Bullous Formation Research Articles

A widespread blistering eruption: diffuse cutaneous mastocytosis.

Diffuse cutaneous mastocytosis with bullous formation is a rare childhood disease. We report a 5-month-old male who presented with a 3-week history of cutaneous bullae and pruritus. On examination, he had erythema of the cheeks bilaterally and diffuse slightly hyperpigmented, indurated skin on his trunk and abdomen. There were tense vesicles, bullae, and erosions linearly arranged on his trunk and extremities. Both the laboratory and imaging workup were normal. Subsequently, a punch biopsy of a vesicle on the abdomen was obtained and findings confirmed a diagnosis of diffuse cutaneous mastocytosis. An EpiPen(r) was prescribed due to the slightly increased anaphylaxis risk compared to other forms of mastocytosis. There are many purported triggers of diffuse cutaneous mastocytosis and there is currently no known cure which makes management of this disease challenging. This case highlights a rare condition for which official treatment guidelines do not exist. A prompt dermatologic diagnosis is necessary to ensure proper workup and regulation is in place.

Lisinopril dose associated bullous lesion formation

Lisinopril dose associated bullous lesion formation

Stem Cell Therapies for Epidermolysis Bullosa Treatment.

Epidermolysis bullosa (EB) includes a group of rare skin diseases characterized by skin fragility with bullous formation in the skin, in response to minor mechanical injury, as well as varying degrees of involvement of the mucous membranes of the internal organs. EB is classified into simplex, junctional, dystrophic and mixed. The impact of the disease on patients is both physical and psychological, with the result that their quality of life is constantly affected. Unfortunately, there are still no approved treatments available to confront the disease, and treatment focuses on improving the symptoms with topical treatments to avoid complications and other infections. Stem cells are undifferentiated cells capable of producing, maintaining and replacing terminally differentiated cells and tissues. Stem cells can be isolated from embryonic or adult tissues, including skin, but are also produced by genetic reprogramming of differentiated cells. Preclinical and clinical research has recently greatly improved stem cell therapy, making it a promising treatment option for various diseases in which current medical treatments fail to cure, prevent progression, or alleviate symptoms. So far, stem cells from different sources, mainly hematopoietic and mesenchymal, autologous or heterologous have been used for the treatment of the most severe forms of the disease each one of them with some beneficial effects. However, the mechanisms through which stem cells exert their beneficial role are still unknown or incompletely understood and most importantly further research is required to evaluate the effectiveness and safety of these treatments. The transplantation of skin grafts to patients produced by gene-corrected autologous epidermal stem cells has been proved to be rather successful for the treatment of skin lesions in the long term in a limited number of patients. Nevertheless, these treatments do not address the internal epithelia-related complications manifested in patients with more severe forms.

Blistering Distal Dactylitis Resembling a Finger Dipped in Milk Cream

Blistering distal dactylitis is an uncommon condition resulting from superficial skin infection, leading to bullous eruption in the distal phalanx. In rare instances, the extent of the lesion beyond classical presentation is reported. The most commonly described causative agent is Staphylococcus aureus and the condition has a good prognosis if diagnosed and managed early. We report a rare presentation of blistering distal dactylitis in the form of an extensive involvement of the affected finger with purulent bullous formation. The drainage of the pus and appropriate antibiotics led to the good outcome. The knowledge of the characteristic clinical picture of blistering distal dactylitis and its varied presentations shall help it diagnose early in routine clinics.

Painful and oozing bullous lesions in a 74‐year‐old woman

Painful and oozing bullous lesions in a 74‐year‐old woman

Recurrent Spontaneous Pneumothorax Secondary to Marijuana-Induced Extensive Bullous Emphysema

Marijuana is the world's most commonly used illicit drug. Its use is estimated to be around 2% to 5% of the worldwide population and the most commonly used route of use is inhalation. Although not completely elucidated, marijuana has been linked with lung damage and bullous formation. We report a case of a young man with a significant history of marijuana smoking who presented with a recurrent large spontaneous pneumothorax refractory to non-surgical management.

