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.

Acute hemorrhagic edema of childhood after H1N1 immunization.

Acute hemorrhagic edema (AHE) is an uncommon self-limited disorder affecting young children triggered by infection, drugs, or immunization. A 2-year-old boy was observed due to sudden onset of painful and edematous purpuric papular and plaque lesions of the face and upper extremities that started 2 weeks after H1N1 immunization. The patient also developed exuberant edema on the face and dorsum of the hands. Complete blood count, biochemistry, and urinalysis results were normal. Histopathological examination revealed perivascular and periadnexial lymphocytic infiltrate with neutrophils and eosinophils, and leukocytoclastic vasculitis. Blood PCR technique was negative to several viruses, namely adenovirus, cytomegalovirus, Epstein Barr, enterovirus, HHV6, parvovirus B19, and H1N1. Symptomatic treatment and parents reassurance was promptly provided. However, new lesions continued to develop and in this setting systemic corticosteroid was prescribed. Complete clinical resolution was achieved within 2 weeks and no relapse was observed. The temporal relationship with H1N1 immunization, absence of previous drug intake, as well as exclusion of viral infections led the authors to propose that H1N1 vaccine was the predisposing factor in AHE development in our patient. To our best knowledge, this is the first reported association between AHE and H1N1 immunization.

A nodulo-ulcerative lesion on the nose.

Lupus vulgaris (LV) is a chronic, progressive, and potentially destructive form of cutaneous tuberculosis commonly seen in previously sensitized individuals with moderate to high immunity. We present a case of LV located on the nose of an 84-year-old female patient, discuss the diagnosis and treatment modalities, and emphasize the importance of having a high index of suspicion for this condition.

Case for diagnosis.

Ketron-Goodman disease was formerly considered a disseminated type of pagetoid reticulosis. However, according to the new classification consensus, it should be regarded as aggressive epidermotropic CD8 T-cutaneous lymphoma, cutaneous gamma/delta T-lymphoma, or tumor-stage mycosis fungoides, depending on the clinical-histological picture. This case highlights a rare and challenging presentation of Ketron-Goodman disease with an indolent presentation and evolution and good response to a low-grade treatment regimen, not fitting well into the new classification criteria.

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.

P-cadherin as prognostic factor for loco-regional relapse in breast cancer.

BACKGROUND: Breast cancer is the most frequent malignant tumor and the leading cause of cancer death in women in Portugal. Due to its relation to an increase in distant metastasis and subsequent death, loco-regional relapse is one major concern in breast cancer women. Several classic prognostic factors as tumour size, nodal stage, histological grade, HER2 status and hormonal receptors have been identified as the most important factors for determining loco-regional relapse, disease free and overall survival. However, there is heterogeneity in prognosis and tumor behaviour in patients with identical disease staging and a similar pattern of expression of known molecular markers, hence the need to discover new prognostic factors. One of the possibilities is P-cadherin, already described by researchers as a possible independent marker of prognosis in breast cancer. The aim of this work was to study in a retrospective series of patients the correlation of P-cadherin expression with loco-regional recurrence in breast cancer women. MATERIAL AND METHODS: We analyzed the clinical records of 1432 consecutive patients with breast cancer and treated in a University Hospital over a 10 year period. Patients with loco-regional relapse (n=101) without prior or simultaneous distant disease were selected as case group. Control group consisted of patients with more than 10 years follow-up and without disease progression. For both groups demographic, clinical, pathological and molecular markers were analyzed. Tissue micro-arrays were constructed to study P-cadherin expression from 86 tumors with available paraffin embedded blocks. RESULTS: Mean time to recurrence was 41 months and mean survival time after recurrence was 33 months, with a 5-year survival rate of 55%. Tumour size, nodal status and histological grade were identified as independent markers of prognosis. P-cadherin was associated with higher histological grades and hormone negative tumours. P-cadherin was identified as an independent prognostic marker for disease free survival, but not for overall survival. CONCLUSION: P-cadherin was related to other known factors of worse prognosis and had an independent relation to disease-free survival. P-cadherin might constitute a novel therapeutic target, but its real biological value is yet to be determined. Doubt persists whether it is an independent marker of tumour behaviour or only a surrogate marker of a set of clinical and molecular features related with worse prognosis.

Case for diagnosis / Caso para diagnostico

Ketron-Goodman disease was formerly considered a disseminated type of pagetoid reticulosis. However, according to the new classification consensus, it should be regarded as aggressive epidermotropic CD8 T-cutaneous lymphoma, cutaneous gamma/delta T-lymphoma, or tumor-stage mycosis fungoides, depending on the clinical-histological picture. This case highlights a rare and challenging presentation of Ketron-Goodman disease with an indolent presentation and evolution and good response to a low-grade treatment regimen, not fitting well into the new classification criteria.

A doença de Ketron-Goodman foi inicialmente considerada uma forma disseminada de reticulose pagetóide. Mas, de acordo com o atual sistema de classificação e dependendo do quadro clínico-patológico deve ser antes vista como um linfoma T CD8 agressivo epidermotrópico, linfoma T gama/delta ou micose fungóide, estadio tumoral. Pretendemos realçar esta doença rara que pode suscitar dúvidas no diagnóstico. Neste caso, a apresentação e evolução foram indolentes com boa resposta a um tratamento pouco agressivo, não se enquadrando bem nas novas propostas de classificação da doença.

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.