Erythrodermic psoriasis is the least common and most severe variant of psoriasis manifesting as erythema and scaling affecting more than 75% body surface area. Infections, such as COVID-19, are proposed triggers due to provoking immune responses that can progress into a hyperinflammatory state. We present a case of a patient with a history of psoriasis evolving into an erythrodermic form after infection with COVID-19 virus.

A 50-year-old female, clinically diagnosed with plaque type psoriasis for 12 years, sought emergency consult then was admitted due to persistence of generalized erythematous scaly plaques, with accompanying skin and joint pains as well as high grade fever. Definitive diagnosis was done with clinicopathologic correlation including dermoscopy and skin punch biopsy. The patient’s Psoriasis Area and Severity Index (PASI) was 70.2, indicating severe score. SARS-COV-2 RT-PCR was done, revealing a positive result for the causative agent of COVID-19. A multidisciplinary approach to treatment was done with dermatology, rheumatology, and infectious disease services. Medications include antibiotics, antimetabolites, pain medications, and topical steroids. The patient was discharged then would follow-up with the dermatologist with phototherapy and with the rheumatologist. After completing treatment, most lesions have recovered.

Erythrodermic psoriasis is a severe and uncommon form of psoriasis that may be exacerbated by various infections such as COVID-19. Proper history, physical examination, and use of diagnostic procedures can aid in pinpointing the cause of the disease which will be indispensable in managing such patients.

Congenital ichthyosiform erythroderma is most severe type of ichthyosis. This disease is characterized clinically by generallized erythroderma covered with flaccid bullae and verruciform scales which are especially prominent on the flexural areas. This disease demonstrates an autosomal dominant mode of inheritance. The distinctive histologic change is the extreme hyperkeratosis associated with granular and vacuolar degeneration of the mid and upper malphigian layers. Cell kinetic studies indicate an increase in the epidermal mitotic turnover tirne. This 8 months old male patient visited to Dermatologic Department of Severance Hospital on April-28, 1973. Seven days after birth the scales were appeared on his flexural region of limbs and shed to leave a raw surface which forms scales anew. And there were the intermittent appearance of flaccid bullae prominantly involve the flexural area of limb but thereafter appearing the other region of body. Skin biopsy shows that of bullous type of congenital ichthyosiform erythroderma. This baby was expired 4 days after the admission day in spite of vigorous systemic and local trea- tments. The autopsy findings had exhibited fatty necrotic degenerative changes of hepatic cells and septic inflammatory changes of meningeal vessels and meninges itself as the causes of death of this pitiful victim.
Autopsy ; Biopsy ; Cause of Death ; Dermatitis, Exfoliative ; Extremities ; Hepatocytes ; Humans ; Ichthyosiform Erythroderma, Congenital ; Ichthyosis ; Infant ; Male ; Meninges ; Parturition ; Skin ; Weights and Measures ; Wills

No abstract available.
Ichthyosiform Erythroderma, Congenital*

Erythroderma can be life-threatening, primarily because of its metabolic burden and complications. It is mandatory to establish its etiopathology in order to facilitate precise and definitive management. This disorder may be the morphologic presentation of a variety of cutaneous and systemic diseases. Detailed history and thorough work-up is therefore essential. Management of erythroderma involves multi-disciplines with progress monitoring especially on signs and symptoms suggestive of acute skin failure induced complications. Early diagnosis and referral of erythroderma to centres with dermatological services is crucial and will directly affect the outcome of the patients.
Dermatitis, Exfoliative

INTRODUCTION: Exfoliative dermatitis is a potentially life- threatening inflammatory reaction that poses a significant risk for morbidity and mortality. Several underlying etiologies of this dermatologic condition include pre-existing dermatoses, drugs and malignancy. Although it is a common disease entity, local studies on exfoliative dermatitis published in literature are very limited. OBJECTIVE: The primary objective of this study is to determine the epidemiological profile of patients with exfoliative dermatitis diagnosed at University of Santo Tomas Hospital Dermatology department from January 2008 to December 2012. METHODS: Inpatient and outpatient clinical records of patients diagnosed and treated as exfoliative dermatitis were retrieved. The prevalence, clinical presentation, history of previous dermatoses or use of any drugs/topical medications, family history and accompanying systemic symptoms were reviewed and analyzed. RESULTS: A total of 67 patients were included in this retrospective study. The prevalence among patients with exfoliative dermatitis in this study was computed at 1 per 1000 dermatologic patients. The highest number of cases belonged to the group aged seventy-one to seventy-nine (25.4%) with a mean age of 56.62 years. There was a male predilection (65.7%). Clinical presentation of patients included pruritus, generalized scaling and erythema, accompanied by bipedal edema (41.8%), chills (22.4%), fever (T ≥ 38 °C), lymphadenopathies (6%) and joint pains (4.5%). Several etiologic factors of exfoliative dermatitis recorded were: pre-existing dermatosis (67.2%), idiopathic or undetermined causes (19.4%), drug-induced (10.4%) and malignancy (3%). CONCLUSION: Exfoliative dermatitis is a condition more commonly found in the older age group. Pre-existing dermatoses, drugs and malignancy are etiologic factors. The most common pre-existing dermatosis causing exfoliative dermatitis in this study is psoriasis while the most implicated drug is allopurinol.
Dermatitis, Exfoliative