Safety and Efficacy of Prophylactic Closed Incision Negative Pressure Therapy after Acute Fracture Surgery

IntroductionSoft tissue swelling after acute fracture surgery is a challenge which may increase wound dehiscence, delay early range of motion, and increase infection rate postoperatively. This study investigates closed incision negative pressure therapy (ciNPT) using wide-range cover over the incision site and the peripheral swelling trauma zone to promote early active motion and to mitigate joint stiffness, bulla formation, and tendon adhesion. MethodsTwenty-nine patients were enrolled between January 2018 and December 2018. Patients with high-energy soft tissue trauma and comminuted fractures over distal end of limbs (hand and foot; wrist and ankle) or muscle scarcity areas (tibial shaft or patella) were included. ciNPT was applied over closed incisions in the operating room and subatmospheric pressure (-125 mmHg) initiated continuously for 5~7 days. ResultsIn hand and foot patients (n= 8), active motion over all fingers or toes occurred after post-operative Day 2. Mild swelling without any bullous formation was observed over the dorsal aspect of hand. In wrist and ankle patients (n= 16), flexion angle over the finger joints over 90 degrees was observed after 5 days post-surgery. For patients with tibial shaft comminuted fractures with impending compartment syndrome, early active motion of knee and ankle joint was observed as soft tissue swelling and distension pain had subsided after surgery. ConclusionThe prophylactic ciNPT use in the trauma area after surgery reduced postoperative distension pain and improved early range of motion of the tendon and joint in these patients.

Long-term Pulmonary Consequences of Coronavirus Disease 2019 (COVID-19): What We Know and What to Expect.

Long-term Pulmonary Consequences of Coronavirus Disease 2019 (COVID-19): What We Know and What to Expect.

MON-331 Pulmonary Carcinoidosis Presenting with Persistent Pneumothorax

Introduction: Lung neuroendocrine (NET) or carcinoid tumors are a rare group of pulmonary neoplasms characterized by insidious clinical behavior. In this report, we present a case of pneumothorax refractory to conservative management with concomitant pulmonary carcinoidosis. We also discuss the diagnostic and management approach followed in such cases. Case: 80 year-old male with history of asthma and Type 2 DM presented to the ER with worsening cough and shortness of breath, and was found to have a left sided spontaneous pneumothorax. Chest tube was inserted. However, due to persistent leak, PleurX catheter was placed, and patient was discharged home afterwards. However, he presented with subcutaneous emphysema on the same day and was subsequently readmitted. Persistent pneumothorax was managed conservatively. PleurX catheter was attached to underwater seal with negative pressure and respiratory status was optimized given evidence of asthma exacerbation. Treatment with Methylprednisolone IV, Montelukast, Tiotropium, Mometasone Furoate/Formoterol inhalers was initiated. However, air leak persisted despite conservative management for 13 days. CT surgery elected to proceed with VATS. Multiple wedge resections were performed along with partial pleurectomy, lysis of adhesions and decortication of left upper lobe. Pathology revealed a sub-pleural 5-mm carcinoid tumor with solid nests of cells along with lung tissue with emphysematous changes, pleural scarring and bullous formation. No nuclear pleomorphism or mitotic figures were noted. Immunoperoxidase stains were positive for TTF-1, synaptophysin, chromogranin and CD56 and negative for CK5/6, calretinin and P-40. Serum Chromogranin A (CgA) was elevated 431 ng/l. Post-op course was uncomplicated and patient was discharged to a rehab facility. Discussion: Lung NETs account for 1-2% of all lung malignancies in adults and 30% of all NETs. Incidence ranges from 0.2-2/100,000 population per year. While some patients may be asymptomatic, others present with obstructive symptoms due to mass effect. Incidence of pneumothorax as a complication of lung neoplasms is rare (0.05-1.4% of all pneumothoraces). Clinical presentation includes carcinoid syndrome, Cushing's syndrome and acromegaly. Diagnosis is confirmed either by bronchoscopic biopsy (for central lesions) or by transthoracic needle biopsy (for peripheral lesions). For patients with localized lung NETs, surgical resection is the treatment of choice. Lung NETs have an excellent prognosis following surgical resection, with 5-year survival rates of 87-100%. Immunohistochemical identification utilizes tissue markers like synaptophysin, neuron-specific enolase, and chromogranin. Serum CgA is usually elevated and is used as a marker to assess disease progression, response to therapy, or recurrence after surgical resection.

