Azathioprine therapy in the management of persistent erythema multiforme

Two cases of recurrent erythema multiforme of unknown aetiology are reported. The term ‘persistent’ is appropriate as the lesions recurred whenever steroid therapy was discontinued. Both patients were eventually controlled with azathioprine alone, and one patient required treatment for 3 years.     

A case of erythema multiforme associated with primary Epstein-Barr virus infection

We present a case of primary Epstein-Barr virus (EBV) infection with erythema multiforme. A 1-year-old Japanese boy presented with skin eruptions, including typical target lesions and a low-grade fever. Just before the skin biopsy, 95 copies/μg DNA of EBV genome was detected in peripheral blood mononuclear cells, which subsequently increased to 6,834 copies/μg DNA. Skin tissue collected from the skin lesion showed the typical pathologic findings of erythema multiforme. EBV-encoded small nuclear RNA signals were not detected in the skin tissue by in situ hybridization.     

Mast cells in oral erythema multiforme.

We compared the number of mast cells in erythema multiforme lesions, in clinically healthy mucosa between the EM attacks and in healthy mucosa from healthy volunteers. The mast cell count in patients with erythema multiforme was numerically higher than in healthy controls, but the differences were not statistically significant. In erythema multiforme lesions the mast cell count was low in the intensely inflamed superficial lamina propria, but high in normal appearing mucosa between the attacks suggesting local mast cell degranulation in the most intensely inflamed areas.     

Lichen planus, erythema nodosum, and erythema multiforme in a patient with chronic hepatitis C.

After identification of the hepatitis C virus (HCV) in 1989, evidence was established supporting its role in the pathogenesis of a number of cutaneous diseases. This evidence ranges from mere epidemiologic associations, such as lichen planus, to molecular biological investigations that have identified the virus in the pathologic tissues of cutaneous vasculitis, vasculitis with mixed cryoglobulinemia, and porphyria cutanea tarda. We describe a 52-year-old man who was diagnosed with chronic hepatitis C, preceding the appearance of lichen planus, erythema nodosum, and erythema multiforme that coincided with the reactivation of viral replication.     

Isomorphic phenomenon in erythema multiforme

The cutaneous lesions of erythema multiforme have a propensity to localize at previously inflamed or traumatized skin sites. Evidence of the isomorphic phenomenon may be demonstrated in most cases of erythema multiforme by a careful history and physical examination. We present three cases of erythema multiforme which illustrate localization of skin lesions around scratches, recent surgical scars, lacerations, traumatized nail folds and folliculitis. The isomorphic phenomenon may serve as an important clue in understanding the clinical features and possibly, the pathogenesis of erythema multiforme.     

Subcorneal pustules in erythema multiforme

A man aged 24, suffering from Stevens Johnson syndrome precipitated by an upper respiratory infection, developed a skin eruption which consisted of sterile pustules. Histological examination showed subcorneal pustules containing polymorphonuciear leukocytes and no abnormality of the basal part of the epidermis, an appearance indistinguishable from subcorncal pustular dermatosis. The eruption cleared rapidly and did not recur. It is emphasized that the histological appearance of a subcorneal pustule does not necessarily indicate a diagnosis of subcorneat pustular dermatosis (Sneddon & Wilkinson, 1956).     

Oculocutaneous manifestations of the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum.

Although they were once believed to be distinct diseases, many observers currently consider erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis to represent a mild-to-severe continuum of the same process. Each shares certain clinical, histologic, and etiologic characteristics; among the latter two are frequent ocular involvement. Because long-term ophthalmic sequelae may be catastrophic, early recognition and therapy of this acute oculocutaneous disease spectrum are critically important.     

Distinguishing between erythema multiforme major and Stevens-Johnson syndrome/toxic epidermal necrolysis immunopathologically

The early clinical presentations of Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are similar to that of erythema multiforme major (EMM). Cytotoxic molecules, especially granulysin, are expressed in the skin lesions of SJS/TEN and cause extensive keratinocyte death. It is postulated that the function of regulatory T cells (Treg) in SJS/TEN is inadequate. This study examined whether an immunohistological examination of cytotoxic molecules and the immunophenotype of Treg is useful for discriminating SJS from EMM in the early period. Over the past 9 years, the lesional skin of 14 patients with SJS/TEN and 16 patients with EMM was biopsied. Double immunofluorescence labeling of CD8 and granulysin, perforin, or granzyme B was performed, and immunohistochemical analyses of granulysin, perforin, granzyme B, CD1a, CD3, CD4, CD8, CD68 and Foxp3 were conducted using a highly sensitive indirect immunoperoxidase technique. The number of cells positive for each antibody per five high‐power fields was counted. The proportions of granulysin⁺ cells/CD8⁺ cells (P = 0.012) and perforin⁺ cells/CD8⁺ cells (P = 0.037) in SJS/TEN were significantly higher than in EMM. The number of Foxp3⁺ cells/five high‐power fields in SJS/TEN was significantly lower than in EMM (P = 0.004). Similarly, the number of CD4⁺ cells/five high‐power fields in SJS/TEN was significantly lower than in EMM (P = 0.0017). These data suggest that these panels of antibodies for labeling cytotoxic molecules, CD4 and Treg are useful for discriminating early SJS/TEN and EMM with a skin biopsy.     

Erythema nodosum and erythema multiforme associated with milker's nodules.

An epidemic of 44 cases of milker's nodules was recorded in the Tampere Central Hospital catchment area in Finland during the autumn of 1974. Exanthema or erythema multiforme-like secondary eruptions were seen in 10 cases. One female patient is reported in detail because of the simultaneous occurrence of erythema nodosum and erythema multiforme. The virological diagnosis was confirmed by electron microscopy.     

妊娠期重症多形红斑一例

患者女．27岁，因双眼肿痛4天，双唇红肿水疱3天，躯干红斑2天于2006年4月17日入住我科。入院前4天患者无明显诱因自觉双眼肿痛，无分泌物，无发热，遂到外院就诊，予红霉素眼膏，鱼腥草注射液治疗4天，疗效不佳。3天前出现双眼结膜充血，双唇红肿，唇周出现数个粟粒大小水疱，疱液澄清，易破（图1），口腔黏膜糜烂，表面出现灰白色假膜，疼痛明显，同时外阴也出现糜烂，[第一段]