Lupus erythematosus associated with erythema multiforme: report of two cases and review of the literature

Rowell's syndrome (RS) is a rare presentation of lupus erythematosus (LE) with erythema multiforme‐like lesions associated with antinuclear, anti‐La (SS‐B)/anti‐Ro (SS‐A) antibodies and rheumatoid factor (RF) positivity. This syndrome is suggested to be a different variant of cutaneous lupus erythematosus by some authors in literature. Here we present a 64‐year‐old woman with LE and a 51‐year‐old woman with LE and Sjögren syndrome (SS) who had erythema multiforme‐like eruptions and discuss the coexistence of lupus erythematosus and erythema multiforme.     

Anti‐signal recognition particle antibody‐positive polymyositis in a patient with Sjögren's syndrome showing various types of annular erythema: Positive correlation between the activities of annular erythema and myositis

Annular erythema with Sjögren's syndrome (AESS) is occasionally found, especially in Asian patients, which is classified into three types. We present a case of Sjögren's syndrome showing various types of AESS with anti‐signal recognition particle antibody‐positive polymyositis. We successfully treated the eruption and myositis with a low dose of prednisolone. Every onset of annular erythema coincided with elevation of serum creatine kinase levels, which suggests the correlation between the activities of annular erythema and polymyositis.     

Paediatric necrolytic migratory erythema as a presenting sign of glucagonoma syndrome

Glucagonoma syndrome is an extremely rare pancreatic neuroendocrine tumour often associated with necrolytic migratory erythema. While glucagonomas are neoplasms of adulthood, we report the first case in a paediatric patient. We present the case of a 15‐year‐old girl with a 4‐year history of a rash, consistent with necrolytic migratory erythema, found to have a localized glucagonoma. Immediately following resection of the tumour, there was complete resolution of her rash and systemic symptoms. Detection of the cutaneous rash of necrolytic migratory erythema can aid in the early diagnosis of a glucagonoma, as well as the prevention of metastatic disease. To our knowledge, this is the first reported paediatric patient with glucagonoma syndrome.     

Onset of cutaneous vasculitis and exacerbation of IgA nephropathy after Bartonella henselae infection

Bartonella henselae is the aetiological agent of cat‐scratch disease. Recently, there have been reports of other conditions associated with this bacterium, including leucocytoclastic vasculitis, thrombocytopenic purpura, maculopapular and urticarial eruptions, granuloma annulare, erythema nodosum, erythema marginatum and erythema annulare. We report the first case, to our knowledge, of the simultaneous occurrence of cutaneous vasculitis and nephrotic syndrome in a 65‐year‐old woman with IgA nephropathy after a B. henselae infection transmitted by a cat scratch. The aetiopathogenetic role of B. henselae was hypothesized on the basis of the serological demonstration of acute B. henselae infection, the immunofluorescence findings, and the prompt resolution after azithromycin treatment. Patients reporting cat scratches or bites should undergo accurate clinical examination, routine laboratory examinations, urinalysis and clinical surveillance.     

Autoimmune thyroid disease in anti-Ro/SS-A-positive children with annular erythema: report of two cases

Anti‐Ro/SS‐A‐associated recurrent annular erythema is a rare disorder, and represents a cutaneous manifestation of primary Sjögren's syndrome (SS). We report two childhood cases complicated with autoimmune thyroid disease, one with Graves' disease and the other with autoimmune thyroiditis. Both children were positive for anti‐Ro/SS‐A and anti‐La/SS‐B antibodies. One patient was lacking clinical SS with objective evidence of salivary gland involvement, while the other was diagnosed with primary SS. Our observation suggests that autoimmune thyroid disease in the subset of anti‐Ro/SS‐A‐positive children with annular erythema might occur with similar frequency to that in adult primary SS.     

Necrolytic migratory erythema: a cutaneous clue to glucagonoma syndrome

Necrolytic migratory erythema (NME) is a cutaneous manifestation of the glucagonoma syndrome. We present a case with a pancreatic glucagon‐secreting tumour, skin eruption and a good response to treatment.     

