Hikaru Eto

Therapeutic depletion of myeloid lineage leukocytes in patients with generalized pustular psoriasis indicates a major role for neutrophils in the immunopathogenesis of psoriasis

BACKGROUND Generalized pustular psoriasis (GPP) is a chronic autoimmune disease characterized by fever, erythema, and neutrophilic pustules over large areas of the skin. GPP does not respond well to pharmacologic intervention. OBJECTIVE We sought to assess efficacy of selectively depleting the myeloid lineage leukocytes in patients with GPP. METHODS Fifteen patients with persistent moderate to severe GPP despite conventional therapy were included. Eligible patients had more than 10% of their skin area covered by pustules. Treatment with oral etretinate, cyclosporine, methotrexate, prednisolone, and topical prednisolone/vitamin D3 was continued if had been initiated well in advance of study entry. Five sessions of adsorptive granulocyte and monocyte apheresis (GMA) with the Adacolumn (JIMRO Co Ltd, Takasaki, Japan) were administered (1 session/wk over 5 weeks) to selectively deplete Fcγ receptor and complement receptor bearing leukocytes. Efficacy was assessed by measuring the skin areas covered by pustules at baseline and 2 weeks after the last GMA session. RESULTS One patient did not complete the first GMA session. Based on the GPP severity scores relative to entry, the overall scores improved (n = 14, P = .0027), and the area of erythroderma (P = .0042), pustules (P = .0031), and edema (P = .0014) decreased. Likewise, Dermatology Life Quality Index improved (P = .0016), reflecting better daily function and quality of life. Twelve patients were judged as responders (85.7%), and 10 patients maintained the clinical response for 10 weeks after the last GMA session without any change in medication. LIMITATIONS This study was unblinded and without a placebo arm. CONCLUSION GMA in this clinical setting was safe and effective, suggested a major role for granulocytes/monocytes in the immunopathogenesis of GPP.

Neonatal lupus erythematosus in Japan: A review of the literature

Neonatal lupus erythematosus (NLE) is an autoimmune disease associated with maternal anti-SS-A/Ro and anti-SS-B/La antibodies. NLE is characterized by cutaneous erythema, congenital heart block (CHB), hepatic dysfunction and hematological abnormalities. CHB is irreversible, usually requiring a pacemaker, but other symptoms are reversible and most disappear within 6 months in parallel with declining antibody levels. In Japan, 193 cases of NLE were reported between 1971 and 2008. Most showed erythema, and only 23% of cases presented with CHB. Conversely, antibody status had not been examined in many infants presenting with CHB during the same period. Most pregnant woman with anti-SS-A/Ro and anti-SS-B/La antibodies are asymptomatic, and antibody status is first indicated when their child shows symptoms of NLE. These women show a greater risk of delivering an infant with CHB than normal. CHB is important because the main morbidity and mortality of NLE is from CHB. All clinicians should be familiar with the characteristics of NLE. We believe all pregnant women should be screened for anti-SS-A/Ro and anti-SS-B/La antibodies.

Transient bullous dermolysis of the newborn: Two additional cases

Two cases of transient bullous dermolysis of the newborn are reported. The first patient, a white boy, had normal skin at birth, but multiple blisters soon developed. The oral mucous membranes were not affected. All lesions healed within 4 months without scars but with many milia. At the age of 17 months the boy was reexamined and was found to have no blisters or milia. The second patient, a Japanese baby girl, had extensive denudation of her hands at birth. Generalized blisters and involvement of the oral mucous membrane developed. Blistering stopped within 1 1/4 months, and all lesions healed without scars. Histologically the blisters were subepidermal in both cases. Some lesional basal cells contained periodic acid-Schiff-positive inclusions. Electron microscopy revealed collagenolysis, diminution or loss of anchoring fibrils, and stellate inclusions in dilated rough endoplasmic reticulum in the keratinocytes of the lower epidermis. These stellate inclusions consisted of filamentous bundles with 25 to 33 nm cross-striations.

Imaging of vascular tumors with an emphasis on ISSVA classification

The International Society for the Study of Vascular Anomalies (ISSVA) classification is becoming the international standard classification system for vascular tumors and vascular malformations. The ISSVA classification strictly distinguishes vascular tumors (neoplastic lesions) from vascular malformations (non-neoplastic lesions) based on whether there is a proliferation of vascular endothelial cells present, and it is an extremely useful classification system for determining therapeutic measures. For vascular tumors, it is clinically significant in terms of discriminating infantile hemangioma and rapidly involuting congenital hemangioma, which are expected to spontaneously regress, from other vascular tumors requiring treatment. Needless to say, clinical courses are important for diagnosis, and it is also important for radiologists to understand imaging findings on vascular tumors because such tumors have unique findings on diagnostic images. In this paper, vascular tumors are classified based on the ISSVA classification, and clinical and imaging findings are reviewed.

