Skin Denudation Research Articles

PA12 A case of pustular psoriasis in epidermolytic ichthyosis: coincidence or common pathway?

Abstract An 8-year-old boy with epidermolytic ichthyosis (EI) due to a heterozygous KRT1 mutation presented with a striking bullous skin eruption. Microbiological swabs were sent, and oral antibiotics were prescribed for suspected bullous impetigo. He deteriorated clinically, developing a fever and more extensive pustular rash reminiscent of generalized pustular psoriasis (GPP). Skin biopsies from two distinct cutaneous sites revealed histology strongly supportive of pustular psoriasis. Oral ciclosporin was commenced in addition to intensive topical emollient therapy. Following widespread desquamation, the pustular eruption fully resolved. Recurrence upon weaning of ciclosporin necessitated the introduction of ustekinumab. The rash remains controlled on this therapy. The EI is caused by heterozygous mutations in KRT1 or KRT10, and presents at birth with erythroderma and widespread skin denudation. The erythema and skin fragility abate in early childhood, giving way to prominent hyperkeratosis. Severe palmoplantar keratoderma is seen in those with KRT1 mutations. The GPP is a rare but life-threatening condition characterized by sterile pustules with or without systemic involvement. Interleukin (IL)-36-mediated inflammatory signalling plays a pathogenic role, as evidenced by loss of function mutations in IL36RN, which encodes an IL-36 receptor antagonist (Marrakchi S, Guigue P, Renshaw BR et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 2011; 365:620–8), and the efficacy of IL-36 receptor inhibitor spesolimab in a recent phase II trial. Both EI and GPP are rare, and the confluence of these entities in this case is striking. Four other cases in the literature describe a GPP-like eruption in patients with congenital ichthyoses. The coexistence of these conditions might be explained by their immunological profiles. Studies of the skin of patients with congenital ichthyoses have shown immune dysregulation with T helper 17/IL-23 skewing, and upregulation of associated inflammatory cytokines, including IL-36γ. The latter encodes for an IL-36 receptor agonist (Paller AS, Renert-Yuval Y, Suprun M et al. An IL-17-dominant immune profile is shared across the major orphan forms of ichthyosis. J Allergy Clin Immunol 2017; 139:152–65). Thus, increased IL-36 receptor agonist activity in congenital ichthyosis could theoretically lead to GPP in genetically predisposed individuals with already upregulated IL-36 receptor-mediated signalling.

Distal Urethral Advance and Glanduloplasty (DUAGPI) for Distal Hypospadias: A 15 Years Experience.

Meatal advancement with glanduloplasty (MAGPI) has been in the recent years one of the most frequently surgical techniques used for the correction of distal hypospadias. Multiple modifications have been described to improve the results ofthis technique. In 2004 we presented the initial results of the New Modern MAGPI or DUAGPI (distal urethral advancement and glanduloplasty) as a surgical alternative to the original MAGPI. The objective of the present study is to evaluate the applicability, safety and the cosmetic results obtained with this technique, as well as the long-term complications. Retrospective and descriptive study of patients with distal hypospadias, corrected using the DUAPI technique, between 2002 and 2018. Initially, an artificial erection was performed, the absence of curvature in all patients is proven. A skin denudation was performed up to the base of the penis in those children with a bend greater than 30. Second, the distal urethral disconnection was performed at the posterior and lateral urethral level. A glandular triangular section is made to accommodate the distal urethra and to allow a conical appearance with a final glandular. Finally, the fixation of the distal urethral to the glandular tip and the anterior glandular closure is performed. We performed an annual follow-up of all patients. The analysis of all the variables collected during surgery and follow-up was done with SPSS 22 statistical package. 90 patients meet inclusion criteria and were included in the study (32 glandular and 58 coronal). The mean surgical time was 47 minutes (Range: 37-71 minutes). The mean follow-up was 9.4 years (2.1-15 years). 2 patients had meatal stenosis after surgery, 4 mild glandular retraction, and 2 urethrocutaneous fistula. Six of the 8 patients with a complication were successfully reoperated. 92% of the patients and their families were satisfied with the cosmetic result obtained after surgery (83 patients). DUAGPI technique is simple, reproducible and optimal for most distal hypospadias. It is a safe technique, with a low complication rate. The final aesthetic result is satisfactory in most cases, with a conical glans and a natural cosmetic appearance.

