 2 or 3 of the 4 mentioned criteria was significantly more frequent in LM than in pigmented actinic keratosis (PAK). However, despite more frequently seen in LM, these features were also displayed in some of the PAK and other FPFL, so we found them not specific for LM. Although dermatoscopy enhances the diagnostic accuracy in evaluating FPFL, histopathology remains the gold standard for correct diagnosis, making evident the need for improvements in early noninvasive diagnosis of LM.

Case for diagnosis. Riga-Fede disease.

Riga-Fede disease is a rare, benign disorder characterized by reactive ulceration of the oral mucosa associated with repetitive dental traumatism. It was first described in children with neurologic disorders and is very rare in adults. This case report describes the occurrence of a large ulcer of the tongue, resembling squamous cell carcinoma, in an adult with hemiparesis. The lesion cleared after neurologic recovery. This case highlights the importance of considering this disorder in the differential diagnosis of oral mucosal ulcerations.

Thrombophilia in venous leg ulcers: a comparative study in early and later onset.

BACKGROUND: Several studies found that the patients with chronic venous ulcers (CVU) have an increased prevalence of thrombophilia (44-75%), similar to that observed in deep vein thrombosis (DVT). The patients who develop CVU before their 50 th birthday appear to represent a distinct group in terms of etiology, natural history and prognosis. AIM: To analyze the nature and prevalence of thrombophilia in patients with early onset of CVU (before 50-years old) compared with a group of patients with later onset. METHODS: Twenty-seven consecutive patients of each group were studied. They underwent clinical assessment and blood testing for factor V Leiden, prothrombin G20210A, methyltetrahydrofolate reductase C677T, plasminogen activator inhibitor type 1 (PAI-1) mutations, antithrombin, proteins C and S levels, and also antiphospholipid antibodies (anticardiolipin antibodies and lupus anticoagulant), cryoglobulins and cryoagglutinins. RESULTS: All the patients had at least one thrombophilia. The prevalences of single, 2 and ≥3 thrombophilias were 29.6%, 40.7% and 29.6%, respectively, in the early onset group, compared with 33.3%, 59.2% and 7.4% in the later onset group. The PAI-1 4G/4G homozygous mutation was significantly more common in patients with early onset of ulcer. The prevalences of factor V Leiden, prothrombin G20210A, elevated titer of antiphospholipid antibodies and the presence of cryoglobulins were higher in the early onset group, although the differences were not statistically significant. CONCLUSION: Our study brings evidence of a higher thrombophilic risk among the patients with early onset of the CVU as they had significantly higher prevalence of multiple (≥3) thrombophilias (P=0.03), homozygous mutations (P=0.03) and family history of leg ulcer (P=0.02) when compared with patients with later onset. Thrombophilia screening is important in patients with CVU before the age of 50 in order to stratify the thrombotic risk and to allow an appropriate prophylactic and therapeutic management.

[Blueberry muffin baby. A rare presentation of congenital cytomegalovirus infection]. / Blueberry muffin baby. A presentação rara de infecção congénita por citomegalovírus.

The 'blueberry muffin baby' designation was used to describe the cutaneous manifestations of congenital rubella. The differential diagnosis includes other TORCH infections, blood dyscrasias, neoplasms, or vascular disorders. We present a case of a newborn admitted at birth for presenting disseminated violaceous cutaneous nodules. Pregnancy was full term and without infectious complications, with prenatal diagnosis of restrictive intrauterine growth, hydramnios and suspected esophageal atresia. Maternal serology with no evidence of infection. The newborn blood study showed thrombocytopenia, direct hyperbilirubinemia, elevated transaminases and coagulopathy. During hospitalization he showed progressive hepatosplenomegaly. Skin biopsy showed extramedullary erythropoiesis. Myelogram revealed absence of megakaryocytic line precursors. The molecular analysis for cytomegalovirus in peripheral blood and bone marrow was positive. Cerebral ultrasound examination revealed bilateral calcifications and periventricular hyperechogenicity. The placental anatomopathological exam showed features suggestive of cytomegalovirus infection. On the fifth day of life was initiated ganciclovir. This case presentation is intended to emphasize that although it is a rare manifestation, congenital cytomegalovirus infection should be considered in the differential diagnosis of 'blueberry muffin baby'.

Case for diagnosis / Caso para diagnóstico

Riga-Fede disease is a rare, benign disorder characterized by reactive ulceration of the oral mucosa associated with repetitive dental traumatism. It was first described in children with neurologic disorders and is very rare in adults. This case report describes the occurrence of a large ulcer of the tongue, resembling squamous cell carcinoma, in an adult with hemiparesis. The lesion cleared after neurologic recovery. This case highlights the importance of considering this disorder in the differential diagnosis of oral mucosal ulcerations.

A doença de Riga-Fede caracteriza-se por ulceração reativa da mucosa oral associada ao traumatismo dentário repetitivo. Foi inicialmente descrita em crianças com déficit neurológico, sendo muito rara em adultos. O presente caso descreve o aparecimento de uma úlcera grande, semelhante ao carcinoma espinocelular, na língua de um adulto com hemiparesia. A lesão teve resolução completa após a recuperação neurológica. O caso salienta a importância de considerar esta doença no diagnóstico diferencial das úlceras da mucosa oral.