Glucagonoma syndrome: a review and update on treatment

Glucagonoma syndrome is defined by the presence of an alpha‐cell secreting tumour of the pancreas, elevated levels of glucagon, and a characteristic rash called necrolytic migratory erythema (NME). NME is usually a specific and often initial finding of glucagonoma syndrome, but it may occur in other settings unassociated with an alpha‐cell pancreatic tumour (pseudoglucagonoma syndrome). Glucagonoma syndrome must be distinguished from pseudoglucagonoma syndrome. Prompt recognition of NME and subsequent workup for a glucagonoma can allow for an earlier diagnosis and enhance the chances of a favourable outcome. In particular, metastases occur late, so early recognition of glucagonoma syndrome before liver metastases can be life‐saving. Surgical resection is the definitive treatment for glucagonoma syndrome, although chemotherapeutic agents, somatostatin analogues and radionuclide therapy are also employed. Herein, we offer an approach to workup after identifying NME and an update on its current treatment modalities.     

Case of late‐onset erythropoietic protoporphyria with myelodysplastic syndrome who has homozygous IVS3‐48C polymorphism in the ferrochelatase gene

We report the case of a 42‐year‐old man with a 5‐year history of myelodysplastic syndrome and photosensitivity who had developed painful erythema and blisters on sun‐exposed sites. Histological examination of a mildly lichenified lesion on the dorsal finger revealed extensive deposits of a hyaline‐like, periodic acid‐Schiff‐positive material around superficial dermal blood vessels. Laboratory tests showed elevated erythrocyte protoporphyrin and normal urinary porphyrins, suggesting a diagnosis of erythropoietic protoporphyria. Late‐onset erythropoietic protoporphyria is rare and is usually associated with an acquired somatic mutation of the ferrochelatase gene secondary to a hematological malignancy such as myelodysplastic syndrome. DNA analysis revealed that our patient has the homozygous IVS3‐48C polymorphism that is a low‐expression variant of wild‐type ferrochelatase allele.     

Erythema multiforme majus (Fuchs syndrome) associated with Mycoplasma pneumoniae infection in two patients

In Germany Fuchs syndrome is used to describe a variant of erythema multiforme majus which mainly involves the mucosal surfaces. As the skin may be completely unaffected, it is an under‐recognized diagnosis and often difficult to confirm. Clinical features involve erythema, erosions and ulcerations of the oral mucosa. In most cases there is severe conjunctivitis and sometimes the genital mucosa is involved. Most cases of Fuchs syndrome are triggered by infections; herpes simplex virus and Mycoplasma pneumoniae are the most common causes. We describe two women presenting with Fuchs syndrome after respiratory illness caused by Mycoplasma pneumoniae.     

Anti-ribosomal-P antibodies in a Sjögren syndrome patient associated with lupus erythematosus

We describe a 36‐year‐old woman who had Sjögren syndrome with anti‐ribosomal P antibodies and who developed erythema on her back and arms. The histological specimen showed lymphocytic infiltration around the sweat and salivary glands. Her serum reacted with ribosomal‐P0 protein in immunoblotting with HeLa cell extract and P0 recombinant protein. Although autoantibodies to ribosomal‐P proteins appear mainly in patients with systemic lupus erythematosus (SLE), a diagnosis of SLE in this patient could not be supported.     

Glucagonoma syndrome and necrolytic migratory erythema

The glucagonoma syndrome is a rare disorder, characterized by necrolytic migratory erythema, elevated serum glucagon levels, abnormal glucose tolerance, weight loss, and anemia in association with a glucagon‐secreting alpha‐cell tumor of the pancreas. We present a 67‐year‐old diabetic patient with extensive cutaneous lesions, weight loss, and poor glycemic control. The clinical investigation revealed a pancreatic glucagonoma with resolution of the cutaneous and systemic features after surgical removal. The dermatologic and endocrine approach to this syndrome is discussed here. Early recognition and treatment may prevent metastatic disease and ensure its cure with resolution of the cutaneous and catabolic manifestations.     

