Bullous Skin Lesions Research Articles

Diagnostic value of Tzanck smear in various erosive, vesicular, and bullous skin lesions.

Background:Cutaneous cytology has long been shown to be useful in the diagnosis of several erosive, vesicular, and bullous skin lesions. The Tzanck smear although an old tool, still remains a simple, rapid, easily applied, and inexpensive test for these skin lesions.Aims and Objectives:The aim of this study was to evaluate the diagnostic value of Tzanck smear by determining its sensitivity and specificity in various erosive, vesicular, and bullous skin lesions.Materials and Methods:One hundred and forty-two patients with erosive, vesicular, and/or bullous skin lesions were included in the study. Four groups of disorders were identified: infections, immunologic disorders, genodermatosis, and spongiotic dermatitis. All the study cases were evaluated by Tzanck smear. Definitive diagnosis was established by standard diagnostic techniques (including when appropriate, viral serology, bacterial culture, histopathology, direct immunoflourescence).Results:The sensitivity and specificity of cytologic findings was respectively 86.36% and 91.30% for viral infections; for bacterial infections, it was 85.7% and 66.6%. The sensitivity and specificity of Tzanck smear was respectively 85.0% and 83.33% for pemphigus; for bullous pemhigoid it was 11.11% and 100.0%. Tzanck smear sensitivity in genodermatoses was 100%. The sensitivity and specificity of the test in spongiotic dermatitis could not be calculated due to an insufficient number of patients.Conclusion:The Tzanck smear is a quick and reliable tool for the evaluation of various erosive and vesiculobullous skin lesions.

Genome-wide gene expression profiling reveals unsuspected molecular alterations in pemphigus foliaceus.

Pemphigus foliaceus (PF) is a complex autoimmune disease characterized by bullous skin lesions and the presence of antibodies against desmoglein 1. In this study we sought to contribute to a better understanding of the molecular processes in endemic PF, as the identification of factors that participate in the pathogenesis is a prerequisite for understanding its biological basis and may lead to novel therapeutic interventions. CD4+ T lymphocytes are central to the development of the disease. Therefore, we compared genome-wide gene expression profiles of peripheral CD4+ T cells of various PF patient subgroups with each other and with that of healthy individuals. The patient sample was subdivided into three groups: untreated patients with the generalized form of the disease, patients submitted to immunosuppressive treatment, and patients with the localized form of the disease. Comparisons between different subgroups resulted in 135, 54 and 64 genes differentially expressed. These genes are mainly related to lymphocyte adhesion and migration, apoptosis, cellular proliferation, cytotoxicity and antigen presentation. Several of these genes were differentially expressed when comparing lesional and uninvolved skin from the same patient. The chromosomal regions 19q13 and 12p13 concentrate differentially expressed genes and are candidate regions for PF susceptibility genes and disease markers. Our results reveal genes involved in disease severity, potential therapeutic targets and previously unsuspected processes involved in the pathogenesis. Besides, this study adds original information that will contribute to the understanding of PF's pathogenesis and of the still poorly defined in vivo functions of most of these genes.

A Case of Esophagitis Dissecans Superficialis With Concomitant Bullous Pemphgoid

Introduction: Esophagitis dissecans superficialis is an exceedingly rare endoscopic finding characterized by sloughing of large fragments of the esophageal mucosal lining that has uncommonly been associated with autoimmune bullous dermatoses. We report a case of a 77-year-old male who presented with 2 months of upper and lower extremity bullous skin eruptions in addition to more recent odynophagia and melenic stools in the setting of ibuprofen use for analgesia. A diagnosis of bullous pemphgoid was confirmed by direct immunofluorescence which identified immunoglobulin G and complement 3 deposits at the dermoepidermal junction. Endoscopy performed the following day revealed, in addition to a 2 centimeter duodenal bulb ulcer, vertical linear fissures with sloughing of the superficial esophageal mucosa consistent with esophagitis dissecans superficialis. The patient was initially treated with intravenous methylprednisolone for 3 days, followed by a prednisone taper with improvement in his bullous skin lesions, odynophagia and melenic stools. With less than 10 cases of esophagitis dissecans superficialis associated with bullous pemphigoid described in the English literature, prompt endoscopic recognition is vital in corroborating a diagnosis and guiding therapy.Figure 1: Sloughing of large fragments of the esophageal mucosal lining.