Bronchial atresia in an accessory lobe caused tension pneumothorax

We report a case in which bronchial atresia in an accessory lobe caused tension pneumothorax. The atretic bronchus was found in an accessory lobe (right S6) that had been incompletely isolated from the right lower lobe during the fetal period. The accessory lobe was overinflated, leading to bullous formation due to the trapping of air. Video-assisted excision of the posterior lobe was successfully performed without any perioperative complications. The pathological findings were compatible with congenital bronchial atresia. This is the first report of bronchial atresia in an accessory lobe. We must keep this lesson in mind because such cases can suddenly develop serious complications.

A Case of Staphylococcal Scalded Skin Syndrome in a 1-Year Old Boy with Otitis Media

Introduction: Staphylococcal scalded skin syndrome (SSSS), also known as Ritter’s Disease, is a severe disease seen mostly in newborns and children aged <5 years, and the mortality rate might reach to 4% despite an appropriate treatment. Exfoliative toxins cause intraepidermal separation in the stratum granulosum and lead to a condition that is characterized with widespread epidermolysis all over the body and bullae with positive Nikolsky’s sign. SSSS might be fatal due to loss of skin barrier. Immediate initiation of an anti-staphylococcal drug therapy is required for the treatment of this toxin-dependent disease, which is one of the emergencies of dermatology practices.Case Report: A 1-year-old boy was referred to a dermatology clinic from pediatric emergency service with widespread epidermolysis all over the body, including erosion and bullous formation that had been appeared in the inguinal region. SSSS was suspected in this case, and skin biopsy was performed to confirm the diagnosis. He was hospitalized in the Pediatric Infectious Diseases Service, and treatment comprising intravenous administration of (i.v.) vancomycin (40 mg/kg/day) and 3 gIVIG ( intravenous immunoglobulin) was initiated.Conclusion: Staphylococcus aureus, a rather rare agent of otitis media, is remarkable in terms of causing the complication of SSSS, which is very rare. In our case report, we aimed to remark to this rare condition (association of otitis media and SSSS)

P68: THE PATHOGENIC ROLE OF AUTOREACTIVE IGE IN BULLOUS PEMPHIGOID: A UNIQUE CASE SUPPORTING CONCLUSIONS FROM EXPERIMENTAL DATA

Bullous pemphigoid (BP), a blistering dermopathy, is a prototypic antibody‐mediated disease. Detection of IgG autoantibodies against hemidesmosomal proteins BP180 and/or BP230 are diagnostic and levels correlate with disease activity. Therapies include corticosteroids and oral immunosuppressants, while intravenous immunoglobulin (IVIG) and rituximab are reserved for resistant cases. These observations together support a central role for B cells and autoreactive IgG in BP pathogenesis. We describe a patient with persistent biopsy‐proven severe BP on a background of stable HIV infection with long‐term viral suppression and normal CD4 T cell counts, type 2 diabetes and chronic renal failure. The disease was widespread and refractory to standard first line therapy, IVIG and also rituximab, despite peripheral B cell depletion. Use of the monoclonal anti‐IgE antibody omalizumab resulted in a rapid resolution, within days, of pruritis and bullae formation. Disease remission has been maintained with ongoing Omalizumab therapy despite persistent high level junctional anti‐skin IgG antibodies. To our knowledge this is the first case of BP responsive to omalizumab after failure of rituximab to be described in the literature. Omalizumab does not currently have an established role in treatment of BP, although this case adds to previous case reports suggesting a role for omalizumab as a novel BP treatment. This is also supported by recent experimental data demonstrating a role for autoreactive IgE in bullous formation and correlation with disease activity. Selection of patients with BP who would benefit from anti‐IgE therapy remains to be defined, although given the speed of response and the low risk associated with its use, a therapeutic trial may be warranted in severe and/or resistant cases, particularly to reduce the side‐effects of standard therapies.