Erythema multiforme due to Mycoplasma pneumoniae infection in two children

Mycoplasma pneumoniae is an important and highly relevant cause of bullous erythema multiforme, isolated mucositis, and Stevens-Johnson syndrome in children. In this article, we present two children with respiratory Mycoplasma pneumoniae infection and associated cutaneous findings within the spectrum of erythema multiforme. We review the literature associating these three entities with Mycoplasma pneumoniae infection and discuss controversies regarding the classification of erythema multiforme, as well as update reported infectious causes of the bullous form. Many understand the erythema multiforme spectrum to include bullous erythema multiforme, mucositis, and Stevens-Johnson syndrome in the order of increasing severity. We feel that this relationship should be reconsidered to help better understand the prognosis and outcomes. It is our opinion that bullous erythema multiforme is a separate, yet related condition that can occur in the context of Mycoplasma pneumoniae infection. With many similarities to mucositis and Stevens-Johnson syndrome, bullous erythema multiforme can be considered part of a spectrum of disease that includes Stevens-Johnson syndrome. Unlike mucositis and Stevens-Johnson syndrome, bullous erythema multiforme caused by Mycoplasma pneumoniae infection has low morbidity for the child. Mycoplasma pneumoniae-associated mucositis and Stevens-Johnson syndrome seem to occur along a spectrum with separate prognosis and potential pathogenesis compared with bullous erythema multiforme. Making the distinction between these conditions is valuable for predicting the child's prognosis. Patients who develop symptoms consistent with these conditions should be appropriately evaluated for Mycoplasma pneumoniae infection and closely monitored.     

Skin manifestations in autoinflammatory syndromes

Autoinflammatory diseases encompass a group of inflammatory diseases that are non‐infectious, non‐allergic, non‐autoimmune and non‐immunodeficient. The term was initially coined for a small group of familial periodic fever syndromes of which familial Mediterranean fever (FMF) is the most common and best known. Genetic and molecular analyses demonstrated for the majority of these diseases an impairment of inflammasomes to cause an increased activity of an interleukin‐1‐dependent inflammatory response. Over the last years an increasing number of either rare hereditary syndromes or acquired common diseases could be summarized under the designation of autoinflammatory disease, thus creating an emerging new rubric of inflammatory diseases. Many of them display cutaneous manifestations as both concomitant or more rarely main symptoms. To name some of them like erysipelas‐like erythema in FMF; urticaria‐like rashes in tumor necrosis factor receptor 1‐ or cryopyrin‐associated periodic syndromes (TRAPS, CAPS), hyperimmunoglobulin D syndrome (HIDS) or Schnitzler syndrome; pyoderma gangrenosum and acne in PAPA syndrome; or behçetoid aphthous ulcerations in HIDS and PFAPA syndrome. Based on the new insights into pathogenesis one increasingly realizes the good response of these diseases to IL‐1 antagonist therapies.     

Clinical and molecular characterization of two patients with palmoplantar keratoderma–congenital alopecia syndrome type 2

Palmoplantar keratoderma–congenital alopecia (PPKCA) syndrome is a rare genodermatosis, with two clinically recognizable forms: dominant (Type 1) and recessive (Type 2). Reports of only 18 patients have been published to date, and the molecular basis of the condition is unknown. We describe two cases with PPKCA Type 2 (PPKCA2), comprising a novel patient, originally reported as an example of autosomal ichthyosis follicularis–atrichia–photophobia syndrome, and the 6‐year follow‐up of a previously published case. Extensive molecular studies of both patients excluded mutations in all the known genes associated with PPK and partially overlapping syndromes. The striking similarities between these two patients confirm PPKCA2 as a discrete genodermatosis, of which the main features are congenital and universal alopecia, diffuse keratosis pilaris, facial erythema, and a specific PPK with predominant involvement of the fingertips and borders of the hands and feet, with evolution of sclerodactyly, contractures and constrictions. Clinical follow‐up of these patients has demonstrated progressive worsening of the hand involvement and attenuation of facial erythema.     