A Review of Fixed Drug Eruption with a Special Focus on Generalized Bullous Fixed Drug Eruption.

Fixed drug eruption (FDE) is a cutaneous adverse drug reaction characterized by the onset of rash at a fixed location on the body each time a specific medication is ingested. With each recurrence, the eruption can involve additional sites. Lesions can have overlying vesicles and/or bullae, and when they cover a significant percentage of body surface area, the eruption is referred to as generalized bullous fixed drug eruption (GBFDE). Due to the widespread skin denudation that can be seen in this condition, GBFDE may be confused clinically with Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). While treatments described for GBFDE include supportive care, topical and/or systemic steroids, and, recently, cyclosporine, the mainstay of management involves identifying and discontinuing the causative drug. This review article will provide an overview of FDE with an emphasis on its generalized bullous variant.

Nivolumab-associated Stevens-Johnson syndrome in a patient with lung cancer

Nivolumab is a fully human immunoglobulin G4 immune checkpoint inhibitor antibody approved for use in the treatment of several malignancies such as lung cancer. Cutaneous reactions to checkpoint inhibitors are frequent, appearing in approximately 40% of patients. Although most of the reactions are well tolerated, these drugs can lead to severe cutaneous adverse reactions, but a quick recognition of the symptoms can significantly decrease their mortality. In this case report, we describe a patient with metastatic squamous lung cell carcinoma suffering from nivolumab-induced Stevens-Johnson syndrome with severe skin denudation and mucosal involvement.

Scrotal Reconstruction—a Simple Method for General Surgeons

Complete denudation of scrotal skin as an aftermath of Fournier’s gangrene is a vexing surgical problem which may leave behind one or both the testes hanging out from the perineum. Healing of this area by second intention not only results in a bad perineal cicatrix with poor cosmetic appearance of scrotum but also potentially endangers the survival of testes by the way either testicular desiccation or cord entrapment in the cicatrix. A new and simple approach is described hereunder, which would achieve the desired goals of both scrotal reconstruction and testicular preservation with relative ease and this procedure could be performed by a general surgeon in a modest surgical setup.

Antibiotic sensitivity and clinical outcomes in staphylococcal scalded skin syndrome.

Staphylococcal scalded skin syndrome causes widespread skin denudation primarily in infants<1 year old. Selection of empiric therapy is complicated by rising rates of antibiotic resistance in community-acquired staphylococcal infections. Consistent with a previous study, this retrospective review found that SSSS-associated isolates were more likely to be clindamycin-resistant and less likely to be methicillin-resistant compared to overall staphylococcal infections. We favor cephalosporins and penicillinase-resistant penicillins (eg, oxacillin) for empiric management of SSSS, with consideration of adding MRSA coverage in communities with high MRSA prevalence or failure to improve following several days of treatment.

Influence of Backscatter Radiation on Cranial Bone Fixation Devices

Postoperative radiation can cause ulcer formation, leading to the denudation of skin over alloplastic materials. The influence of backscatter radiation from fixation devices has not been investigated. The aim of this study is to evaluate backscatter dose variations for different cranial bone fixation devices in an experimental model designed to simulate postoperative radiotherapy. The authors assessed the radiation backscatter doses associated with resorbable (PLLA-PGA) and titanium plates. The samples were irradiated with 6 and 10 MV photon beams from a linear accelerator. Measurements were obtained using an ionization chamber and radiochromic films cut from the same batch. As a result, the backscatter radiation of water and PLLA-PGA proportionally decreased as the depth increased. However, the backscatter radiation of the titanium plate increased just above the plate. This depth lies in the region of the scalp. Each material showed a dose of radioactivity that was higher at 10 MV than that at 6 MV. These devices showed a significant difference, which suggested that these materials amplified the dose compared with water at 6 MV. In conclusion, it is supposed that PLLA-PGA should be used to fix the cranium to decrease the potential for radiation ulcers.