Rare Adverse Effect of Treatment With Minocycline

An 18-year-old woman presented with a 4-week history of a solitary painful bullous skin lesion over the lower right anterior tibia accompanied by generalized myalgia, polyarthalgia, joint stiffness, paraesthesia, and episodic ankle swelling. The lesion first appeared as a skin-coloredpapule and,within aweek, developedanerythematoushalo.Hermedical history includes asthma and acne. To our knowledge, there is no family history of this condition. Her medications included minocycline hydrochloride (for 25 months for acne), ranitidine hydrochloride, contraceptive pills, and iron supplements. An initial physical examination revealed mild synovitis in both ankles and an erythematous, raised papule overlying the distal aspect of the right anterior tibia. Treatment with prednisone acetate (10 mg daily) was initiated. Seven weeks later, the original skin lesion evolved into an ulcer with erythematous, raised borders (Figure 1). The case patient had new eruptions of nonindurated, violaceous, nontender, reticulated patches overlying the skin on the bilateral anterior tibias, consistent with livedo reticularis. She denies any previous episodes, and the results of a review of mitigating factors were negative. A laboratoryworkup revealed apositive antinuclear antibody 1:640nucleolar pattern, anelevatederythrocyte sedimentation rate, andelevatedC-reactiveprotein andserum IgG levels. The results of the following laboratory studies were negative or normal: complete blood cell count, urinalysis, liver profile, Lymedisease, parvovirus B19,Monospot test, uric acid, rheumatoid factor, antistrepolysin antibody, anti-Scl70, antihepatitis C virus, hepatitis B surface antigen, hepatitis B core antibody, cytomegalovirus antibody, and human T-lymphotropic viruses 1 and 2 antibody. Culture results of the skin lesion were negative. The results from chest radiography were normal. Figure 1.Ulcer with raised border and erythematous halo in distal right shin.

Pilomatricoma: an unusual presentation.

Dear Editor: Pilomatricoma, a slow-growing, benign adnexal tumor of the skin, originates from primitive cells of the hair matrix. It usually appears as a painless, firm, subcutaneous nodule, which adheres to the overlying skin. The overlying skin normally has a bluish discoloration. Although lesions with bullous changes on the overlying skin are very rare, they have been reported1,2,3. Herein, we report a case of bullous pilomatricoma to enhance the recognition and proper management of this variant, which represents a particular diagnostic challenge. An 18-year-old woman chiefly complained of a slightly tender, bullous skin lesion on her right upper back (Fig. 1A). Three months earlier, the lesion had appeared as a painless, firm nodule that enlarged with time beneath the skin. The bullous appearance was detected 1 month before her visit. The bulla was thick-walled, semitransparent, and sized 2.5×3 cm. A palpable mass was faintly noted inside the bulla. Systemic and laboratory examination results were normal. Preoperative bulla bisection revealed a firm central tissue core, surrounded by a sac and serous fluid (Fig. 1B). The lesion was totally excised and closed directly. The 1×2 cm mass inside the bulla was similar to a classical pilomatricoma: well demarcated, lobulated, and hard. On microscopic examination, we observed typical cells; anucleate keratinized basaloid cells (Fig. 2A, B). Endothelial cells of the dilated lymphatic channels were positive, on immunohistochemical staining, for CD34 and D2-40. Pseudocysts without endothelial cell lining were noted in the superficial dermis (Fig. 2C). The patient remained well without recurrence during follow-up for six months. Fig. 1 (A) Preoperative photograph showing the semi-transparent bulla, sized approximately 2.5×3 cm, with a whitish nodule inside, located on the right upper back of the patient. (B) Appearance inside the bulla: a firm central tissue core, within a serous ... Fig. 2 (A) Scanning view (1:1) demonstrating the whole architecture of the tumor with cystic change (H&E). Microscopic finding shows multiple dilated lymphatics above the typical nests of shadow cells and basophilic cells. (B) Arrow indicates ghost cells, ... Reports on bullous pilomatricoma are limited. To date, a total of 18 cases have been published1. Bullous pilomatricoma has a predilection for the shoulder and upper arm regions, predominantly affecting women aged 10 to 20 years. Lymphatic fluid fills the bulla-like space. It has been postulated that the pressure surrounding the hard core of the pilomatricoma induces obstruction of lymphatic vessels and congestion of lymphatic fluid, resulting in dilation of lymphatic vessels, with leakage of lymphatic fluid, and edema in the dermis surrounding the tumor, producing a bullous appearance2. Nearly all the cases of bullous pilomatricoma are formed by typical tumor nests-composed of shadow and basophilic cells-surrounded by a fibrotic capsule. Most cases present dilated lymphatic vessels and lymphedema in the superficial dermis2,3. Ghost, or shadow, cells are the most characteristic microscopic findings in pilomatricoma cases. They have distinct cell borders, with eosinophilic cytoplasm and a central clearing, representing the outline of an absent nucleus. Commonly, there is an associated foreign-body multinucleated giant-cell reaction and fibrosis with calcification; hence, this lesion was originally described as calcifying epithelioma of Malherbe. As for the classical pilomatricoma, surgical excision is the treatment of choice because this lesion does not regress spontaneously. The prognosis is excellent, without reported recurrences or malignant changes after excision.