Fever in Steven Johnson Syndrome , a case report

Steven Johnson syndrome is a life threatening clinical condition that is usually manifested with fever , rash, bullous formation, oral and ocular lesions and genital and anal lesions. This syndrome, in majority of cases begins with influenza-like symptoms and continues with a red rash and blisters . Then the top layer of the affected skin dies and sheds.[1,2] This case report is about a 28 year old male patient who was referred to the Infectious Disease Hospital of University Hospital Center Mother Theresa Tirana with the suspected diagnosis of Hemorrhagic Fever. The patient referred 5 days of continuous fever, malaise, rash, myalgia, headache, and nausea As soon as the patient was admitted, we immediately performed specific serologic tests, therapeutic regimen for the suspected diagnosis of hemorrhagic fever and complementary examination. During the first 10 hours, we noticed that the rash became more intense and other skin lesion and bullous formation appeared. The patient was admitted in ICU and initial diagnosis of Steven Johnson Syndrome was made. This case report elaborates the particular clinical appearance and the misdiagnosis that was associated with Steven Johnson Syndrome.[3] Specific conditions as the prolonged prodromal phase of SJS, the strong epidemiological data for hemorrhagic fever, the absence of information for misuse of drugs led us towards a “sure diagnosis” in the first place. However, the close follow-up in the ICU revealed new clinical signs and rapid differential diagnosis was done. Due to the intensive care therapy, which was applied for Hemorrhagic fever at first place, we managed to overcome complications and safe the patient life.[4]

Acute intensive care unit management of mustard gas victims: the Turkish experience

Purpose: Sulphur mustard (SM) is an highly toxic and vesicant chemical weapon that was used in various military conflicts several times in the history. The severity of ocular, dermal, and pulmonary symptoms that may appear following a characteristic asymptomatic period are depending on the SM concentration and exposure duration. The aim of this study is to present the clinical features and share the intensive care unit (ICU) experiences for the medical management of mustard gas victims.Materials and methods: Thirteen Free Syrian Army soldiers near Al-Bab region of North Syria were reportedly exposed to oily blackish smoke with garlic smell due to the explosion of a trapped bomb without causing any blast or thermal effect on 26th November 2016. None of them wore any chemical protective suits or gas masks during explosion. Since they observed skin lesions including bullous formation next day, they were admitted to the Turkish Field Hospital at the Turkish – Syrian border and then evacuated to the State Hospital of Gaziantep Province, Turkey for further management. Eight victims who were very close to point of explosion suffered burning eyes, sore throat, dry cough and dyspnoea after the chemical attack.Results: On admission to hospital, all cases had conjunctivitis, hoarseness and bullae on various body areas. Blepharospasm and opacity were found in 8 patients and 5 of them had corneal erosions and periorbital oedema. Temporary loss of vision in 4 cases lasted for 24 h. Multiple fluid-filled blisters were observed especially on the scalp, neck, arms and hands, where direct skin exposure to the agent occurred. A definitive clinical care and infection prophylaxis measures along with the burn treatment and bronchodilators for respiratory effects were applied in ICU. Two patients received granulocyte-colony-stimulating factor due to the SM-mediated bone marrow suppression on the 16th day of exposure and one of them died because of necrotic bronchial pseudomembrane obstruction resulting in cardiopulmonary arrest.Conclusions: SM was first used during the First World War and it is still considered one of the major chemical weapons recently used by non-state actors in Syria and Iraq. In case of SM exposure, medical treatment of SM-induced lesions is symptomatic because no antidote or causal therapy does exist even though SM is very well known for over 100 years. However, clinical management in intensive care medicine of SM victims have improved since the 1980s, this study which is one of the largest recent SM-exposed case series since that time is important for the contribution to the clinical experience.

