Stevens‐Johnson syndrome and toxic epidermal necrolysis‐like cutaneous presentation of chikungunya fever: A case series

Chikungunya fever is a benign, self‐limiting, acute viral illness. An epidemic occurred in New Delhi, India, in August and September 2016. We observed many cases with atypical cutaneous features mimicking Stevens‐Johnson syndrome and toxic epidermal necrolysis during this epidemic, especially in infants and children. Twenty‐one children (13 [61.9%] boys, 8 [38%] girls) presenting with vesico‐bullous and necrotic lesions were reviewed. Cutaneous presentation included vesicles and bullae with purpuric macules and necrosis, seen in 16 (76%) patients. Skin lesions resolved in 5‐7 days, leaving behind hyperpigmentation in seven (33.3%) patients and hypopigmentation in three (14.2%). Minor oral erosions were observed in three (14.2%) patients, and palmoplantar erythema was seen in four (19.04%). It is essential for dermatologists to understand the Stevens‐Johnson syndrome and toxic epidermal necrolysis‐like presentation of chikungunya and not to misinterpret it as true Stevens‐Johnson syndrome and toxic epidermal necrolysis, which will lead to unnecessary intervention and management.     

Photo‐induced graft‐versus‐host disease

Overlap chronic graft‐versus‐host disease (GVHD) associates both features of acute and chronic GVHD. Trigger factors for chronic GVHD are unclear. We describe two patients who received allogenic haematopoietic stem‐cell transplantation, and who later developed overlap chronic GVHD after sun exposure. Available data from in vivo investigations suggest ultraviolet B radiation (UVB) has a beneficial effect on acute and chronic GVHD. The role of sun irradiation as a trigger for isomorphic cutaneous GVHD has been rarely reported in the literature. Herein, we demonstrate for the first time, using repetitive broadband phototesting, that UVB triggers chronic GVHD.     

Early‐onset lichenoid graft‐vs.‐host disease: a unique variant of acute graft‐vs.‐host disease occurring in peripheral blood stem cell transplant recipients

Background: A complication of stem cell transplantation is chronic graft‐vs.‐host disease (GvHD), developing months to years after transplant; the two commonest manifestations are lichenoid GvHD and scleroderma. The purpose of this study was to characterize early‐onset lichenoid GvHD. Methods: A retrospective study identified patients diagnosed with early‐onset lichenoid GvHD. This diagnosis was correlated with type of transplant and concurrent or prior episodes of acute GvHD. Results: Patients in whom a sex mismatch was present between donor and recipient were included, representing a study population of 17. All received an allogeneic peripheral blood stem cell transplant (PBSCT). All patients had biopsy proven lichenoid GvHD within 60 days or less following transplantation. All had concurrent gastrointestinal symptoms which was biopsy proven GvHD in thirteen of the cases. FISH XY studies revealed that the infiltrating lymphocytes were of donor origin in 12 of the cases, mixed in three and of host origin in two cases. Conclusions: Early‐onset lichenoid GvHD is exclusive to the PBSCT setting and appears to be mediated by donor lymphocytes, reflecting the higher numbers of donor T cells encountered in PBSCT. We consider this reaction pattern a distinctive subtype of acute GvHD. Magro CM, Kerns MJ, Votava H, Vasil KE, Dyrsen ME, Morrison CD. Early‐onset lichenoid graft‐vs.‐host disease: a unique variant of acute graft‐vs.‐host disease occurring in peripheral blood stem cell transplant recipients.     

Extracorporeal photopheresis in graft‐versus‐host disease

Graft‐versus‐host disease (GvHD) is a common and often serious complication of hematopoietic stem cell transplantation. There are two major forms of GvHD: an acute form which develops in the first 100days after HSCT, and a chronic form which develops later. Chronic GvHD is a multiorgan syndrome with many features of autoimmune diseases, such as sclerodermatous skin changes, cholestasis, pulmonary fibrosis, xerostomia, oral ulcerations, myositis and fasci‐itis. Unlike acute GvHD which is characterized by acute alloreactivity,the etiology of chronic GvHD is controversial and is believed to be either an extension of acute GvHD and/or a result of dysfunctional immune reconstitution with generation of autoantibodies and autoreactive T‐cell clones. GvHD is usually treated with corticosteroids and other immunosuppressants which do not always succeed in arresting its evolution. Extracorporeal photochemotherapy has been used in patients with both acute and chronic GvHD.     