Pemphigus vulgaris in pregnancy associated with herpes virus type 1 infection.

Dear Editor: Pemphigus vulgaris (PV) rarely occurs during pregnancy. We report a case of PV associated with herpes simplex type 1 virus (HSV-1) which occurred in the third trimester of pregnancy. A 32-year-old second gravida at 37 weeks' gestation was admitted for multiple bullous skin lesions that persisted for over a month. These vesicular and erosive lesions initiated from the periumbilical region and spread to the oral mucosa and the skin of the back (Fig. 1A, B). The diagnosis of PV was confirmed by biopsy (Fig. 1C), and direct immufluorescence detected anti-immunoglobulin G and C3 antibodies. Anti-desmoglein 1 and anti-desmoglein 3 antibodies were elevated at 82.1 U/ml (normal, <14 U/ml) and 184.9 U/ml (normal, <7 U/ml) in peripheral blood. Tzanck smear and viral polymerase chain reaction (Seeplex STD B41 Detedtion; Seegene, Seoul, Korea) were done on the base of a vesicular lesion on the trunk. Tzanck smear was negative, but, viral polymerase chain reaction (PCR) was positive for HSV-1 (Fig. 2). Fig. 1 (A) Multiple erosions on oral mucosa and (B) scattered crusted vesicles and erosions on the back. (C) Suprabasal blisters with eosinophils and acantholytic cells in the epidermis (H&E, ×400). Fig. 2 Polymerase chain reaction results show a positive band on 189 bp. M: 100 bp DNA ladder, Lane 1: positive result of the patient's tissue for herpes simplex type 1 virus (189 bp), Lane 2: normal control, Lane 3: positive control, Lane 4: sensitivity test, ... Prednisolone at a dose of 20 mg/d was initiated. Following PCR results, antiviral agent (valacyclovir, total 4,500 mg) was also given for 5 days. Lesions started to improve after starting the medication. Two weeks after the diagnosis the patient was transferred to the labour unit for induced labour. The baby was in a good state. After giving birth, the dose of prednisolone was increased to 25 mg/d, and mycophenolate mofetil 1.0 g/d was added. After improvement in both vesicles and general health status, the patient was discharged. During follow-up, a subtle amount of skin lesion occurred which was controlled with topical steroid, oral mycofenolate mofetil 1.0 g/d, and intermittent oral steroid at a dose of 4 mg/d. Though it is known that pemphigus develops as a result of the interaction between endogenous (genetic) factors and exogenous factors1, the primary stimulus for the autoimmune response of PV is still unknown. Pregnancy may aggravate certain autoimmune diseases, such as systemic lupus erythematosus, pemphigoid gestationis, and PV2. The patient's vesicular eruptions developed during the period of conception and were not controlled well at that time, so we supposed her condition was related to pregnancy. Bullous diseases that are reported to be associated with pregnancy include pemphigoid gestationis pemphigus foliaceus, and PV2,3. The most important diagnostic tool is histologic examination, and the two diseases mentioned above show subepidermal, subcorneal blisters. However PV, as seen in our patient, shows a suprabasal epidermal split with acantholysis and few eosinophils in the dermis, and perilesional skin shows intercelluar deposits of IgG and C3 in the epidermis on direct immunofluorescence4. A diagnosis of PV in this patient was made according to the histological features and clinical involvement of oral mucosa. The possible role of viruses, especially HSV-1, has been proposed in the pathogenesis of PV. Positive viral PCR results suggest that viral factors, especially HSV and Epstein-Barr virus, are capable of inducing and/or exacerbating pemphigus in a genetically susceptible host1. Infectious agents can stimulate the immune response in genetically susceptible individuals or in those with immune deviating conditions such as pregnancy, leading to an increase in the production of cytokines. High levels of interferon-gamma induce the expression of human leucocyte antigen type 2 in the membranes of keratinocytes, making the structural site of PV antigen (epitope spreading) immunologically active. Chronic, recurrent viral infections can also stimulate excessive production of interleukin (IL)-4 and IL-10, which result in a shift from TH1 to TH2 response, increasing antibody production. In addition, they can also directly infect B and T lymphocytes, contributing to the production of autoreactive B lymphocytes and autoimmune antibodies5. When infected, keratinocytes can pass through structural changes which favor the exposure of antigens1. In summary, we report a case of a woman who showed PV associated with HSV-1 in her second pregnancy, which was cured with steroids and antiviral agents.

