Henoch-Schönlein purpura in a child with hyperimmunoglobulinemia D and periodic fever syndrome

Abstract: This report describes a 3‐year‐old girl with a long history of periodic fever who presented with Henoch‐Schönlein purpura. She was diagnosed with hyperimmunoglobulinemia D and periodic fever syndrome by means of mutation analysis of the mevalonate kinase gene. The serum IgA concentration was markedly elevated, but the serum IgD concentration was normal. This report emphasizes that Henoch‐Schönlein purpura may be an important clinical feature of hyperimmunoglobulinemia D and periodic fever syndrome. In addition, this syndrome should be considered in patients with Henoch‐Schönlein purpura in whom there is a history of recurrent fevers, even when the serum IgD concentration is normal.     

Large annular purpura and paraneoplastic purpura in a patient with Sjögren's syndrome and cervical cancer

We report a 79‐year‐old female with anaphylactoid purpura on her legs and unusual large annular purpura on the trunk. Histopathological characteristics of leukocytoclastic vasculitis were observed in the upper and middle dermis of both types of skin lesions. She was complicated by Sjögren's syndrome and advanced cervical cancer. The annular purpura spontaneously resolved in a week and did not recur. However, the anaphylactoid purpura relapsed more frequently and spread more widely following the elevation of her serum SCC antigen levels from the onset of purpura until her death. We consider that the characteristic annular configuration was caused by the complication of Sjögren's syndrome and that the recurrent anaphylactoid purpura indicated paraneoplastic vasculitis primarily caused by the tumor specific protein immune complexes. Complication by Sjögren's syndrome many also play a role in the development of allergic vasculitis.     

Hemorrhagic bullous lesions in a child with Henoch-Schönlein purpura

Abstract: An 8‐year‐old boy with Henoch–Schönlein purpura who developed hemorrhagic bullae is described. The occurrence of hemorrhagic bullae in children with Henoch–Schönlein purpura is rare. A perusal of the literature revealed but seven occurrences to which we add another one.     

Predictive factors of relapse in adult with Henoch-Schönlein purpura

Henoch-Sch?nlein purpura (HSP) is an IgA-mediated small vessel vasculitis with a predominant cutaneous involvement. We assessed adult patients with HSP to identify the clinical and histopathological features and evaluate predictive factors of relapse. We reviewed the records of 29 adult patients with HSP who presented at our department between 2002 and 2009. Adult HSP was confirmed by skin biopsy showing leukocytoclastic vasculitis and direct immunofluorescence showing IgA deposit. Among the 29 patients (15 men, 14 women; mean age 36.2 years old), renal involvement was initially found in 22 patients (75.9%). They were divided into 2 groups according to the presence or absence of relapse. We compared clinical and histopathologic differences between 15 patients with relapse and 14 patients without relapse. By univariate analysis, older age at onset, persistent rash, abdominal pain, hematuria, and underlying disease at the onset of HSP are significantly related to relapse. Among the histopathological variables, severity of leukocytoclasis and absence of IgM deposit on the vessel walls are significantly associated to relapsing disease (P < 0.05). Our results are significant, because, they may help to understand the predictive factors related to relapses of HSP in adults. Further studies are necessary to identify whether more aggressive treatment in adults with HSP with these predictive factors can prevent relapse and severe renal sequelae.     

Adult Henoch-Schönlein purpura with severe abdominal pain treated with dapsone and factor XIII concentrate

We describe an adult patient with Henoch‐Schönlein purpura who had arthralgia, severe abdominal pain, and low plasma factor XIII activity. Corticosteroids were not used because of his history of multidrug‐resistant pulmonary tuberculosis. Dapsone had no immediate effect on his abdominal pain, but appeared to have some effect on the purpura and arthralgia. Marked improvement of the abdominal pain was observed immediately after the administration of factor XIII concentrate. Factor XIII concentrate may be useful for alleviating abdominal pain in Henoch‐Schönlein purpura patients when corticosteroids are contraindicated     

Henoch-Schönlein purpura in pregnancy

Henoch–Schönlein purpura (HSP) is an IgA‐mediated small vessel vasculitis which commonly involves the skin, gastrointestinal system and kidneys. Numerous HSP triggers have been identified, and pregnancy has been reported as an exacerbating factor. After a pregnant woman had been diagnosed as having new‐onset HSP, we reviewed all cases of immunofluorescence‐proven HSP evaluated by the Department of Dermatology at the Johns Hopkins Hospital between 1990 and 2002, and report three cases of HSP occurring during pregnancy. Two patients developed new‐onset HSP, one at 16 weeks gestation and one at 22 weeks, while the third developed a recurrence of HSP at 12 weeks gestation after 19 years of remission. We conclude that pregnancy may be a trigger for HSP onset or recurrence in susceptible individuals.     

Systemic Hodgkin's lymphoma in a patient with Sézary syndrome

We report a case of a 71-year-old male with Sézary syndrome diagnosed in 1996 who subsequently developed systemic Hodgkin's lymphoma. His only past treatment was bath psoralen plus ultraviolet A. He has since been treated with multiagent chemotherapy (ChlVPP/PABLOE) which induced a remission in his Hodgkin's disease. Eighteen months later he remains in remission from Hodgkin's disease but the Sézary syndrome remains active. He has also developed a squamous cell carcinoma on the upper lip. Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by a malignant proliferation of CD4-positive cells in the skin and peripheral circulation. The CD4 count may be markedly elevated but this results from expansion of a neoplastic T-cell clone and there is a relative lymphopenia of normal T cells leading to a degree of immunoparesis. Immunosuppression is known to be associated with an increased rate of malignancies and this may account for the occurrence of Hodgkin's disease and squamous cell carcinoma in this patient with Sézary syndrome.     