Painful indurated erythema suggestive of Kikuchi-Fujimoto disease in a patient with primary Sjögren's syndrome

We report a patient with primary Sjögren's syndrome who developed pyrexia, cervical lymphadenopathy, and painful indurated erythema on the forehead, back, chest, abdomen, and limbs. Laboratory data showed an elevated erythrocyte sedimentation rate, C‐reactive protein and CH50 in addition to existing autoantibodies including anti‐nuclear antibody, anti SS‐A antibody, and anti SS‐B antibody. A skin biopsy specimen showed focal infiltration of histiocytes with non‐neutrophilic karyorrhetic debris in the dermis and subcutaneous fat tissue. Immunohistochemically the infiltrated cells were stained for CD68, suggesting cutaneous involvement of Kikuchi‐Fujimoto disease. All symptoms and laboratory data improved within three weeks after treatment with 20 mg/day of prednisolone. The present case suggests that a pathophysiological condition similar to Kikuchi‐Fujimoto disease can develop during the long‐term course of Sjögren's syndrome.     

Clinical studies of skin manifestations of Sj?gren's syndrome

The skin manifestations of the patients with definitive Sj?gren's syndrome were clinically studied. Chief complaints at the first consultation included annular erythema, pernio-like erythema, Raynaud's phenomenon, purpura, and malar erythema. Drug eruptions and xerotic eczema were also occasionally observed during the course. However, annular erythema, pernio-like erythema, drug eruptions, purpura, facial pigmented patches, and erythema nodosum were observed much more frequently in patients with primary Sj?gren's syndrome. These skin manifestations might be useful clues for the diagnosis of Sj?gren's syndrome in dermatological fields.     

Thalidomide: dermatological indications, mechanisms of action and side-effects

Thalidomide was first introduced in the 1950s as a sedative but was quickly removed from the market after it was linked to cases of severe birth defects. However, it has since made a remarkable comeback for the U.S. Food and Drug Administration‐approved use in the treatment of erythema nodosum leprosum. Further, it has shown its effectiveness in unresponsive dermatological conditions such as actinic prurigo, adult Langerhans cell histiocytosis, aphthous stomatitis, Behçet's syndrome, graft‐versus‐host disease, cutaneous sarcoidosis, erythema multiforme, Jessner–Kanof lymphocytic infiltration of the skin, Kaposi sarcoma, lichen planus, lupus erythematosus, melanoma, prurigo nodularis, pyoderma gangrenosum and uraemic pruritus. This article reviews the history, pharmacology, mechanism of action, clinical uses and adverse effects of thalidomide.     

Methotrexate-induced acral erythema with bullous reaction

Chemotherapy-induced acral erythema (CIAE), a toxic reaction to a number of different chomotherapeutic agents, causes a symmetrical, painful erythema of both the palms and soles which is self-limiting. The association of this syndrome with methotrexate is unusual; only nine cases have been reported in the literature. We describe the tenth case of this syndrome associated with methotrexate, which is also the third case of the bullous variant of methotrexate-induced acral erythema. Our case is unusual in that the acral erythema was present only on the soles of the feet and in that it was associated with the presence of diffuse maculopapular lesions over the legs and trunk.     

Erythema nodosum in Sweet's syndrome

A 36-year-old man had Sweet's syndrome associated with plaques on the legs that were typical of erythema nodosum both clinically and histologically. To our knowledge, true erythema nodosum has not previously been recognized as part of Sweet's syndrome.     

Sweet综合征28例误诊分析

目的分析Sweet综合征的误诊原因,为正确诊断Sweet综合征提出建议。方法对28例Sweet综合征的临床资料及误诊经过进行了回顾性分析。结果28例Sweet综合征患者分别被误诊为多形红斑、荨麻疹、结节性红斑、体癣、变应性血管炎等。结论临床医生应加强对Sweet综合征诊断技术的学习,避免误诊。