Urethrocutaneous fistula after use of Tegress bulking agent: Case report and review of the literature

We report a case of a 68-year-old man who presented with a urethrocutaneous fistula after off-label use of Tegress (C. R. Bard, Inc., Murray Hill, NJ) Urethral Implant for post-prostatectomy incontinence. He was treated for prostate cancer with an open radical retropubic prostatectomy and adjuvant external beam radiation therapy. He was treated unsuccessfully for stress incontinence with a Tegress Urethral Implant and presented to our clinic initially with extrusion of the material urethrally. Four years later he re-presented with a large bullous skin lesion on his suprapubic area. Contrast-enhanced magnetic resonance imaging and retrograde urethral cystogram demonstrated a urethrocutaneous fistula. Subsequent cystoscopy revealed the calcified extruded material in the same location as the site of Tegress injection. The patient underwent simple cystectomy with ileal diversion. He recovered well postoperatively. This appears to be the first reported case of urethrocutaneous fistula after use of a Tegress Urethral Implant for post-prostatectomy stress urinary incontinence.

A Case Report of Paraneoplastic Pemphigus Associated with Esophageal Squamous Cell Carcinoma

Paraneoplastic pemphigus is an autoimmune blistering and erosive mucocutaneous syndrome associated with underlying neoplasm. It is primarily associated with lymphoproliferative disorders, and uncommonly with malignancies of epithelial origin. We report on a case of a 68-year-old male who presented with whole body bullous and erosive skin lesions. Findings on upper gastrointestinal endoscopy and skin biopsy revealed esophageal squamous cell carcinoma and paraneoplastic pemphigus. Palliative chemotherapy and systemic glucocorticoid were started, however, the patient died of overwhelming sepsis on the ninth day of chemotherapy. This case demonstrates that paraneoplastic pemphigus can occur in esophageal squamous cell carcinoma and could be a cause of morbidity.

Is bullous skin lesion a risk factor for renal amyloidosis in patients with familial Mediterranean fever?

Is bullous skin lesion a risk factor for renal amyloidosis in patients with familial Mediterranean fever?

Effective Treatment of Nodular Amyloidosis with Carbon Dioxide Laser

Nodular amyloidosis is a rare form of localized cutaneous amyloidosis that is characterized by nodules located on the extremities, trunk, genitalia, or face. In treatment regimens, many approaches have been described, including carbon dioxide (CO2) laser therapy. We present a case of a 60-year-old white male with a 20-year history of disseminated waxy, purpuric, yellowish, and bullous skin lesions on the trunk and extremities. The skin changes were accompanied by pain during palpation and were temporarily pruritic. Based on histologic and direct immunofluorescence test findings, the diagnosis of cutaneous nodular amyloidosis was established. Skin lesions were treated with a CO2 laser. During surgery, treated tissue was found to be slightly friable, and there was a little problem with hemostasis that correlated with amyloid infiltration of the dermis and blood vessels. However, after 8 weeks, we observed clinical improvement of all treated areas with the presence of atrophic scars. In the regions of laser therapy, no recurrence of the disease was observed during a 12-month follow-up. Based on these results, we conclude that CO2 laser has a beneficial effect in the treatment of nodular amyloidosis; however, surgery procedures may be associated sometimes with tissue friability and poor hemostasis.

