Transverse spinal cord infarction following immunoglobulin treatment in a patient with exfoliative dermatitis: A case report.

RATIONALE: Transverse spinal cord infarction (SCI) is rare but highly disabling. Aortic thrombosis was described as one of the most common etiologies. Thromboembolic complications associated with intravenous immunoglobulin (IVIG) have been reported. PATIENT CONCERNS: A previously well, 64-year-old man who was given the treatment of IVIG (0.4 g/kg/d for 5 days) for exfoliative dermatitis 2 weeks before, progressively developed flaccid paraplegia of lower extremities, loss of all sensations below T3 level and urinary incontinence within 50 minutes. DIAGNOSES: A diagnosis of SCI and pulmonary embolism was made. IVIG was considered the possible cause. INTERVENTIONS: Anticoagulation treatment and continuous rehabilitation were administered. OUTCOMES: The neurologic deficiency of the patient was partially improved at the 3-year follow-up. LESSONS: The rapid development of severe deficits within 4 hours mostly contributes to the diagnosis of SCI. Heightened awareness of possible thrombotic events is encouraged for a month-long period following IVIG therapy.

Newborn Skin: Part I. Common Rashes and Skin Changes.

Rashes in the newborn period are common and most are benign. Infections should be suspected in newborns with pustules or vesicles, especially in those who are not well-appearing or have risk factors for congenital infection. Congenital cytomegalovirus infection can cause sensorineural hearing loss and neurodevelopmental delay. Skin manifestations of cytomegalovirus may include petechiae due to thrombocytopenia. The most common skin manifestations of early congenital syphilis are small, copper-red, maculopapular lesions located primarily on the hands and feet that peel and crust over three weeks. Erythema toxicum neonatorum and neonatal pustular melanosis are transient pustular rashes with characteristic appearance and distribution. Neonatal acne is self-limited, whereas infantile acne may benefit from treatment. Milia can be differentiated from neonatal acne by their presence at birth. Cutis marmorata and harlequin color change are transient vascular phenomena resulting from inappropriate or exaggerated dilation of capillaries and venules in response to stimuli.

Homozygous CARD14 variant presenting as infantile erythroderma.

A wide range of inherited and acquired conditions can manifest as infantile erythroderma, among which CARD14-associated papulosquamous eruption (CAPE) is a rare cause. An infant boy presented with a psoriasiform rash that progressed to erythroderma and was unresponsive to topical steroids and cyclosporine. The early onset of the disease, its severity and resistance to conventional treatment were suggestive of a genetic cause. Genetic evaluation revealed a homozygous CARD14 variant of uncertain significance establishing the diagnosis of CAPE, and his parents were heterozygous carriers. There was only minimal improvement in the condition with supportive management and treatment with acitretin. Unfortunately, the child succumbed to sepsis and metabolic complications following a sudden worsening of skin disease. This case highlights the significance of genetic studies in diagnosing treatment-refractory cases of infantile erythroderma and emphasises the importance of early recognition of this rare condition.

Erythroderma in a Patient With Thymoma-Associated Myasthenia Gravis.

A woman in her 30s with myasthenia gravis diagnosed at age 27 years presented to the emergency department with severe erythroderma over the past 2 months. What is your diagnosis?

Erythroderma combined with deeper dermal dermatophytosis due to Trichophyton rubrum in a patient with myasthenia gravis: first case report and literature review.

BACKGROUND: Dermatophytes are the most common causative pathogens of mycoses worldwide and usually cause superficial infections. However, they can enter deep into the dermis lead to invasive dermatophytosis such as deeper dermal dermatophytosis on rare occasions. Erythroderma is a severe dermatological manifestation of various diseases resulting in generalized skin redness, but erythroderma due to fungi infections is barely reported. In this article, we reported the first case of erythroderma combined with deeper dermal dermatophytosis due to Trichophyton rubrum (T. rubrum) in a patient with myasthenia gravis. CASE PRESENTATION: A 48-year-old man was hospitalized because of erythema with scaling and nodules covering his body for a month. The patient had a history of myasthenia gravis controlled by regularly taking prednisolone for > 10 years and accompanied by onychomycosis and tinea pedis lasting > 8 years. Based on histopathological examinations, fungal cultures, and DNA sequencing results, the patient was finally diagnosed with dermatophyte-induced erythroderma combined with deeper dermal dermatophytosis caused by T. rubrum. After 2 weeks of antifungal treatment, the patient had recovered well. CONCLUSIONS: This case report shows that immunosuppressed patients with long histories of superficial mycoses tend to have a higher risk of developing invasive dermatophytic infections or disseminated fungal infections. Dermatologists should be alert to this condition and promptly treat the superficial dermatophytosis.