An Experimental Lymphedema Animal Model for Assessing the Results of Lymphovenous Anastomosis.

The lymphatic system is the least studied of the body's systems, and as a result its contribution to human health and diseases is not well known. The failure of the lymphatic system to drain extravasated fluid efficiently leads to accumulation of lymph in the interstitial tissue, thus causing lymphedema. Despite developments in microsurgery procedures, the results of lymphedema treatment remain disappointing. The aim of this study was to develop an experimental animal model that is able to reproduce the disease and to apply various surgical and therapeutic approaches. Lymphedema was induced in New Zealand white rabbits by a total skin denudation and destruction of the lymph channels via microsurgery in the right ear, leaving the left ear as control. Lymphedema generation was observed between days 7 and 15 after intervention. On day 15, a terminal venous lymphatic anastomosis was performed on the posterior edge of the right ear. Serial measurements of ear thickness were made during the study. Ear thickness was significantly (p < 0.05) increased after lymphedema induction compared to baseline (3.2 ± 0.4 vs. 1.3 ± 0.04 mm). After anastomosis, ear thickness was reduced (2 ± 0.26 mm). This animal model of lymphedema induction in the ear proved to be a reproducible alternative for studying new approaches to lymphedema treatment and for developing microsurgical skills.

Staphylococcal Scalded Skin Syndrome in Child. A Case Report and a Review from Literature.

Staphylococcal scalded skin syndrome (SSSS) is the medical term used to define a skin condition induced by the exfoliative toxins produced by Staphylococcus aureus. The disorder is also known as Ritter disease, bullous impetigo, neonatal pemphigus, or staphylococcal scarlet fever. The disease especially affects infants and small children, but has also been described in adults. Prompt therapy with proper antibiotics and supportive treatment has led to a decrease in the mortality rate. The current case report describes the clinical progress of a patient with generalized erythema and fever, followed by the appearance of bullous lesions with tendency to rupture under the smallest pressure, and with extended areas of denudation. The patient aged four years and six months was admitted to our clinic to establish the aetiology and treatment of a generalized bullous exanthema, followed by a skin denudation associated with fever and impaired general status. Based on clinical and paraclinical examinations a diagnosis of Staphylococcal scalded skin syndrome was established which responded favourably to antibiotic treatment, hydro-electrolytic re-equilibration, and adequate local hygiene. Staphylococcal infection can represent a problem of significant pathological importance sometimes requiring a multidisciplinary approach involving paediatricians, dermatologists, infectious diseases specialists, and plastic surgeons.

Case 1: Newborn with Skin and Other Abnormalities

A female infant is born precipitously via vaginal delivery after spontaneous rupture of membranes. The mother is a 24-year-old African American woman whose history includes 2 cesearean deliveries with no other medical concerns. The current pregnancy is complicated by limited prenatal care. The gestational age is unknown. The infant’s birthweight is 1,870 g, and her Ballard score is consistent with an estimated 32 weeks of gestation. In the delivery room, the neonate has a spontaneous cry but with frothy oral secretions and a low heart rate requiring positive pressure ventilation, intubation, and brief cardiopulmonary resuscitation. Apgar scores are 5 and 7 at 1 and 5 minutes, respectively. Physical examination is significant for extensive blistering of her face, neck, chest, and extremities. She is placed in a plastic bag for skin protection, thermoregulation, and reduction of insensible water losses. The patient is then transferred to the NICU, where mechanical ventilation is ordered and umbilical line placement is attempted. Admission examination reveals sloughing of oral mucosa as well as diffuse sloughing of perioral skin, right eyelid contraction, auricle skin denudation with hypoplastic and posteriorly rotated bilateral ears, and depressed nasal bridge (Fig 1). The extremities show congenital localized absence of skin (Fig 2), nail dystrophy, and muscle wasting (Fig 3). The skin is dressed with moisturizer and sterile dressing. Figure 1. Denudation of eyelid, ears, and perioral and oral mucosa with depressed nasal bridge. …

Comparison of Medical Adhesive Tapes in Patients at Risk of Facial Skin Trauma under Anesthesia.