Response of hemorrhagic bullous skin lesions of the breast secondary to primary systemic amyloidosis to a five-drug combination chemotherapy: a case report and review of the literature

Two major types of amyloidosis are primary amyloidosis or amyloid light chain amyloidosis and secondary amyloidosis. Although amyloidosis involves a variety of organ systems including skin, the occurrence of bullous skin lesions is rare. Little is known about the mechanism of blister formation. These blisters are often hemorrhagic and typically occur in the oral mucosa. Only a few case reports have described skin involvement in systemic amyloidosis. The manifestation of bullous lesions on the breast in association with primary amyloidosis has not been previously reported. Therefore, we report a case of cutaneous hemorrhagic bullous of the breast secondary to primary systemic amyloidosis, which may be important for medical oncologists to be aware of this uncommon presentation of plasma cell dysrasias. Furthermore, this case only partially responded to the commonly used multiple myeloma-type regimen, the skin lesions responded completely to a five-drug combination chemotherapy regimen, utilizing immunomodulators, liposomal doxorubicin, cyclophosphamide, bortezomib, and dexamethasone, suggesting that a more aggressive modality of chemotherapy may be necessary to treat such cases.

A 6-Year-Old Girl with Extensive Bullous Skin Lesions

6-year-old previously healthy girl presented with extensive bullous skin lesions involving approximately 25% of her total body surface area (TBSA). She initially presented 9 days earlier with low-grade fever, bilateral non-exudative conjunctival injection, cough, and rash. Her rash appeared as raised, erythematous target lesions that began behind her ears and then progressed to involve her face and body. Her symptoms at first presentation were attributed to erythema multiforme and she was treated with hydroxyzine and olopatadine eye drops. Five days later, fluid-filled bullae developed at the centers of the target lesions and increased in number and size up to the time of admission. Physical examination on admission revealed a nontoxic-appearing girl with extensive fluid-filled bullous lesions of varying sizes behind her ears, around her eyes and mouth, and on her neck, trunk, back, axillae, and perineum (Figures 1 and 2). Mucous membrane involvement included small bullae in her nares, hard palate, and perianal region. She had scattered bullae on her wrists and ankles, although her extremities were relatively spared. The skin at the base of the bullae was erythematous, slightly raised, and pruritic. Initial work-up included a normal complete blood count and electrolytes, clear chest radiograph, and negative herpes simplex virus antigen swab from one of her lesions. She was admitted to the hospital for wound care, pain control, nutritional support, and diagnostic evaluation. She continued to develop bullae during her initial hospital days until approximately 60% of her TBSA was affected (Figure 3, see page 230). Her palms and soles also developed lesions. Further testing and a skin biopsy revealed the diagnosis.

Kutane Arzneimittelreaktionen im Kindes- und Jugendalter

Adverse drug reactions (ADR) occur in nearly 10% of hospitalized children and in about 1.5% of ambulatory pediatric patients. The skin is the most frequently affected target organ in drug hypersensitivity (DH) reactions, which account for 20% of all ADR. Due to its pathophysiological heterogeneity and the ensuing morphological diversity, DH often represents a clinical and therapeutic challenge. Urticarial and maculopapular eruptions are usually restricted to the skin and rarely require systemic treatment or hospital admission once the culprit drug has been withdrawn. However, extracutaneous affections should be ruled out promptly in individuals with polymorphous rashes accompanied by fever and lymphadenopathy as well as in patients with bullous skin lesions. Children affected by severe drug reactions usually require in-hospital surveillance and interdisciplinary supportive therapy.

Methimazole-Induced Bullous Systemic Lupus Erythematosus: A Case Report

Bullous systemic lupus erythematosus (SLE) is a kind of LE-non-specific bullous skin disease that is rarely induced by a medication. We describe the first case of bullous SLE to develop after administration of methimazole. A 31-yr-old woman presented with generalized erythematous patches, multiple bullae, arthralgia, fever, conjunctivitis, and hemolytic anemia. Biopsy of her bulla showed linear deposition of lgG, lgA, C3, fibrinogen, and C1q at dermo-epidermal junction. She was diagnosed as bullous SLE and treated with prednisolone, dapsone, hydroxychloroquine, and methotrexate. Our experience suggests that SLE should be considered as a differential diagnosis when bullous skin lesions develop in patients being treated for hyperthyroidism.

