Epidermolysis Bullosa Acquisita Mimicking Linear IgA Bullous Disease in a 5-year-old Child

We present a case of a 5-year-old child with epidermolysis bullosa acquisita, clinically resembling linear IgA bullous disease. The case demonstrates that autoimmune bullous dermatoses in childhood may show a clinical overlap, which makes the diagnosis based on clinical features highly unreliable. Specific immunofluorescence and immunoserological tests are crucial for precise diagnosis – in our case circulating antibodies against collagen VII were detected using ELISA and indirect immunofluorescence on transfected cells. The disease was treated with systemic and topical steroids with excellent results

Epidermolysis Bullosa Acquisita Mimicking Linear IgA Bullous Disease in a 5-year-old Child

We present a case of a 5-year-old child with epidermolysis bullosa acquisita, clinically resembling linear IgA bullous disease. The case demonstrates that autoimmune bullous dermatoses in childhood may show a clinical overlap, which makes the diagnosis based on clinical features highly unreliable. Specific immunofluorescence and immunoserological tests are crucial for precise diagnosis – in our case circulating antibodies against collagen VII were detected using ELISA and indirect immunofluorescence on transfected cells. The disease was treated with systemic and topical steroids with excellent results

Case Report: Coexistence of bullous pemphigoid and psoriasis: Therapeutic challenge and IL17A-targeted parallel treatment strategy

Autoimmune blistering diseases of the skin have all been reported in patients with psoriasis, bullous pemphigoid (BP) being the most frequently observed. The pathophysiologic triggers for BP in psoriatic patients are unclear. Recent observational studies have suggested that chronic psoriatic inflammation may cause pathological changes to the basement membrane zone, thus inducing autoimmunity against BP antigens through cross reactivity and “epitope spreading.” The coexistence of BP and psoriasis poses challenging therapeutic dilemmas related to the incompatibility of their standard treatments. Considering the probable common immunologic mechanisms in the pathogenesis of these inflammatory skin disorders, a suitable treatment regimen should be applied for their parallel control. We report three patients, who developed BP in the course of preceding long-lasting psoriasis. Secukinumab was administered as first-line treatment with promising therapeutic effect for both skin disorders and long-term disease control in two of the cases. In the third case, parallel disease control was initially achieved with methotrexate. A few years later, secukinumab was used for the treatment of a relapse of both dermatoses but worsening of BP was observed and methotrexate was reintroduced. Our experience on the therapeutic potential of secukinumab in BP is supported by the data in the literature. Recently, it was demonstrated that the proinflammatory cytokine IL17A has a functional role in the process of skin inflammation in BP, similarly to psoriasis. IL17A inhibition has emerged as a promising therapeutic strategy in patients with extensive or refractory BP but paradoxical development of BP after secukinumab treatment for psoriasis has also been described. This controversy emphasizes the need for further investigation into the development of optimal treatment strategies and recommendations

Rowell\u27s syndrome triggered by omeprazole

No abstract available</p

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).

BACKGROUND Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. OBJECTIVES These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. RESULTS Chronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. CONCLUSIONS These are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies

The gut microbiome in bullous pemphigoid: implications of the gut-skin axis for disease susceptibility

