A case of varicella-associated idiopathic thrombocytopenic purpura in adulthood

We describe here an adult case of varicella-associated idiopathic thrombocytopenic purpura (ITP). A 23-year-old Japanese male presented with generalized hemorrhagic vesicles and fever. Laboratory examinations revealed severe thrombocytopenia associated with anti-platelet IgG auto-antibody. The patient rapidly recovered within a few days after antiviral agent (vidarabine) administration, and the platelet count returned to normal with skin lesion improvement. Idiopathic thrombocytopenic purpura associated with viral infection in adulthood is relatively rare.     

Pityriasis lichenoides and idiopathic thrombocytopenic purpura in a young girl

Pityriasis lichenoides is an inflammatory skin disorder characterized by erythematous, desquamative papules and plaques. An acute form, pityriasis lichenoides et varioliformis acuta, and a chronic form, pityriasis lichenoides chronica, represent the two ends of the spectrum of this disorder. Most commonly seen in children and young adults, its etiology is unknown. We describe a young patient with concurrent pityriasis lichenoides and idiopathic thrombocytopenia purpura, a previously unreported association.     

Tuberculous gumma associated with idiopathic thrombocytopenic purpura: report of a Japanese female patient

Our patient was a 77-year-old Japanese woman, who was under treatment for idiopathic thrombocytopenic purpura (ITP) and chronic renal failure. She had warm nodules and cord-like induration on her left knee that appeared similar to Bazin's erythema induratum. A chest X-ray examination revealed miliary tuberculosis; after anti-mycobacterial therapy, the warm nodules and cord-like induration were transformed into a cold, non-tender abscess that drained. Histopathological findings showed caseation necrosis in the subcutaneous tissue, however, mycobacteria were directly detected by Ziehl-Neelsen staining from cutaneous lesions, and cultures from the same lesion also grew Mycobacterium tuberculosis. Tuberculous gumma demonstrated multiple, cold, painless abscesses and thrombosis were also seen. This is a rare and unusual clinical form of cutaneous tuberculosis, and the result of hematogenous dissemination from a primary focus during periods of lowered resistance.     

A novel anti-ENA antibody in sera of patients with childhood idiopathic thrombocytopenic purpura

Circulating antibodies against certain extractable nuclear antigens (ENA) have been shown to have diagnostic and prognostic importance in connective tissue diseases. We described here an antibody against ENA found in the sera of patients with idiopathic thrombocytopenic purpura (ITP). The antigen, tentatively called WK according to the patients' initials, was distinct from U1 RNP/Sm, SSA/SSB, Scl-70, PCNA, PM-Scl, Jo-1, and Ku by immunodiffusion. On immunoblotting, the anti-WK serum recognized polypeptides of 99 kd, 98 kd, and 96 kd in rabbit thymus extracts and a 99 kd polypeptide in KB cell extracts. The anti-WK antibody was detected in the sera of 2 out of 360 ANA positive patients, both children with clinical features of ITP. One patient developed systemic lupus erythematosus eight years after the onset of ITP.     

水痘并血小板减少性紫癜一例

患儿女,10岁,因躯干、四肢瘀点、无痛痒1天于2008年6月5日来诊.就诊前1天,患儿父母无意中发现其躯干、四肢散在分布针尖大瘀点,无发热、乏力、咳嗽、咽痛等自觉症状,既往无贫血、出血病史.体检:体温36.5℃,神志清,精神可,左眼内眦处球结膜下出血,咽部略红,扁桃体不肿大,心肺腹无异常发现.肝脾未触及,浅表淋巴结未触及肿大.皮肤科检查:躯干、双上肢、双下肢弥漫性分布针尖至粟粒大紫红色瘀点,无融合,未见明显丘疹、水疱等损害.     

Simultaneous reactivation of herpes simplex virus and varicella-zoster virus in a patient with idiopathic thrombocytopenic purpura

Simultaneous reactivation of distinct Herpesviridae with development of clinical manifestations is exceptional. We report a 48-year-old woman suffering from idiopathic thrombocytopenic purpura. As the disease remained refractory to corticosteroids, immunoglobulins and splenectomy, a cure of vinblastine was administered. An atypical stomatitis developed few days later. Immunohistochemistry on a Tzanck smear and a biopsy evidenced a Herpes simplex virus type 1 (HSV-1) infection. The patient presented simultaneously a single necrotic lesion on the abdomen. Immunohistochemistry on a skin biopsy revealed the presence of the varicella-zoster virus (VZV) gE, gB and IE63 proteins. Intravenous aciclovir was initiated. The present case of simultaneous clinical infections by HSV-I and VZV underlines the importance of complementary viral identification testing in the event of unusual clinical presentations.     