Relapsing Henoch-Schönlein purpura in an adult patient associated with hepatitis B virus infection

Henoch‐Schölein purpura is usually a disease of children presenting with arthralgia, abdominal pain, renal involvement, and palpable purpura. Viral and bacterial infections may have a role in its etiology. We present a 32‐year‐old male patient with recurrent Henoch‐Schölein purpura in association with a chronic hepatitis B infection of ten years duration. The patient had received lamuvudine and interferon‐α for the treatment of hepatitis B infection for a year. The skin lesions disappeared with the treatment of the hepatitis B infection. Four months after discontinuation of the therapy, the purpuric papules reappeared with reactivation of the hepatitis B infection. Although rarely reported, hepatitis B virus infection should be considered in patients with Henoch‐Schölein purpura.     

Nonnecrobiotic necrobiotic xanthogranuloma as an initial manifestation of paraproteinemia and small lymphocytic lymphoma in a patient with Sjögren syndrome

We report a unique case of periocular nonnecrobiotic necrobiotic xanthogranuloma in a 52-year-old white woman with Sj?gren syndrome who was subsequently found to have an immunoglobulin G paraproteinemia and coexisting small lymphocytic lymphoma. Therapy with fludarabine, cytoxan, and rituximab (FCR) resulted in a dramatic resolution of her sicca symptoms and periocular xanthogranulomas. This case further illustrates the association of hematolymphoid disorders with cutaneous xanthogranulomatous disease and the importance of additional appropriate laboratory and radiologic investigation for the accurate diagnosis of an underlying malignancy.     

Henoch-Schönlein purpura presenting with anuria in an adult

Henoch-Sch?nlein purpura (HSP) is an immune complex-mediated systemic small vessel vasculitis that is most commonly described in children but may affect patients of any age. Our patient, a 91-year-old man, presented with anuria caused by IgA-mediated nephropathy; he later developed cutaneous leukocytoclastic vasculitis, thereby meeting the criteria for a diagnosis of HSP. This case is unique because of the patient's initial presentation with anuria, the possible underlying malignancy associated with his HSP, and his advanced age.     

Henoch-Schönlein purpura presenting with orchitis: a case report and review of the literature

Henoch-Sch?nlein purpura (HSP) is the most common cause of nonthrombocytopenic purpura in children. The clinical picture is classically a cutaneous purpuric eruption of the legs and buttocks and infrequently the upper torso and extremities. Arthritis, gastrointestinal tract symptoms, and nephritis are other common findings typically associated with the cutaneous findings. We present an unusual case of HSP with scrotal swelling and orchitis.     

Bullous lesions in Henoch-Schönlein purpura

Abstract: Henoch‐Schönlein purpura (HSP) is a small‐vessel vasculitis characterized by purpura, arthritis, abdominal pain, and hematuria. It is the most common form of vasculitis affecting children. The skin manifestations typically present as palpable purpura over the buttock and the lower extremities, but bullous lesions are particularly rare in children. We report a case of HSP presented as painful bullae on both lower limbs.     

Livedoid vasculopathy and hypercoagulability in a patient with primary Sjögren's syndrome

Background A 31‐year‐old woman presented with a 5‐year history of painful ulcerations, palpable purpura, porcelain‐white atrophic scars of the malleolar region and dorsal aspect of the feet, livedo reticularis on the limbs, arthralgia, xerophthalmia, and xerostomia. Methods Skin biopsy revealed vessel wall hyalinization and thrombosis of the microvasculature with a very scarce dermal inflammatory infiltrate. Biopsy of the oral mucosa showed mononuclear infiltration of an intralobular duct of a salivary gland. Results Laboratory studies, including autoantibodies and inflammation markers, were normal, except for a positive rheumatoid factor. Coagulation screening revealed C677T methylenetetrahydrofolate reductase (MTHFR) mutation, with a normal serum homocysteine. The patient was treated with oral methylprednisolone (32 mg/day with progressive reduction) and enoxaparin (20 mg/day subcutaneously), with complete ulcer healing within 4 months. Conclusion Livedoid vasculitis or vasculopathy has not been referred to previously in association with Sjögren's syndrome, but may be associated with other autoimmune disorders and anomalies of coagulation, namely factor V Leiden mutation, protein C deficiency, and MTHFR mutation, associated or not with hyperhomocysteinemia, a condition that seems to confer an increased risk of recurrent arterial and venous thrombosis. We stress the importance of anticoagulant therapy for ulcer healing and for the prevention of other thrombotic events.     

Possible association of Henoch-Schönlein purpura in adults with odontogenic focal infection

Background Odontogenic focal infection (OFI) is considered to be an important precipitating factor in some cases of palmoplantar pustulosis and psoriasis. In Henoch–Schönlein purpura (HSP), it is clear that acute bacterial infection is one of the causes. However, it remains unclear how OFI participates in the pathogenesis of HSP. Methods To clarify how OFI is related to the disease activity of HSP in terms of skin lesions and organ involvement, we conducted a retrospective study of 28 cases of adult‐type HSP. Results Panoramic x‐ray screening detected OFI in 14 of the 28 patients. Patients with OFI had higher incidences of renal and gastrointestinal complications than those without OFI. Seven patients had severe OFI and underwent tooth extraction, resulting in a marked improvement of their skin lesions. Five of those seven patients exhibited transient flare‐up after the tooth extraction. Conclusions Latent OFI is an important infectious focus that is involved in the pathogenesis of HSP and influences its disease activity.