ABSTRACT
Graft-versus-host disease (GvHD) is common after haematopoietic cell transplantation (HCT). Mucocutaneous manifestations are variable and may simulate autoimmune bullous dermatoses. However, the association of GvHD with autoimmune disorders, including bullous dermatoses, is also well recognized. We describe a patient with GvHD in whom severe and relapsing epidermolysis bullosa acquisita (EBA) was diagnosed 3 years after transplant and propose a causal association with GvHD. A 66-year-old woman developed GvHD following allogeneic HCT for acute myeloid leukaemia in 2016. This affected her gastrointestinal tract and skin but improved with oral corticosteroids and ciclosporin. In 2019 she presented with a widespread rash consisting of large, tense, haemorrhagic blisters. Histological features were in keeping with EBA. Direct immunofluorescence was also consistent with EBA, demonstrating linear positivity for IgG and C3 confined to the blister base, as was detection of collagen VII antibodies on indirect immunofluorescence. She was admitted and treated with high-dose oral steroids, ciclosporin and intravenous immunoglobulin (IVIg) with eventual resolution of blistering. Although further IVIg administration was planned as an outpatient, this coincided with the start of the COVID-19 pandemic and she elected not to attend and also stopped all medication. Despite this, her EBA remained quiescent until September 2021 when she was readmitted with a severe deterioration in blistering and significant dysphagia due to an oesophageal stricture, with a weight of 31.7 kg. Once again, she responded rapidly to oral prednisolone and IVIg. Dapsone was considered but precluded by G6PD deficiency and there were clinical and adherence concerns about using mycophenolate mofetil. Upon discharge she was again nonadherent to medication and failed to attend for planned IVIg. She flared and was admitted for a third time in December 2021, requiring gastrostomy for nutritional support;her weight at this time was 26.4 kg. Her EBA is currently well controlled on prednisolone and IVIg. EBA is a rare, acquired blistering disorder secondary to autoantibodies targeting type VII collagen. Previous studies have found circulating basement membrane zone (BMZ) antibodies in 24% of chronic GvHD patients, possibly generated in response to chronic BMZ damage (Hofmann SC, Kopp G, Gall C et al. Basement membrane antibodies in sera of haematopoietic cell recipients are associated with graft-versushost disease. J Eur Acad Dermatol Venereol 2010;24: 587-94). Corresponding clinical manifestations are rare, with bullous pemphigoid the most frequently reported. EBA is much less common with four previously reported cases [Brassat S, Fleury J, Camus M, et al. (Epidermolysa bullosa acquisita and graftversus- host disease). Ann Dermatol Venereol 2014;141: 369-73 (in French)]. As a fifth case of EBA, our patient provides further evidence of a likely pathophysiological relationship between GvHD and autoimmune subepidermal bullous dermatoses, and highlights the significant challenges of managing these vulnerable patient groups during the COVID-19 pandemic.
ABSTRACT
With the rapid outbreak of the coronavirus disease 2019 (COVID-19) pandemic, considerable concerns about the safety of systemic treatments of immune-mediated dermatologic disorders has been raised by dermatologists and their patients. We aimed to perform a rapid review of latest American and European guidelines on the use of systemic treatments in patients with immune-mediated dermatologic disorders and confirmed COVID-19 infection and to provide recommendations to inform practice. Based on the current limited guidelines and evidence, systemic corticosteroids should not be abruptly discontinued and the lowest effective dose should be continued. Systemic immunosuppressants (including methotrexate, cyclosporine, azathioprine, cyclophosphamide, and leflunomide), biologics, and sulfasalazine should be withheld in patients with confirmed COVID-19 infection. Whether to continue Janus kinase inhibitors should be determined following a shared decision-making process between dermatologists and patients after considering patients' medical conditions and risk for severe COVID.
ABSTRACT
Objective: The number of older adults has increased throughout the world. Aging affects all the organs and creates psychological, physiologic and anatomic changes. One of the most important organs of the human body is the skin, which shows the effects of aging the most. This study aims to determine whether age, gender, and season of biopsy play a significant role in skin biopsy results. Additionally, the study investigates whether the frequency of skin diseases differs before and after the coronavirus disease-2019 (COVID-19) pandemic. Methods: We conducted a retrospective study on the histopathology results of patients over 65 years old between June 2016 and September 2021. The histopathology results were obtained from the Acibadem Pathology Department. Results: Of the 677 patients, 310 (45.8%) were male and 367 (54.2%) were female. The most common disease in all patients were benign cutaneous neoplasms (23%), followed by eczematous disease (18.5%) and epithelial cutaneous cancers (16.8%). We divided the results into 12 groups: group 1: Urticaria, erythema and purpuras, group 2: Papulosquamous and eczematous diseases, group 3: Infectious diseases, group 4: Rheumatologic diseases and alopecia, group 5: Benign cutaneous neoplasms, group 6: Precancerous lesions, group 7: Basal cell carcinoma, squamous cell carcinoma, group 8: Cutaneous metastasis and other skin cancers, group 9: Pigmentation disorders, group 10: Pschycology related dermatological disorders, group 11: Granulomatous dermatitis, group 12: Bullous dermatitis. Before the COVID-19 pandemic, the most prevalent results were group 2 (21.3%), followed by group 5 (20.4%) and group 7 (16.7%) whereas, after the COVID-19 pandemic, the most frequent results were group 5 (28.4%) followed by group 7 (17.1%), and group 6 (14.9%). In terms of seasons, the most common diseases were group 5 (24.1%) in winter, group 2 (21.6%) in spring, group 5 (30.0%) in summer, and group 2 (18.9%) in autumn. Before the COVID-19 pandemic, the most common result was group 2 (21.3%), followed by group 5 (20.4%) and group 7 (16.7%), and during the COVID-19 pandemic, the most common result was group 5 (28.4%), followed by group 7 (17.1%), group 6 (14.9%). Conclusion: Many skin diseases affect the geriatric population. Geriatric patients face challenges such as multiple drug use, comorbidities, mobility problems and cognitive disorders. In our study, the most common diseases in all patients were benign cutaneous neoplasms (23%), followed by eczematous diseases (18.5%), and epithelial cutaneous cancers (16.8%). Knowing about the frequency of skin diseases is critical for the early detection of precancerous and cancerous lesions.