Acquired reactive perforating collagenosis successfully treated with narrow-band ultraviolet B.

© 2009 The Authors. doi: 10.2340/00015555-0685 Journal Compilation

Experience with the use of hydroxychloroquine for the treatment of lupus erythematosus

Hydroxychloroquine (HCQ) is generally used to treat systemic lupus erythematosus (SLE) in Western countries. However, chloroquine retinopathy became a problem in Japan, and chloroquine has never been used since then. Even now HCQ remains non‐approved. Therefore, the Japanese Hydroxychloroquine Study Group has been organized, and activities have started to have HCQ approved within Japan. In the present study, we investigated the effectiveness of HCQ against the skin manifestations of lupus erythematosus. There were seven patients, all female, and they consisted of four patients with SLE (skin lesion type: discoid lupus erythematosus [DLE] in three, subacute cutaneous lupus erythematosus in one and lupus erythematosus profundus in one), two patients with cutaneous lupus erythematosus (both DLE), and one patient with a combination of SLE and dermatomyositis. HCQ was effective in three patients and ineffective in the two patients. We could not judge the efficacy of HCQ in the other two patients. There were no adverse effects in any of the patients. Efficacy was exhibited against telangiectasia and erythema. HCQ is also an effective and safe treatment for Japanese patients, and it is hoped that it will be approved for use in Japan very soon.

Microscopic polyangiitis presenting urticarial erythema and Henoch-Schönlein purpura: Two case reports

Microscopic polyangiitis (MPA) is well known as a life‐threatening member of a group of systemic vasculitis diseases. We report two cases of MPA. Case 1 was a 79‐year‐old‐man who had been diagnosed with anti‐neutrophil‐cytoplasmic‐antibody associated vasculitis (ANCA associated vasculitis) with alveolar hemorrhage and crescentric glomerulonephritis (CrGN). He presented with urticarial erythema in the abdomen, legs and back. The skin biopsy specimens showed leukocytoclastic vasculitis on the upper dermis. Case 2 was a 74‐year‐old‐man, who presented with purpura on the abdomen, buttocks and legs that were similar to Henoch‐Schönlein purpura (HSP). He also suffered from interstinal pneumonia. His renal biopsy specimens showed glomerulosclerosis and the peripheral pattern anti‐neutrophil cytoplasmic antibody (P‐ANCA) was positive. We reviewed the skin eruptions that had been reported with MPA, including our cases.

A Case of Cytophagic Histiocytic Panniculitis with Sicca Symptoms and Lupus Nephritis

A 48‐year‐old Japanese woman presented with many subcutaneous nodules. The skin was purplish in color and tender; the nodules were scattered over the entire surface. Histological findings of biopsy specimens from the nodules indicated septal panniculitis comprised of histiocyte and/or macrophage infiltrates, often with erythro‐ and/or leukophagocytosis. Phagocytic cells were OKM1 (CD11b), MT1 (CD43), LeuM3 (CD14), and histiocyte antigen positive, indicating the presence of histiocytes and/or macrophages. The patient had sicca symptoms, positive homogenous, speckled pattern ANA (×320), and diffuse proliferative lupus nephritis.

Erythema nodosum associated with Yersinia enterocolitica infection.

We report a 74‐year‐old woman who presented to hospital with fever, vomiting, diarrhea, and 2 weeks later developed erythema nodosum (EN) on the legs, and was diagnosed with Yersinia enterocolitica infection based on her clinical course and microbiological examination of the stool. She also had a complication of pancreatitis, which made the diagnosis challenging. We should suspect infection by Y. enterocolitica when diagnosing cases of EN with gastrointestinal symptoms. We assume EN is likely to appear 2 weeks after the onset of gastrointestinal symptoms from our case and other case reports.

Human papillomavirus-associated plantar epidermoid cysts: MR and US imaging appearance

Epidermoid cysts are epithelial cysts that present as slow-growing intradermal or subcutaneous lesions. While recent epidemiological studies have isolated human papillomavirus (HPV) from plantar epidermoid cysts, imaging findings in HPV-associated epidermoid cysts have not been previously reported. We describe imaging findings in two patients with HPV-associated plantar epidermoid cysts. Magnetic resonance (MR) imaging and ultrasonography (US) showed linear arrangement of several adjacent globular cysts. This appearance is hypothesized to result from HPV-associated eccrine duct metaplasia leading to cyst formation and later traumatic rupture leading to formation of multiple adjacent cystic components. It may be useful to suggest assessing the presence of HPV antigen in plantar lesions having these imaging findings.