Nafcillin-Induced Bullous Skin Eruption with Granulocytopenia in a Patient with End-Stage Renal Disease

Nafcillin is an antibiotic used for infections due to penicillin-resistant Staphylococcus aureus. In general, the adverse reactions to nafcillin have not been frequent and serious. We report here a new type of adverse reaction to nafcillin in a patient with end-stage renal disease in whom nafcillin caused the unexpected complication of bullous drug eruption and subseqent neutropenia. Three weeks after the start of intravenous nafcillin for the treatment of peritoneal dialysis peritonitis, the patient developed blisters on his right ankle. These became progressive and widespread, with bullae affecting most of the body surface. In addition, neutropenia developed three days after the appearance of bullous skin lesions. Spontaneous recovery of skin lesions and neutropenia was observed after the withdrawal of naficillin. When treating patients with chronic renal failure, physicians should be aware of these rare but potentially severe adverse reactions.

Pemphigoid gestationis

Pemphigoid gestationis is a rare autoimmune subepidermal bullous dermatosis that occurs during pregnancy and postpartum. Diagnosis is made on the basis of the presence of a subepidermal vesicle on routine histologic examination and of linear deposition of complement along the basement membrane zone of perilesional skin. The disorder is accompanied by severe pruritus and polymorphous bullous skin lesions. Clinical diagnosis is confirmed by histology and positive cutaneous immunofluorescence and immunoelectron microscopy tests (linear deposition of C3, with or without immunoglobulin G, along the basement membrane zone, within the lamina lucida, and localized to the proximal part of anchoring filaments of the epidermal fragment of salt-split skin). Enzyme-linked immunosorbent assay for pemphigoid gestationis antibody (BP180) is commercially available. If local treatment fails, systemic corticosteroid therapy should be administered. Oral corticosteroids are the therapeutic mainstay in pregnancy and postpartum. The prognosis is good for mother and child, except that there is a risk of preterm delivery and of moderate fetal growth restriction. Recurrence is possible during subsequent pregnancies. There is no significant maternal morbidity or mortality.

Fulminate mycoplasma infection and severe cutaneous vasculitis: an unusual presentation of complete MBL deficiency

A 12 year old Caucasian female presented with malaise, pyrexia and painful bullous hemorrhagic skin lesions on both upper and lower extremities. She was the second child of a healthy unrelated Caucasian couple and her past medical history was unremarkable. Laboratory tests showed raised inflammatory markers and chest radiograph showed a left lower lobe collapse. She was started on intravenous broad spectrum antibiotics and steroids, with a provisional diagnosis of vasculitis secondary to infection. Autoimmune tests (including ANCA) were negative and she had no evidence of systemic vasculitis. Given the extent of the cutaneous lesions, tests looking at the immune system and for an infection precipitant were performed. Fluid aspirate from the lesion was sterile and skin biopsy showed non specific inflammation. Mycoplasma serology titers were extremely high 1:5200 and the patient had complete mannose binding lectin deficiency (MBL). She was treated with macrolides and was started on chemoprophylaxis. Mannose binding lectin (MBL) is a serum protein believed to play an important role in the innate immune response. Defective MBL production is regarded as the most common immune deficiency in the general population, affecting approximately 5-7% of individuals. Severe MBL usually presents in the early infancy or in early childhood. Late presentation with severe clinical features such as this is not well documented. Patients with primary antibody deficiencies are susceptible to mycoplasma infections (mainly arthritis) due to defective function on the mannose binding lectin pathway. Conversely, mycoplasma is a common pathogen in the paediatric population whilst erythema multiforme associated with mycoplasma infection is a rare presentation. This case is first report in literature where complete MBL deficiency presented in late childhood with severe mycoplasma infection related severe cutaneous vasculitis and no previous history of recurrent infections.