Delayed Allergic Cutaneous Hypersensitivity to Icodextrin

Rashes pose a unique challenge to general internists. Here we present a case of a 51-year-old female with end-stage renal disease (ESRD) on peritoneal dialysis who presented to a General Internal Medicine clinic with a vesiculobullous rash on palms and soles 6 months after using icodextrin dialysate, a widely used osmotic agent in peritoneal dialysis. The investigations were positive for an elevated eosinophil count, elevated IgE level and a skin biopsy consistent with an allergic reaction. Her symptoms and labs normalized after stopping icodextrin. There are some reports of allergic reactions to icodextrin which usually occur within a month of initiation of the agent and are self-limiting. Here we hope to report a case of a reaction that occurred 6 months after using icodextrin.&#x0D; Resume&#x0D; Les éruptions cutanées posent un défi particulier aux internistes généraux. Nous rapportons ici le cas d’une femme de 51 ans atteinte d’insuffisance rénale terminale (IRT), sous dialyse péritonéale, qui se présente à une clinique de médecine interne générale avec une éruption cutanée vésiculo-bulleuse sur les paumes et la plante des pieds. Depuis six mois, la patiente utilise un dialysat d’icodextrine, un agent osmotique largement utilisé en dialyse péritonéale. Les analyses ont révélé une numération élevée des éosinophiles, un taux élevé d’IgE et une biopsie cutanée a indiqué la présence d’une réaction allergique. Les symptômes et les résultats d’analyses se sont normalisés après l’arrêt de l’icodextrine. Des réactions d’allergie à l’icodextrine ont déjà été rapportées, mais celles-ci surgissent habituellement dans le mois suivant le début de l’usage de l’agent et se résorbent d’elles-mêmes. Nous désirons signaler un cas de réaction survenue six mois après le début de l’utilisation de l’icodextrine.&#x0D; &#x0D; &#x0D;

Childhood mastocytosis: results of a single center.

We aimed to retrospectively evaluate histopathological, demographic and clinical findings of children with mastocytosis diagnosed with mastocytosis in our clinic. The files of 21 patients diagnosed with mastocytosis between 2000 and 2014 in our clinic were retrospectively analyzed. All patients had cutaneous mastocytosis, 19 patients had urticaria pigmentosa and 2 patients had mastocytoma. The male-female ratio was: 1/1.6. The median age for onset of disease was 12.1 months and the disease occured in the newborn period in 3 patients. While all patients had eruption, 10 patients had pruritis, 1 patient had a bullous formation, 1 patient had abdominal pain and 1 patient had attacks of redness throughout the body and a sense of burning in the chest. Two patients had a positive familial history. The diagnosis was confirmed with skin biopsy in all patients. The median follow up time of the patients were 5 years. The patients were treated with H1, H2 antihistaminics, local moisturizing creams and topical corticosteroid drugs. The lesions resolved completely in 4 patients who reached to puberty and 7 patients had marked improvement in a 5.5 year-follow-up period. Ten patients had stabile lesions in a 3.6 year-follow-up period. Most cases of childhood mastocytosis are observed in the form of cutaneous mastocytosis. The prognosis is good; the disease limits itself and is prone to regress in the adolescent period.

A case of soft fibroma of the nipple with a cauliflower-like appearance.