Coronavirus‐days ın dermatology

Severe acute respiratory syndrome‐corona virus‐2, which causes coronavirus disease 2019 (COVID‐19), is highly contagious and a particularly popular problem in all around the World and also in all departments of every hospital. In order to protect the well‐being of health care providers while providing a sufficient workforce to respond to the COVID‐19 are vital for pandemic planning. In this article, we will discuss this problem from a dermatological aspect.     

Peas,beans, and the Pythagorean theorem – the relevance of glucose‐6‐phosphate dehydrogenase deficiency in dermatology

Glucose‐6‐phosphate (G6PD) deficiency is a common disease characterized by acute hemolysis induced by oxidative stress. More than 400 million subjects throughout the world carry the hereditary enzyme defect with the highest prevalences in Africa, Asia, and the Mediterranean region. In individuals affected by the erythrocytic enzymatic disorder, besides infectious diseases and diet, acute hemolytic crisis can be triggered by numerous drugs frequently used for the treatment of dermatoses.Taking into account the increasing number of immigrants from geographic regions with high preva‐lences of G6PD deficiency, dermatologists should be alert to the presence of disease.     

Entero‐Behçet's disease coexisting with long‐term epilepsy and schizophrenia‐like symptoms

Entero‐Behçet's disease coexisting with long‐term epilepsy and schizophrenia‐like symptoms is presented. A 43‐year‐old woman presented with repeatedly occurring aphthous stomatitis for several years. She had been treated for absence seizures, epilepsy and schizophrenia since she was 9 years old. She presented with multiple aphthous stomatitis on her gingiva, erythema nodosum‐like symptoms on the right lateral aspect of her leg and genital ulcers on her perianal area. She also showed polyarthritis. Laboratory examinations revealed elevated C‐reactive protein, elevated neutrophil counts, decreased serum Fe and elevated serum Cu. Histological examination showed perivascular neutrophil and mononuclear cell infiltrates and eosinophilic change of the vessel wall in the lobules of subcutaneous fat tissue. Six weeks after the oral prednisolone therapy, she showed resolution of aphthous stomatitis, folliculitis‐like eruption and genital ulcer. She experienced severe abdominal pain after the start of treatment of Behçet's disease. Plain computed tomography revealed edematous change in the appendix, and ascending and transverse colon. These results led to the diagnosis of entero‐Behçet's disease acute exaggeration. Treatment with infliximab (300 mg/once) was started. Eight weeks after the start of infliximab, her abdominal pain disappeared and C‐reactive protein decreased, followed by the successful change to adalimumab infusion therapy.     

Graft‐versus‐Host‐Disease: Ein interdisziplinäres Problem aus der Sicht des Dermatologen

Graft‐versus‐host disease (GVHD) is a frequent complication following hematopoietic stem cell transplantation. Acute and chronic GVHD are identified based on the onset of clinical symptoms and signs. Whereas acute GVHD is relatively uniform in its appearance, chronic GVHD is characterized by a broad spectrum of clinical manifestations. The aim of this review is to introduce the reader to the pathophysiologic processes underlying GVHD, to demonstrate its manifestations in the skin and to review current therapeutic options.     

Congenital Chikungunya—A Cause of Neonatal Hyperpigmentation

A 12‐day‐old neonate presented with ill‐defined dark pigmentation over the centrofacial area with flagellate pigmentation on the trunk and patchy pigmentation on the extremities. The mother had a history of fever starting a week before delivery and continuing for 3 days in the postpartum period. Together these led to consideration of a possible diagnosis of congenital chikungunya, which was confirmed according to the immunoglobulin M antibodies to chikungunya in the mother and child. The rare occurrence of cutaneous pigmentation was the only clue to the retrospective diagnosis of neonatal chikungunya. Chikungunya is an emerging viral disease that can be transmitted maternally during pregnancy and in the peripartum period. It can be added to the list of viral infections that can lead to fetal demise or, when present during labor and delivery, can cause neonatal disease with cutaneous signs.     