Bullous pemphigoid (BP) is an autoimmune blistering disease that primarily affects the elderly. An altered skin microbiota in BP was recently revealed. Accumulating evidence points toward a link between the gut microbiota and skin diseases; however, the gut microbiota composition of BP patients remains largely underexplored, with only one pilot study to date, with a very limited sample size and no functional profiling of gut microbiota. To thoroughly investigate the composition and function of the gut microbiota in BP patients, and explore possible links between skin conditions and gut microbiota, we here investigated the gut microbiota of 66 patients (81.8% firstly diagnosed) suffering from BP and 66 age-, sex-, and study center-matched controls (CL) with non-inflammatory skin diseases (132 total participants), using 16S rRNA gene and shotgun sequencing data. Decreased alpha-diversity and an overall altered gut microbial community is observed in BP patients. Similar trends are observed in subclassifications of BP patients, including first diagnoses and relapsed cases. Furthermore, we observe a set of BP disease-associated gut microbial features, including reduced Faecalibacterium prausnitzii and greater abundance of pathways related to gamma-aminobutyric acid (GABA) metabolism in BP patients. Interestingly, F. prausnitzii is a well-known microbiomarker of inflammatory diseases, which has been reported to be reduced in the gut microbiome of atopic dermatitis and psoriasis patients. Moreover, GABA plays multiple roles in maintaining skin health, including the inhibition of itching by acting as a neurotransmitter, attenuating skin lesions by balancing Th1 and Th2 levels, and maintaining skin elasticity by increasing the expression of type I collagen. These findings thus suggest that gut microbiota alterations present in BP may play a role in the disease, and certain key microbes and functions may contribute to the link between gut dysbiosis and BP disease activity. Further studies to investigate the underlying mechanisms of the gut-skin interaction are thus clearly warranted, which could aid in the development of potential therapeutic interventions

EPIDERMOLYSIS BULLOSA SIMPLEX DOWLING-MEARA - A case report

A case with Epidermolysis bullosa simplex Dowling-Meara is presented. It concerns a 20 days breast-fed girl with severe blistering and erosions on the skin formed at birth after minor mechanical trauma. There are no other affected relatives. A medical examination showed normal somatic and visceral status. There were multiple herpetiform grouped bullous and erosive lesions on the face, trunk, upper and lower limbs, palms and soles. The mucous membranes were not affected. There was also a marked nail dystrophy on the fingers and toenails. The electron microscopic examination revealed cytolysis of the basal keratinocytes with clumping of the perinuclear tonofilaments in the lesional skin. Because of the clinical features and the results of the electron microscopic examination of the skin the case here reported should be considered as an Epidermolysis bullosa simplex Dowling-Meara. The child is followed up to the age of three. The course of the disease was benign with a decreased formation of blisters and erosions. The marked nail dystrophy was persistent. There was palmar and plantar hyperkeratosis. The child’s growth, neural and psychic development wsas normal. The early diagnosis of the bullous epidermolysis is helpful in the prognostic assessment of the disease and in the medical and genetic advice for the parents

HERPES GESTATIONIS.

Herpes gestationis, also known as pemphigoidgestationis (PG) is an extremely rare autoimmune bullous dermatosis of the gestation and postpartum period. The disease was originally named herpes gestationis on the basis of the morphological herpetiform feature of the blisters. We report a 21-year-old woman, pregnant in the third trimester, who presented with a pruritic bullous cutaneous eruption of two weeks duration. The disease started with a red plaque in the abdominal area accompanied by mild itching. Soonafter, blisters appeared and affected almost the entire body. Physical examination revealed a primiparous woman in good general state, pregnant in 36 weeks of gestation. The skin changes affected the abdomen, back of the trunk, upper and lower extremities, hands and feet. They were manifested by a polymorphous eruption, consisting of erythematous urticaria-like plaques, small tense vesicles and multiple excoriations. Mucous membranes were not affected. Routine laboratory examinations were within normal limits. Direct immunofluorescence (DIF) on perilesional skin showed linear deposition of IgG (++) and C3 (++) at the cutaneous basement membrane zone (BMZ). Indirect immunofluorescence (IIF) on human esophagus substrate revealed circulating IgG anti-BMZ antibodies at a titer of 1:80. ELISABP180 NC16A was strongly positive. The diagnosis of PG was confirmed and a treatment with systemic methylprednisolone 60 mg/day was initiated, later gradually tapered to 20 mg/day, together with topical corticosteroids. As a result on the 10th day of the treatment we already achieved significant improvement with reduction of erythema and itching, absence of new skin lesions. The pregnancy ended in term with successful childbirth. No flare of the skin disease was observed in the puerperal period