Retinal manifestations in catastrophic antiphospholipid syndrome

AbstractPurpose We present a case report with an ocular vascular event and multiple organ thrombosis and dysfunction in the presence of antiphospholipid antibodies.Methods We report a case of a 79 years old female related visual loss in left eye. She had a venous occlusion and vitreous haemorrhage. In two weeks presented a maculopapular, bullous skin lesions on the legs, rapidly followed by widespread cutaneous necrosis and skin ulceration on all the limbs showing the tendons tissues of the heels. Later, she had an extension of thrombotic events to other vessels in the body. With medical treatment, there was initially a small functional improvement and then a general degradation in 10 weeks, followed by the death of the patient secondary to sever pulmonary emboli in the intensive care unit.Results It was found histopathologic evidence of multiple small‐vessel occlusions, and laboratory confirmation of the presence of antiphospholipid and anticardiolipin antibodies, in high title. It was refractory to anticoagulation, steroids and immunomodulation treatmentConclusion Catastrophic antiphospholipid syndrome is an autoimmune disorder characterized by a rapidly progressive life‐threating disease. Catastrophic occurs when three or more organ systems are affected by thromboses in a very short time less than a week. Roughly 50% of these patients have systemic lupus erythematosus. The overall mortality of this desvasting syndrome is about one‐half.

Index of Suspicion in the Nursery

A 2.94-kg male infant is born at 39 weeks to a 19-year-old G1P0 woman. The pregnancy was complicated by gestational diabetes. The woman had no significant past medical history, including sexually transmitted diseases, and no significant family history. Prenatal screening for group B Streptococcus, Chlamydia, gonorrhea, syphilis, and human immunodeficiency virus were negative. Delivery is complicated by prolonged rupture of membranes for 18 hours, maternal fever (temperature of 38.9°C), and failure to progress. The baby is delivered in good condition by cesarean section. Apgar scores are 8 at 1 minute and 9 at 5 minutes. Vital signs are within normal limits, and physical examination at birth shows no abnormal findings of note. The infant is admitted to the newborn nursery for observation. Blood culture is drawn and empiric antibiotics are started. Hematologic parameters are within normal limits and C-reactive protein measures 0.5 mg/dL. Antibiotics are continued for suspected chorioamnionitis. He is otherwise feeding well, euthermic, and looking well, and his blood culture remains negative. On postnatal day 7, the nurse notices two vesicular skin lesions: one on the right side of the forehead (Fig. 1) and the other on the vertex of the scalp (Fig. 2). Both comprise groups of clustered tiny vesicles that have minimal erythema at their bases. Vital signs and physical examination findings are within normal limits. The result of a simple test supports the diagnosis, and further laboratory investigations confirm the cause. Figure 1. Vesicular lesions on the right side of the forehead. Figure 2. Vesicular lesions on the vertex of the scalp. # Case: Blister in a Baby: Benign or Bothersome? {#article-title-2} ### Differential Diagnosis Vesiculobullous lesions in neonates should never be ignored because the cause could range from benign, self-limited conditions to serious fatal diseases. Early diagnosis and management are crucial …

Epidermolysis bullosa acquisita with moderately severe dysphagia due to esophageal strictures

Epidermolysis bullosa acquisita (EBA) is a chronic, autoimmune condition involving the skin and mucous membranes. Symptomatic mucosal involvement is rare, but can impact on quality of life, due to esophageal strictures and dysphagia. We report a case involving a 60-year-old male presenting with bullous skin lesions on areas of friction on his hands, feet and mouth. Milia were visible on some healed areas. Biopsy showed a subepidermal vesicle. Direct immunofluorescence showed intense linear junctional IgG and C3 at the dermo-epidermal junction. Serological tests also supported the diagnosis of EBA. Screening tests for underlying malignancies were negative. Despite treatment with systemic steroids, the patient developed increasing dysphagia, requiring further investigation with esophagoscopy and a barium swallow. Confirmation of extensive esophageal stricturing prompted adjustment of medications including an increase in systemic steroids and addition of azathioprine. Currently, the patient's disease remains under control, with improvement in all his symptoms and return of anti-basement membrane antibody levels to normal, whilst he remains on azathioprine 150 mg daily and prednisolone 5 mg daily. This case highlights the fact that the treatment of a given patient with EBA depends on severity of disease and co-morbid symptoms. Newer immunoglobulin and biological therapies have shown promise in treatment resistant disease. Considering that long-term immunosuppressants or biologicals will be required, potential side effects of the drugs should be considered. If further deterioration occurs in this patient, cyclosporin A or intravenous immunoglobulin (IV Ig) will be considered. Vigilance for associated co-morbidities, especially malignancies, should always be maintained.