Ulceroglandular tularemia.

An increasing number of patients with the zoonosis tularemia have been reported in the last few years in Europe. Tularemia can be divided into different forms depending on its appearance. Tularemia must be considered in the differential diagnosis of diseases that present with an ulcer and regional lymphadenopathy. The diagnosis can be confirmed by culturing Francisella tularensis. With effective antibiotic intervention, the prognosis is favorable. Typically tularemia develops after outdoor activities; it is generally transferred by blood-sucking arthropods from infected wild animals to humans.

[Desmoglein 1-negative, desmoglein 3-positive pemphigus herpetiformis with involvement of oral mucous membranes]. / Desmoglein-1-negativer, Desmoglein-3-positiver Pemphigus herpetiformis mit oraler Beteiligung.

A 46-year-old woman presented with a two year history of pruritic erythematous plaques with blisters, as well as oral erosions. Even though the cutaneous lesions fit best with dermatitis herpetiformis, bullous pemphigoid or pemphigoid gestationis, the histologic examination revealed eosinophilic spongiosis, most compatible with some form of pemphigus. The identification of intercellular IgG deposition on direct immunofluorescence and circulating IgG pemphigus antibodies on indirect immunofluorescence microscopy led to diagnosis of pemphigus herpetiformis. This rare form of pemphigus does not often involve the oral mucosa. In our patient, the explanation is that she had antibodies against desmoglein 3 but not desmoglein 1. Treatment with prednisolone and azathioprine caused rapid and complete healing.

Ulcerated epithelioid hemangioendothelioma of the foot in childhood.

Epithelioid hemangioendothelioma is an uncommon malignant vascular tumor usually involving soft tissue and, in rare cases, the skin. Histologically and biologically it is considered to be a borderline neoplasm between an angiolymphoid hyperplasia with eosinophilia and an epithelioid angiosarcoma. Here we describe an 11-year-old girl with a 1-year history of an isolated, spontaneously appearing, painful ulceration on the instep of the right foot. The histopathologic examination of a wedge biopsy specimen revealed epithelioid eosinophilic cells with intracytoplasmic vacuoles containing erythrocytes. Immunohistochemical staining was positive for the endothelial markers CD31, CD34, and factor VIII. On the basis of these findings the diagnosis of epithelioid hemangioendothelioma was made. Two weeks after complete excision of the tumor, a lymph node metastasis in the right groin was excised. Because of another inoperable lymph node metastasis on the proximal right femur, polychemotherapy was started. As our case report shows, in the event of a nonhealing cutaneous ulceration the possibility of a malignant tumor such as epithelioid hemangioendothelioma should be considered, even in children.

Molecular diagnostics facilitate distinction between lethal and non-lethal subtypes of junctional epidermolysis bullosa: a case report and review of the literature.

UNLABELLED: The term epidermolysis bullosa (EB) encompasses a heterogeneous group of genodermatoses, characterised by fragility and blistering of the skin, often associated with extracutaneous manifestations. The clinical picture comprises severe subtypes with lethal outcome in the first years of life as well as milder subtypes with localised blistering or minimal symptoms confined exclusively to nail or teeth abnormalities. We present the case of a male infant, who was born with a few bullae and rapidly developed extensive blistering of the skin. The disease was complicated by painful erosions of the oral mucosa, refused ingestion, and recurrent infections. The child died at the age of 4 months because of cardiac failure due to severe sepsis. Antigen mapping of a skin biopsy showed a split within the lamina lucida of the epidermal basement membrane zone and junctional epidermolysis bullosa (JEB) was diagnosed within the first 3 weeks of life. Markedly reduced staining for laminin 5 indicated the Herlitz type of JEB (OMIM 226700), which could be confirmed by mutation analysis in the LAMB3 gene, showing homozygous nonsense mutations. CONCLUSION: early antigen mapping using antibodies against the proteins affected in epidermolysis bullosa, is a useful tool providing early mutation analysis and valuable prognostic information needed for adequate therapeutic strategies. The recently published literature on current diagnostic procedures and the revised classification system for inherited epidermolysis bullosa aim towards a better understanding of the disease.