Introduction. Adhesive tapes are used for taping eyelids closed and securing endotracheal tubes during general anesthesia. These tapes can cause facial skin injury. We compared the incidence of facial skin injury and patient satisfaction with different tapes used. Methods. A total of 60 adult patients at risk of skin trauma were randomized to use 3M™ Kind Removal Silicone Tape or standard acrylate tapes: 3M Durapore (endotracheal tube) and Medipore (eyelids). Patients were blinded to tape used. Postoperatively, a blinded recovery nurse assessed erythema, edema, and denudation of skin. Anesthesiologist in charge also assessed skin injury. On postoperative day 1, patients rated satisfaction with the condition of their skin over the eyelids and face on a 5-point Likert scale. Results. More patients had denudation of skin with standard tapes, 4 (13.3%) versus 0 with silicone tape (p = 0.026) and in anesthesiologist-evaluated skin injury 11 (37%) with standard versus 1 (3%) with silicone (p = 0.002). No significant differences were found in erythema and edema. Patient satisfaction score was higher with silicone tape: over eyelids: mean 3.83 (standard) versus 4.53 (silicone), Mann-Whitney U test, p < 0.001; over face: mean 3.87 (standard) versus 4.57 (silicone) (p < 0.001). Conclusion. Silicone tape use had less skin injury and greater patient satisfaction than standard acrylate tapes.

Penile Strangulation by Self‐Placement of Metallic Nut: Corporal Cavernosa Tissue Showed Resilience to Thermal Burns Sustained at Removal

We report a case of a 37-year-old male who presented with a 12-hour history of a strangulating 2cm wide by 0.8 cm thick metallic nut on the penile shaft at the peno-scrotal junction. Unlike instances where these metallic objects are placed to enhance sexual stimulation this nut was rather placed to prevent intercourse. A Bosch electric circular grinder was successfully used for removal but a thermal burn to the penile tissues was sustained in the process as the hardness of the nut required a high energy to cut and its thickness did not allow for effective cooling during the process of removal. This resulted in a circumferential denudation of penile skin, a urethro-cutaneous fistula at the peno-scrotal junction and a mid-bulbar urethral stricture. The penile wound was subsequently covered with a split skin graft with a delayed closure of the urethrocutaneous fistula and a buccal mucosa patch urethroplasty for the mid bulbar stricture. Despite the degree of thermal burns sustained the patient has maintained good erectile function with grade four rigidity. The tunica albuginea and the underlying corpora cavernosa have shown a significant degree of resilience to thermal burns compared to the corpora spongiosum where the thermal burns led to a urethrocutaneous fistula.

A case of toxic epidermal necrolysis in a young infant successfully treated with intravenous immunoglobulin

Toxic epidermal necrolysis (TEN) is a severe adverse cutaneous drug reaction characterized by widespread keratinocyte death. Clinically, TEN is characterized by rapidly progressing erythematous, purpuric rash to widespread blistering and denudation of skin and severe mucositis, with fatal complications like sepsis and multiorgan failure. TEN is considered to be rare in infants and usually has a fatal outcome due to sepsis. As yet, only few reports have been documented in the literature. We hereby report a case of TEN in a young infant successfully treated with intravenous immunoglobulin (IVIG).

Scrotal necrosis to total de-gloving injury of the male genitalia: an experience from Sub-Saharan Africa

Two patients with very different aetiologies of their genital injuries are presented: one lost his scrotal skin as a result of Fournier&amp;rsquo;s gangrene, the other experienced complete denudation of scrotal and penile skin plus the amputation of his glans penis through an agricultural machinery. The placement of denuded gonads in thigh pouches and delayed skin grafting provide safe treatment options in a low budget setting of a Sub-Saharan country.