Erythrodermic Presentation of Atopic Dermatitis in a Patient with Secondary Adrenal Insufficiency Caused by Oral Glucocorticosteroid Abuse.

Dear Editor, A 41-year-old man presented to the Department of Dermatology for the first time due to an exacerbation of atopic dermatitis (AD) in the form of erythroderma. The patient had a history of atopic diseases, with being AD active from infancy. On clinical examination, generalized erythematous skin lesions causing acute pruritus and accompanied by severe skin exfoliation and dryness were present. On closer examination, the patient had a collection of signs and symptoms characterizing Cushing syndrome that included a round and full face (''moon face''), supraclavicular fat pads, and proximal muscle atrophy. The patient stated that AD had exacerbated six years earlier. He had received systemic treatment consisting of methotrexate followed by cyclosporine in another medical facility. However, both medications had proven ineffective and caused malaise. Only oral glucocorticosteroids had proven successful. The patient had been satisfied with the quick and observable effects, and, as he stated, he refrained from regular dermatological visits for six years. During that time, he consistently took 4 mg of methylprednisolone twice daily. Laboratory tests showed undetectably low levels of cortisol, triacylglycerols (TAG) at 288 mg/dL, and total cholesterol levels (CHC) of 81 mg/dL. Based on laboratory findings, clinical presentation, and histopathological evaluation of the skin biopsy, the diagnoses were secondary adrenal insufficiency caused by oral glucocorticosteroid abuse and AD in the form of erythroderma. The endocrinologist suggested a progressive reduction of the dose of methylprednisolone, starting at 2 mg twice daily. Total and sudden drug withdrawal was unacceptable, as it could cause an adrenal crisis. Methylprednisolone was eventually discontinued after being administered for 5 months while the blood levels of ACTH, cortisol, ionized sodium, and ionized potassium were monitored every 4 weeks. 25 mg of hydroxycortisol in divided doses was the actual treatment for adrenal insufficiency, with plans to also gradually reduce the dose. Since the commencement of endocrinological treatment, the dose was reduced to 15 mg after 5 months and to 10 mg after 7 months. Following an 8-month period, the patient began taking 10 mg as needed, usually a few times each month. Calcium carbonate in a dose of 1000 mg taken once daily before a meal for 5 months and vitamin D3 protected the patient from osteoporosis, another manifestation of Cushing syndrome. An initial dose of 4000 IU was prescribed. It is vital to emphasize that all dose adjustments in the endocrinological treatment of Cushing syndrome were a direct consequence of laboratory testing that was performed. In terms of erythrodermic AD management, the patient was treated with cyclosporin, which was once again ineffective. The patient was then prepared for the introduction of dupilumab. A 300 mg dose of the medication was subcutaneously administered every 2 weeks for over a year with positive outcomes, with an initial dose of 600 mg. The patient developed gynecomastia at the beginning of the treatment, initially categorized as another manifestation of Cushing syndrome. However, due to its unilateral nature, it was later identified a benign adverse event of dupilumab, as described in the literature (1). Due to a decline in effectiveness, the treatment was recently switched from dupilumab to baricitinib, with positive outcomes. Erythroderma, which the patient presented in our case, is an acute condition characterized by erythema and scaling that involves more than 90% of the skin's surface area (2,3). It can be potentially fatal due to electrolyte imbalance, fluid loss from capillary dilation, and significant heat dissipation (3). According to estimates, erythroderma is relatively rare, affecting approximately 1-2 patients for every 100,000 people per year, with AD comprising 8.7% of all cases of erythroderma (2,4). Despite growing therapeutic possibilities for AD, corticosteroids remain the drug of choice in severe exacerbations, including erythroderma, when we cannot afford to wait for the effects of therapy. Oral glucocorticosteroids can be an effective treatment for acute flares of AD (5). However, there is a lack of evidence for the long-term efficacy and safety of oral glucocorticosteroids in the treatment of AD (5). Reported side-effects include endocrine disturbances, gastric ulcers, cardiovascular disorders (arterial hypertension, atherosclerotic disease), osteoporosis, glaucoma, cataracts, and an increased risk of infections. Corticosteroids also have an undesired action on the skin that can result in steroid acne, skin atrophy, striae, telangiectasias, hypertrichosis, and impaired wound healing. The psychological adverse effects of steroid treatment can be quite severe and include depression and psychosis (6), The therapy should only be applied in the short-term, not exceeding one week, due to the occurrence of the abovementioned side-effects, which presented in as Cushing syndrome our patient (5). However, glucocorticoids are one of the most commonly used drugs in clinical dermatology practice, raising concerns about the risk of their misuse, which can lead to secondary adrenal insufficiency, among other complications (7). When no other treatment options are available, it should be noted that many of the side-effects of oral glucocorticosteroids can be mitigated through close monitoring and the implementation of appropriate preventive measures (7).