Herpes zoster with cutaneous dissemination in a patient 21 years after splenectomy for idiopathic thrombocytopenic purpura

Background:Varicella-zoster virus is a member of the herpes virus family that causes varicella during a primary infection and herpes zoster (HZ) when reactivated. Patients who are immunocompromised often have atypical presentations of HZ and experience complications such as multidermal involvement and dissemination.Objective:We report a case of disseminated HZ in an immunocompetent patient with a history of splenectomy for idiopathic thrombocytopenic purpura (ITP). Our 46-year-old female patient presented with a painful vesiculopapular dermatomal rash with approximately 80 other lesions diffusely spread over her body. She was in good health but had a splenectomy for ITP 21 years earlier and a history of recurrent herpes labialis. The latter led to the tentative diagnosis of a widespread herpes simplex infection. However, laboratory results confirmed a diagnosis of disseminated herpes zoster. A workup of the patient's immune status did not reveal any abnormalities other than the patient's previously noted splenectomy.Conclusions:This case adds to the two reports of patients developing cutaneous disseminated HZ several years after splenectomy. Our case serves as a reminder that patients with a history of splenectomy appear to be at increased risk for cutaneous dissemination of HZ.     

Case of atopic dermatitis concurrent with idiopathic thrombocytopenic purpura,whose serum thymus and activation‐regulated chemokine level remained undetectable

We report a 9‐year‐old Japanese female patient with atopic dermatitis associated with idiopathic thrombocytopenic purpura. She demonstrated high serum immunoglobulin (Ig)E and IgE specific to several environmental allergens, but extremely low serum thymus and activation‐regulated chemokine (TARC) levels regardless of the disease progression. This case suggests platelets as the main source of serum TARC.     

Thyroid autoimmunity in chronic idiopathic urticaria: implications for therapy

The association between thyroid autoimmunity and chronic idiopathic urticaria has long been recognized, although prevalence rates differ in the studies reported to date (from 12 to 29%). There is, therefore, a strong indication to screen patients affected by chronic urticaria of unknown origin for thyroid antibodies (antithyroperoxidase and antithyroglobulin) and, when positive, for serum thyrotropin to assess thyroid functional status. Less clear is the implication of thyroid autoimmunity for therapy, as most patients with urticaria who have associated thyroid autoimmunity are euthyroid. There is no doubt that cases with clinical or subclinical thyroid dysfunction should undergo treatment with either levothyroxine or antithyroid drugs for hypo- or hyper-function, respectively. Although the best remission rates for symptoms of urticaria have so far been obtained with levothyroxine in patients who are euthyroid, monitoring of thyroid function through serum thyrotropin determination is highly recommended because of the risk of hyperthyroidism, especially in the elderly.     

Skin inflammation: reactive oxygen species and the role of iron.

Superoxide and hydrogen peroxide are reactive oxygen species (ROS) primarily produced by phagocytic cells as a consequence of the process of phagocytosis. This defensive role, may, however, become one of attack when production of ROS is excessive and overwhelms cellular scavenging systems. This happens in situations such as acute inflammation and results in host cell membrane damage, which is particularly prevalent in the presence of transition metal catalysts such as iron and copper. The skin is uniquely vulnerable to this attack being rich in polyunsaturated fatty acids and exposed to high oxygen tensions and ultraviolet light, both of which promote production of ROS. Additionally, the respiratory burst of infiltrating polymorphonuclear leukocytes and macrophages in inflamed skin will produce high local levels of superoxide that can release "catalytic iron" from storage proteins such as ferritin. The role of iron and ROS in the pathogenesis of inflammatory skin disease is discussed as is the possibility of novel therapeutic strategies based on their removal.     

Long-term after care in chronic skin diseases exemplified by idiopathic hyperlipidemic xanthomatosis

Based on two detailed case reports of idiopathic hyperlipemic xanthomatosis, we discuss the problems concerning xanthomatosis. The important role of a long-term follow-up of these patients as, in our cases, over 16 and 27 years, resp., by the same doctor is pointed out.     

Clinical characteristics of pruritus in chronic idiopathic urticaria

BACKGROUND: Although pruritus is a predominant symptom of chronic idiopathic urticaria (CIU) its clinical characteristics have not been explored. OBJECTIVES: To characterize the clinical pattern and sensory and affective dimensions of the itch experience, utilizing a comprehensive itch questionnaire. METHODS: A structured questionnaire based on the McGill pain questionnaire was used in 100 patients suffering from CIU randomly recruited from a tertiary referral centre. RESULTS: All 100 patients recruited with CIU completed the questionnaire. In 68 patients pruritus appeared on a daily basis. Most patients experienced their pruritus at night and in the evening (n = 83), and 62 reported difficulty in falling asleep. Pruritus involved all body areas, but mostly the arms (n = 86), back (n = 78) and legs (n = 75). Accompanying symptoms were a sensation of heat in 45 patients and sweating in 15. Most patients (n = 98) were prescribed antihistamines (mainly sedating), of whom 34 experienced long-term relief. The sensation of itch was reported to be stinging (n = 27), tickling (n = 25) and burning (n = 23). Seventy-six patients found their pruritus bothersome, 66 annoying and 14 complained of depression. The itch intensity at its peak was more than double that felt after a mosquito bite. The worst itch scores of those who felt depressed were significantly higher than of those who did not (P = 0.018). There was a positive correlation between the sensory and affective scores during worst itch (P < 0.001). CONCLUSIONS: This study describes the itch experienced in CIU, highlighting sensory and affective dimensions. The itch questionnaire was found to be a valuable tool for evaluating pruritus in CIU and its unique features.