Limitations of 3‐Dimensional Sonography in the Prenatal Evaluation of a Skin Denudation Syndrome

Limitations of 3‐Dimensional Sonography in the Prenatal Evaluation of a Skin Denudation Syndrome

Integra Bilayer Matrix Wound Dressing Closure of Large Periorbital Traumatic Wound

We report the first use of the Integra Bilayer Matrix Wound Dressing (Integra LifeSciences Corp), a collagen sheet with glycosaminoglycans and a silicone layer, in an innovative reconstruction approach to devastating traumatic tissue loss in the periocular area. A 36-year-old woman was involved in a motor vehicle crash with a resultant large defect from the medial canthus to the temporal fossa and from the pretarsal skin to the brow. There was denudation of skin and soft tissue to the bone at the superolateral orbital apex. The severity of tissue loss precluded placement of an autograft or allograft; thus, a skin substitute was instead used, with a successful reconstructive outcome. Application of the newer bioengineered skin products for full-thickness skin wounds should be considered for reconstruction of the periocular area.

Anti‐IL‐33 antibody has a therapeutic effect in a murine model of allergic rhinitis

Interleukin (IL)-33 is involved in the Th2 immune response and could play an essential role in nasal allergy. Therefore, we aimed to investigate the therapeutic potential of anti-IL-33 for allergic rhinitis (AR). Twenty-four BALB/c mice were used. In group A (control group, n = 6), mice were sensitized and challenged with saline. Group B [ovalbumin (OVA) group, n = 6] mice received intraperitoneal and intranasal OVA challenge. In group C (control IgG group, n = 6), mice were injected intraperitoneally with rabbit control IgG before OVA challenge. In group D (anti-IL-33 group, n = 6), anti-IL-33 was injected before challenge. We evaluated the number of nose-scratching events and external morphology; serum total and OVA-specific IgE; number of eosinophils, neutrophils, and lymphocytes in bronchoalveolar lavage (BAL) fluid; histopathologic examination of nasal cavity; and IL-4, IL-5, and IL-13 in BAL fluid. Anti-IL-33 treatment significantly reduced the nose-scratching events and ameliorated skin denudation. Serum total and OVA-specific IgE was significantly decreased in group D. The number of eosinophils in BAL fluid was also significantly decreased. Eosinophilic infiltration in the nasal cavity was significantly decreased in group D. IL-4, IL-5, and IL-13 in BAL fluid were also significantly decreased after treatment. Anti-IL-33 antibody has a therapeutic potential for experimental AR.

Congenital herpes simplex II (HSV-2) with limb abnormalities

Background and aimsThe classical presentation of congenital HSV infection is grouped vesicles on an erythematous base and/or widespread skin erosions. Associated limb malformations have been reported only once.MethodsWe present a...

An innovative local treatment for staphylococcal scalded skin syndrome

Staphylococcal scalded skin syndrome (SSSS) is the clinical term used to describe a range of blistering skin disorders induced by the exfoliative toxins of Staphylococcus aureus and prevalently affects neonates, infants and toddlers who lack antibodies to S. aureus toxins. SSSS is a highly contagious disease and is characterised by erythema and fever, followed by the formation of large fragile superficial blisters, which rupture only to leave extensive areas of denuded skin. A diagnosis of SSSS relies on the clinical picture, as well as on histological and microbiological findings. Neonates and young infants are particularly susceptible to a lack of the protective skin barrier, which may cause excessive protein and fluid losses, hypothermia and secondary infection. Due to a complete denudation of skin, the patients also suffer from almost unbearable pain. In our communication, we present an innovative temporary coverage of the denuded skin with Suprathel (PolyMedics Innovations GmbH, Denkendorf, Germany). Suprathel relieves pain, prevents heat loss and secondary infection, accelerates wound healing, does not need to be changed and makes daily care easy for the nurses and is well tolerable for the patient.