Dear Editor: Soft fibroma (acrochordon, fibroepithelial polyp, or skin tag) is a common pedunculated skin neoplasm and usually appears as a furrowed papule, filiform lesion, or large bag-like protrusion. Soft fibromas primarily occur on the neck, axillae, and groin. However, it may present at unusual sites of the body such as the penis, urethra, and vulva. Moreover, a few reports on soft fibromas occurring on the breast and nipple have been published1. Herein, we report a rare and interesting case of a soft fibroma arising from the nipple, showing an unusual cauliflower-like appearance. A 51-year-old obese woman presented with a 25-year history of a painless pedunculated polyp originating from her right nipple. The lesion measured 2.4×2.3×1.4 cm and demonstrated a verrucous and cauliflower-like surface (Fig. 1A, B). Neither acanthosis nigricans nor epidermal nevus was observed on the adjacent skin. Dermoscopy revealed irregular epidermal projections and focal dotted vessels (Fig. 1C). Total surgical excision of the polyp, sparing the right nipple, was performed. Histopathological analysis showed papillomatosis, hyperkeratosis, and regular acanthosis of the epidermis with fibrocollagenous tissue in the dermis (Fig. 1D). On the basis of these findings, we diagnosed the lesion as a soft fibroma. Fig. 1 (A) A large solitary pedunculated polyp originating from the right nipple. (B) The polypoid lesion measuring 2.4×2.3×1.4 cm and demonstrating a cauliflower-like surface. (C) Dermoscopic findings showing irregular epidermal projections ... There have been only a few reports on soft fibromas of the nipple presenting with bullous formation or comprising a large elastic mass with a short pedicle2,3. Furthermore, there has been no report of a large and cauliflower-like soft fibroma of the nipple in the Korean literature. Because the clinical manifestations of soft fibroma of the nipple are nonspecific, histological examination is required for differential diagnosis, including pedunculated seborrheic keratosis, pleomorphic fibroma, nevoid hyperkeratosis of nipple and areola, and verruca vulgaris. In our case, the unusual clinical presentation of the cauliflower-like nipple tumor made the diagnosis of the lesion difficult. However, the histopathological findings provided important clues for making an accurate diagnosis. Thus, we have described a rare and interesting case of a cauliflower-like soft fibroma of the nipple.

Acantholytic dermatosis of the vulva

Acantholytic dermatosis of the vulva is a rare condition, presenting with papular eruption in the genital area without history of Darier disease or Hailey-Hailey disease. We report a case with a papular pruritic eruption in the region of the vulva, coalescing into plaques. Biopsy specimen showed irregular acanthosis with an area of split-like bullous formation in the deeper part of the epidermis, as well as acantholytic cells, marked hypergranulosis and hyperkeratosis, compatible with the rare diagnosis of acantholytic dermatosis of the vulva. We review the clinical and histological characteristics of this uncommon disease.

Electrical stimulation-induced bullous formation in a pemphigoid diabetic patient: a case report

In 2002, the use of electrical stimulation was approved as an adjuvant treatment for non-healing ulcers. A 50-year-old man with type 2 diabetes of 3 years’ duration was referred to our diabetic foot clinic. The patient was a known case of bullous pemphigoid (BP) for the past 15 years and was under steroid therapy (20 mg/day Prednisolone). Electrical stimulation was used as one of the ancillary methods for the treatment of his chronic diabetic foot ulcers. After two sessions, physical examination revealed a bullous eruption (approximately 14 cm) on an erythematous base in the right leg. The literature is devoid of any case of bullous induced by electrical stimulation in pemphigoid diabetic patients. Further studies are required to validate our observation and fully explain the pathogenesis.

Nodular localized primary cutaneous amyloidosis: a bullous variant

Primary cutaneous amyloidosis describes a group of disorders in which amyloid is deposited in the skin without evidence of systemic involvement. Nodular localized primary cutaneous amyloidosis (NLPCA) is a rare form of these skin-restricted amyloidoses. We present an unusual case of NLPCA in a 51-year-old man, who had clinical and histopathological evidence of subepidermal bullous formation, a unique feature in NLPCA. The possible pathogenesis of this change is discussed.