Staphylococcus epidermidis induced toxic shock syndrome (TSS) secondary to influenza infection.

BACKGROUND: To date, few cases of TSS caused by coagulase negative (CoN) staphylococci have been reported in the literature. Recent data show that CoN staphylococci are capable of secreting a number of enterotoxins and cytotoxins, normally produced by S. aureus. Herewith, we describe a case of TSS caused by Staphylococcus epidermidis with a favorable outcome. CASE PRESENTATION: We report a case of a 46-year-old man who developed TSS from S. epidermidis. The patient was admitted for a 7-day history of general malaise and headache following a recent influenza infection and a 3-day history of vomiting, diarrhea, diffuse erythroderma, and fever. The main laboratory findings on admission were leukopenia (WBC 800/mm3), thrombocytopenia (Plt count 78.000/mm3), elevated urea, creatine levels and increased inflammatory markers (CRP 368 mg/ml). The patient had clinical and radiological evidence of pneumonia with chest computed tomography (CT) showing diffuse bilateral airspace opacifications with air bronchogram. On the second day, a methicillin resistant S. epidermidis (MRSE) strain was detected in both sets of blood cultures, but the organism was unavailable for toxin testing. All other cultures and diagnostic PCR tests were negative. His clinical signs and symptoms fulfilled at that stage four out of five clinical criteria of TSS with a fever of 39 °C, diffuse erythroderma, multisystem involvement and hypotension. On the same day the patient was admitted to the ICU due to acute respiratory failure. The initial treatment was meropenem, vancomycin, levofloxacin, clindamycin, IVIG and steroids. Finger desquamation appeared on the 9th day of hospitalization, fulfilling all five clinical criteria for TSS. CONCLUSIONS: To our knowledge, this is the first adult case with TSS induced by CoNS (MRSE) secondary to an influenza type B infection, who had favorable progression and outcome. Further research is warranted to determine how TSS is induced by the CoNS infections.

HLA-DR Helps to Differentiate Erythrodermic Cutaneous T-cell Lymphoma from Erythrodermic Inflammatory Dermatoses in Flow Cytometry.

Differential diagnosis of erythroderma is challenging in dermatology, especially in differentiating erythrodermic cutaneous T-cell lymphoma from erythrodermic inflammatory dermatoses. This study retrospectively reviewed the peripheral blood flow cytometric results of 73 patients diagnosed with erythroderma at Peking University First Hospital from 2014 to 2019. The flow cytometry antibody panel included white blood cell markers, T-cell markers, B-cell markers, T-cell activation markers, and T helper cell differentiation markers. Features of the cell surface antigens were compared between 34 patients with erythrodermic cutaneous T-cell lymphoma and 39 patients with erythrodermic inflammatory dermatoses. The percentage of HLA-DR+/CD4+T cells was the most pronounced marker to distinguish erythrodermic cutaneous T-cell lymphoma from erythrodermic inflammatory dermatoses, with a threshold of 20.85% (sensitivity 96.77%, specificity 70.37%, p = 0.000, area under the curve (AUC) 0.882), suggesting its potential capability in the differential diagnosis of erythrodermic cutaneous T-cell lymphoma from erythrodermic inflammatory dermatoses. Moreover, in contrast to erythrodermic inflammatory dermatoses, the percentage of Th17 cells was significantly downregulated in erythrodermic cutaneous T-cell lymphoma (p = 0.001), demonstrating a dysregulated immune environment in erythrodermic cutaneous T-cell lymphoma.

Crusted Scabies Presenting as Erythroderma in a Patient With Iatrogenic Immunosuppression for Treatment of Granulomatosis With Polyangiitis.

The diagnosis of scabies can be difficult when the infection presents as erythroderma. Crusted scabies is a severe form of scabies caused by cutaneous ectoparasitic infection by the mite Sarcoptes scabiei var hominis. Crusted scabies most commonly occurs in patients with underlying immunosuppression from acquired infection or subsequent to solid organ or bone marrow transplantation. We present a rare case of a patient with granulomatosis with polyangiitis (GPA) who developed azathioprine-induced myelosuppression and subsequent erythrodermic crusted scabies. It is critical to maintain a broad differential when patients present with erythroderma, especially in the setting of medication-induced immunosuppression for the treatment of autoimmune disease.