Cutaneous manifestations of graft‐versus‐host disease: role of the dermatologist

Graft‐versus‐host disease (GVHD) is the major complication of hematopoietic stem cell transplantation and is associated with high mortality in severe cases. The skin is one of the major organs affected in both acute and chronic GVHD. This review aims to elucidate the basic characteristics of GVHD, and the role and contribution of dermatologists in the care of patients with this condition.     

Severe Mucha–Habermann‐Like Ulceronecrotic Skin Disease in T‐Cell Acute Lymphoblastic Leukemia Responsive to Basiliximab and Stem Cell Transplant

A 5‐year‐old girl with T‐cell acute lymphoblastic leukemia (T‐ALL) developed a progressive eruption of crusted papules and ulcerative plaques involving 80% of her body surface area with histopathology consistent with febrile ulceronecrotic Mucha–Habermann disease (FUMHD), although multiple specimens also contained clonal leukemic cells. Her skin disease was refractory to many classic treatments for FUMHD, including methotrexate, and became so severe that concern about superinfection prevented intensification of chemotherapy for her malignancy. The addition of basiliximab promoted gradual improvement of the skin, allowing for chemotherapy intensification and subsequent bone marrow transplantation, after which the eruption resolved completely. This report describes a severe case of FUMHD‐like eruption associated with clonal leukemic cells that improved with basiliximab, suggesting anti‐CD25 therapy as a novel treatment for ulceronecrotic skin disease in the setting of high interleukin‐2 levels.     

Acute phase inflammatory markers in patients with non‐steroidal anti‐inflammatory drugs (NSAIDs)‐induced acute urticaria/angioedema and after aspirin challenge

Background Active chronic urticaria, identified as a mast cell‐ and basophil‐dependent inflammatory disorder of the skin is able to elicit acute phase response (APR). However, systemic inflammatory response in different types of urticaria is poorly characterized. Aim To determine APR pattern in a clearly defined group of patients with acute urticaria and/or angioedema – induced by NSAIDs. Methods Plasma IL‐6 and serum C‐reactive protein (CRP) concentrations were studied in 17 patients with NSAIDs‐induced acute urticaria/angioedema (NSAIDsAU) and in 20 healthy controls. Eleven patients who used NSAIDs were presented at the emergency room with acute urticaria/angioedema while the remaining six manifested the symptoms during the aspirin challenge test. Patients were examined in a dynamic manner: during the acute phase, and next, after subsidence of the symptoms. Results CRP and IL‐6 concentrations increased significantly in patients with NSAIDsAU as compared with their asymptomatic period and the healthy subjects. In addition, NSAIDsAU patients showed elevated concentration of the biomarkers following aspirin provocation with the baseline values recovered in the asymptomatic period. Conclusion These results indicate that an acute systemic inflammatory response is activated in patients with NSAIDs‐induced urticaria and/or angioedema. The study supports the evidence proving that up‐regulation of CRP and IL‐6 in urticaria/angioedema does not necessarily reflect any concomitant infection or other inflammatory processes, but may be due to the disease itself.     

Cryopyrin‐Associated Periodic Syndrome

Abstract: Cryopyrin‐associated periodic syndromes (CAPS) are characterized by apparently unprovoked attacks of fever, rashes, and musculoskeletal and sensorineural inflammation accompanied by high acute‐phase reactants. Excessive interleukin‐1 (IL‐1) signaling appears to be a constant feature in the pathomechanism of the disease, driven by a gain‐of‐function mutation in the NLRP3 gene. Herein, we present the case of a 9‐month‐old boy with recurrent nonpruritic rashes and episodes of fever. The difficulties of early diagnosis due to initially mild clinical symptoms and the dramatic response to anti‐IL‐1 therapy after diagnosis emphasize the practical relevance of considering CAPS as a differential diagnosis in these patients.     