Skin Ulcerations in a Preterm Newborn

A preterm female infant has had skin ulcerations since birthCongenital absence of skin(1):Skin biopsy and ocular, rectal, and oral swabs were positive for HSV-1 by polymerase chain reaction (PCR). Skin biopsy revealed abnormal hair follicles that had extensive dystrophic calcification and total loss of follicular epithelium. The placenta exhibited areas of acute necrotizing villitis as well as multinucleated giant cells with nuclear staining positive for HSV-1. Given the grave neurologic and overall prognosis, the infant’s care was redirected and she was allowed to die a natural death.Intrauterine HSV infection is a rare but devastating illness. Approximately 1,500 cases of neonatal herpes infection occur annually in the United States, affecting approximately 1 in 3,200 neonates. (2,3) Although most neonatal herpes infections are acquired at the time of delivery (85%) and postnatally (10%), a small percentage (<5%) are acquired in utero. (2,4) Intrauterine HSV is acquired from ascending infection or hematogenous transplacental spread and is more likely to occur with primary rather than recurrent infections due to higher viral loads and longer periods of viral excretion. (5,6) A recent study of 69 consecutive cases of intrauterine fetal death found that 5% resulted from intrauterine HSV infection. (7)Symptoms of intrauterine infection are present at delivery or within the first 48 hours of birth(8) and usually involve the skin (94%), brain (79%), and eyes (59%). (9) Cutaneous manifestations include vesicles, ulcerations, pustules, bullae, hypopigmented scarring, zosteriform skin denudation in dermatomal distributions, (10) lesions resembling epidermolysis bullosa, (11,12) or aplasia cutis congenita. (12,13) Although rare, infants without mucocutaneous lesions have been reported. (14) Among the central nervous system (CNS) manifestations are hydrocephalus, cystic lesions, (1) hydraencephaly, brain atrophy, (15) hemorrhage, and encephalomalacia; (16) unlike the predilection for the temporal lobe seen in older infants who have HSV encephalitis, neonatal HSV can involve any and multiple regions of the brain. (2) Eye findings include chorioretinitis, microphthalmia, and persistent fetal ocular vasculature. (15,17) Systemic involvement is common, with many infants exhibiting disseminated disease on autopsy, especially of the liver, lungs, and adrenal glands. (15) Radiographs of the long bones may reveal metaphyseal lucencies as well as soft-tissue densities consistent with cutaneous calcification (dystrophic calcinosis cutis). (18) Additional rare reported findings include hydrops fetalis (19) and limb hypoplasia. (20)HSV is diagnosed via PCR testing of blood, cerebrospinal fluid (CSF), or scrapings of lesions. Acyclovir remains the current treatment (60 mg/kg per day divided every 8 hours), and the length of therapy is 21 days for disseminated or CNS disease. (21) The dosing interval may need to be increased in preterm infants based on creatinine clearance. (22) All infants who have CNS involvement should undergo a repeat lumbar puncture at the end of therapy to determine that CSF is PCR-negative. (23)Most cases of intrauterine HSV infection are identified as HSV-2 (90%), (24) but several cases of intrauterine infection with HSV-1 have been reported. (8,14,25) Infants infected with HSV in utero are more likely to be born preterm (59%) and have lower birthweights and shorter lengths. (24) The prognosis for intrauterine HSV infection is dismal. In the largest series of intrauterine HSV infection, 4 of 13 infants died; 7 of 13 had severe impairment, including intellectual disability, severe developmental delay, seizures, blindness, and hearing deficits; and 2 of 13 were expected to have severe impairment. (15) A single report describes a term infant who had large areas of atrophic skin scarring, erythema, and dystrophic nails present at birth and developed vesicles 4 days later that were positive for HSV-1. (8) He was presumed to have intrauterine infection due to skin scarring present at birth, was treated with acyclovir, and remained healthy at 18 months of age.In summary, intrauterine HSV is a rare but devastating illness that most often manifests with skin findings present at birth. A high index of suspicion is necessary to attempt to ameliorate the disease process, but outcomes are nearly uniformly poor.JoDee M. Anderson, MD, Division of Neonatal Medicine, Oregon Health & Science University, Portland, OR