Hyper IgE syndrome—case history

The hyper IgE syndrome is a specific primary immunodeficiency disease of highly variable severity. Though the disorder is rare, patients are quite likely to present at some time to the dermatologist. Although the cutaneous manifestations of the hyper IgE syndrome are characteristic, they are non-specific and their significance may not be appreciated if a history of other infections, particularly of the respiratory tract, is not obtained. We describe here a typical case which presented in a paediatric dermatology clinic.     

Iatrogenic Cushing's syndrome—a cautionary tale

A case of Cushing's syndrome associated with relatively low dose application of topical clobetasol propionate (0.05%) is reported.     

Sweet's syndrome complicating isotretinoin therapy in acne

BACKGROUND: We report a case of juvenile acne aggravated in the form of Sweet's syndrome by isotretinoin treatment. The late onset of ulcerative-hemorrhagic rectocolitis in this patient raised doubts about a possible relationship between Sweet's syndrome, acne and inflammatory colitis. PATIENTS AND METHODS: A 19 year-old male patient with no disease history of note was treated for juvenile polymorphous acne resistant to standard topical acne treatment using isotretinoin (Roaccutane) at a dose of 0.5 mg/kg/d. After one week of treatment, the patient presented a fever of 38.5 degrees C, joint pain and congestive, erythematous-edematous, maculopapular plaques in ring-like layout subsequently becoming pustular and necrotic. These lesions occurred on the face, neck and pinna of the ear. Some nodules were also noted on the lower limbs. Biological tests and histology examination of a skin biopsy were evocative of Sweet's syndrome. The outcome was rapidly favorable following discontinuation of isotretinoin and institution of systemic corticosteroids (0.5 mg/kg/d). Two years later, ulcerative-hemorrhagic rectocolitis was diagnosed with episodes of bloody diarrhea. DISCUSSION: Treatment of acne with isotretinoin can occasionally induce inflammatory episodes of acne. To date there have been no reported cases of isotretinoin-induced Sweet's syndrome. The subsequent onset of ulcerative-hemorrhagic rectocolitis provides an indication of the complexity of the pathogenic mechanisms involved.     

Sweet's syndrome associated with Behçet's disease

A case of acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with Behçet's disease occurring in a 48 year old woman is reported. She fulfilled the diagnostic criteria for both Sweet's syndrome and Behçet's disease. There have been several reports of this association. Herpes simplex virus was isolated from a genital ulcer in our patient. The possible role of herpes simplex virus in this interesting association of Behçet's disease and Sweet's syndrome is discussed and a review of the literature is made.     

Carbamazepine-induced anticonvulsant hypersensitivity syndrome—pathogenic and diagnostic considerations

Two epileptic patients developed an infectious mononucleosis-like illness which subsequently proved to be a carbamazepine-induced anticonvulsant hypersensitivity syndrome. Patch testing to carbamazepine 3 years later was positive in the one patient tested and negative in normal controls. The second patient died a few weeks after the illness, secondary to long-standing cardiac disease without having undergone patch testing. A skin biopsy was, however, consistent with an immune complex mediated drug reaction. Patch testing for systemically administered drugs is generally believed to be of little value in diagnosing drug allergies. However, we reinforce a previous suggestion that this investigation may be helpful in some cases of anticonvulsant hypersensitivity syndrome caused by carbamazepine.¹ The pathogenic role of type 3 and 4 hypersensitivity is also discussed.     

Efficacious treatment of psoriasis with low‐dose and intermittent cyclosporin microemulsion therapy

Cyclosporin is used for moderate to severe psoriasis and improves not only the skin lesions but also quality of life of the patients. To improve its safe use, we evaluated a low‐dose, intermittent regimen of cyclosporin in the treatment of psoriasis vulgaris. Seventy‐three patients received approximately 2.5 mg/kg per day of cyclosporin microemulsion twice daily before breakfast and dinner for 2–12 weeks until 75% reduction was achieved in Psoriasis Area and Severity Index (PASI) score. When the skin lesions relapsed after cessation of cyclosporin and showed less than 50% reduction from baseline in PASI score, cyclosporin was restarted. This cessation and restart cycle was repeated if necessary. Treatment outcomes were assessed at 12, 48 and 96 weeks after initiation of the therapy. The initial dose of cyclosporin was 2.32 ± 0.27 (standard deviation [SD]) mg/kg per day. At baseline, the mean PASI score was 11.3 ± 5.3 (SD). An average of 49.8 ± 23.8 (SD) days of the therapy achieved PASI 75% reduction. In 20 of 73 patients, the second course of cyclosporin was required. The mean interval between the first and second course was 94 days. An average of 60.8 ± 26.9 days was required to achieve PASI 75% reduction in the second course, which was not significantly longer than that in the first course. Only six patients required cyclosporin for 96 weeks. The adverse effects included one case of hypertension. Our study suggests that low‐dose, intermittent cyclosporin microemulsion is efficacious for the treatment of moderate to severe psoriasis.     

Sweet's syndrome with pregnancy

A 31-year-old pregnant woman had eruptions on her wrist, face and neck. We diagnosed her as having Sweet's syndrome from clinical symptoms, histopathological and laboratory findings. We successfully treated her with prednisolone and there are no relapses after delivery. We studied her polymorphonuclear leukocyte activity by the polarization assay with N-formyl-methionyl-leucyl-phenylalanine as a chemoattractant and it is higher than that of control. It is known that Sweet's syndrome is accompanied with collagen diseases and malignant diseases or others as underlying diseases or conditions. Three cases of Sweet's syndrome associated with pregnancy have been reported before and this is the first one in Japan.     

Drug-induced Sweet's syndrome secondary to hepatitis C antiviral therapy

Pegylated interferon-alpha in combination with ribavirin currently represents the therapeutic standard for the hepatitis C virus infection. Interferon based therapy may be responsible for many cutaneous side effects. We report a case of drug-induced Sweet's syndrome secondary to hepatitis C antiviral therapy. To our knowledge, this is the first reported case of Sweet's syndrome in association with pegylated interferon-alpha therapy.     

Acrodermatitis continua responding to cyclosporin therapy

A 69-year-old man with painful, recalcitrant, localized acrodermatitis continua developed widespread pustulation which was resistant to high-dose methotrexate therapy. Low-dose cyclosporin induced a rapid and persistent remission.