Extramedullary acute leukemia developing in a pre‐existing osteoma cutis

Primary osteoma cutis (cutaneous ossification) is an uncommon disease in which there is bone formation within the skin in the absence of a demonstrable pre‐existing condition. Osteoma cutis is a chronic and benign condition. We report a case of a 45‐year‐old man who developed extramedullary acute leukemia with a myeloid immunophenotype (myeloid sarcoma) with its initial presentation within an isolated pre‐existing osteoma cutis in the post‐auricular scalp without evidence of systemic acute leukemia or chronic myeloid stem cell disorders. The tumor was surgically excised without complications. Four months later, acute leukemia recurred in the contralateral posterior mandible and showed an immunophenotype consistent with acute lymphoblastic leukemia/lymphoma. The patient now has been treated by standard protocols for acute leukemia. The diagnosis of an extramedullary acute leukemia is challenging because of its inconsistent clinical and histopathologic presentations. Extramedullary acute leukemia developing in a pre‐existing osteoma cutis is very unusual and has not been previously reported in the literature.     

Follicular Mucinosis in a Male Adolescent with a History of Acute Myelogenous Leukemia and Graft‐versus‐Host Disease

Although many cases of follicular mucinosis are idiopathic, numerous others are associated with mycosis fungoides or, rarely, other neoplastic or inflammatory disorders. There are only three reported cases, all in adults, of follicular mucinosis arising in association with acute myelogenous leukemia, two of which involved mycosis fungoides–associated follicular mucinosis, including one case in which the patient had a preceding bone marrow transplant. We present the first reported case of follicular mucinosis arising in an adolescent with acute myelogenous leukemia and acute graft‐versus‐host disease after an allogeneic bone marrow transplantation.     

A Case of Eczematoid Graft‐Versus‐Host Disease

A 13‐year‐old boy underwent allogeneic hematopoietic stem cell transplantation (HSCT) for underlying acute lymphoblastic leukemia and achieved neutrophil engraftment 28 days after HSCT. He developed ichthyosis 6 weeks after HSCT and then keratotic follicular papules, palmoplantar keratoderma, and a seborrheic dermatitis–like eruption 18 weeks after HSCT. From skin biopsies he was diagnosed with eczematoid graft‐versus host disease (GVHD), which showed spongiosis with scattered necrotic keratinocytes. He responded to oral and topical steroids and an increase in cyclosporine dose. Although uncommon, eczematoid GVHD must be considered in children who have undergone HSCT and then develop an atypical eczematous eruption, especially in the absence of a history of atopy.     

An Atypical Case of Bullous Systemic Lupus Erythematosus in a 16‐Year‐Old Boy

Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune subepidermal blistering disease with a female predominance characterized as an acute vesicobullous eruption in patients with systemic lupus erythematosus (SLE). Here we report a case of BSLE in a 16‐year‐old boy that does not adhere to the criteria originally established and suggest a new outlook on this condition.     

Terminal osseous dysplasia with pigmentary defects; Case and brief review of filamin A‐related disorders

Terminal osseous dysplasia with pigmentary defects (TOD) is an extremely rare X‐linked dominant disorder, which is characterised by cutaneous digital fibromas, pigmentary skin defects and skeletal abnormalities. A single mutation in the last nucleotide of exon 31 of the filamin A gene (FLNA) has recently been identified as a cause of the disease. We describe a case of an 18‐month‐old girl with the clinical phenotype of TOD and the disease‐specific FLNA mutation confirmed by genetic testing. This report highlights the importance of recognising this distinct phenotype that can present to a wide variety of health‐care professionals, and reviews the spectrum of filamin A disorders.     

Post‐steroid panniculitis in an adult

Post‐steroid panniculitis is known to be very rare and most of the reported cases have been in children after corticosteroid therapy. We present a case of post‐steroid panniculitis occurring in a 60‐year‐old man after massive, long‐term administration of corticosteroids for acute exacerbation of chronic obstructive pulmonary disease (COPD). Histopathological examination of a nodule revealed a patchy area of fat necrosis, several multinucleated giant cells containing needle‐shaped clefts. The lesions subsided completely in approximately 12 weeks without any treatment. We suggest that post‐steroid panniculitis is not confined to childhood but also occurs in adulthood.