No abstract available.
Dermatitis, Exfoliative ; Phototherapy ; Psoriasis

Netherton's syndrome is an unusual disorder which consists of triad of ichtyosiform dermatosis, multiple defects of hair shaft and an atopic diathesis. The finding of bamboo hair is pathognomic in Netherton's syndrome and the ichthyosiform dermatosis may consist of either ichtyosis linearis circumflexa or congenital ichthyosiform erythroderma. Often, variability in the clinical features leads to a delay in diagnosis in many cases. We report a case of Netherton's syndrome diagnosed in the neonatal period. The patient presented with severe ichthyosis and confirmed microscopically distinctive bamboo hair.
Diagnosis ; Disease Susceptibility ; Hair ; Humans ; Ichthyosiform Erythroderma, Congenital ; Ichthyosis ; Infant, Newborn* ; Skin Diseases

OBJECTIVE: To explore the genetic cause for a child with congenital ichthyosis. METHODS: The child was subjected to next generation sequencing using a specific gene panel. Suspected mutation was validated by Sanger sequencing. RESULTS: The proband was found to harbor compound heterozygous mutations c.327delG (p.Met109Ilefs*2) and c.791G>A (p.Arg264Gln) of the TGM1 gene, which were respectively inherited from his mother and father. The same mutations were not found among 101 healthy controls. c.327delG was not reported previously. By bioinformatic analysis, both mutations are likely to impair the function of TGase-1 protein. CONCLUSION The compound heterozygous mutations of c.327delG and c.791G> A of the TGM1 gene probably underlie the ichthyosis in the proband. The result has facilitated prenatal diagnosis for this pedigree.
Female ; Humans ; Ichthyosiform Erythroderma, Congenital ; genetics ; Infant, Newborn ; Mutation ; Pedigree ; Phenotype ; Pregnancy ; Transglutaminases ; genetics

A 4-year-old female with Congenital Hemidysplasia with Ichthyosiform erythroderma and Limb Defects (CHILD) syndrome, with a pathogenic variant of the NSDHL gene, c.130G>A (p.Gly44Ser), and unilateral right-sided erythematous verrucous plaques with scaling and ipsilateral limb defects, was started on 5% simvastatin ointment. It was applied twice daily for four months, with improvement already seen starting week 2. Monotherapy with 5% simvastatin ointment was able to decrease the thickness of the verrucous plaques seen in our patient, highlighting that the accumulation of toxic metabolites may play a more crucial role in its disease pathogenesis.

Collodion baby is one of the rare congenital ichthyosiform dermatoses first described by Seeligmann in 1841. It is a phenotypic expression of several genotypes such as aex-linked ichthyosis and bullous and non-bullous congenital ichthyosiform erythroderma. The infant is enveloped in a smooth and shiny collodion-like membrane at birth, which temporarily may deform the facial feature and extremities. The skin clears almost completely in the first month of life and remains free of ichthyosis. Etiology of this condition is still controversy, but it has been known that abnormalities of other organs are not accompanied but prematurity frequently. We present two cases of collodion baby with electron microscopic findings. In the upper stratum spinosum, keratinosomes and desomoaomes were increased in the keratinocytes, and intercellular spaces widened, however, desmosomes and tonofilaments were intact. These findings were similar to those of nonbullous congenital ichthyosiform erythroderma. But in some widened intercellular spaces of the upper stratum spinosum, multiple finely granular electron dense particles were seen within large round membranous structures.
Collodion* ; Desmosomes ; Extracellular Space ; Extremities ; Genotype ; Humans ; Ichthyosiform Erythroderma, Congenital ; Ichthyosis ; Ichthyosis, Lamellar ; Infant ; Intermediate Filaments ; Keratinocytes ; Membranes ; Parturition ; Skin ; Skin Diseases