Cutanenous lupus mucinosis is a rare variant of lupus erythematosus eruptions. A 36-year-old female presented with a 1-year history of 1-2cm sized, skin colored, multiple nodules on the back. She had previously been diagnosed as having systemic lupus erythematosus 10 years ago. A complete blood count showed lymphopenia. Antinuclear antibodies (ANA) were positive at a titer of 1: 640. Anti-Sm and ds-DNA were also positive. Histopathological findings showed that the collagen fibers in the upper dermis were loosely arranged and separated by amorphous materials. These materials were all positive for alcian blue staining. This case is compatible with cutaneous lupus mucinosis.
Adult ; Alcian Blue ; Antibodies, Antinuclear ; Blood Cell Count ; Collagen ; Dermis ; DNA ; Female ; Humans ; Lupus Erythematosus, Systemic* ; Lymphopenia ; Mucinoses* ; Skin

Transdermal drug delivery systems (TDDSs) overcome the hurdle of an intact skin barrier by penetrating the skin to allow molecules through. These systems reduce side effects associated with conventional hypodermic needles. Here, we introduce novel microneedle (MN) TDDSs that enhance drug delivery by creating micron-sized pores across the skin. Many MN TDDSs designed to deliver a diverse array of therapeutics, including allergen-specific immunotherapy, skin disease treatments, and vaccines, are under pre-clinical and clinical trials. Although epicutaneous approaches are emerging as new options for treating food allergy in many clinical trials, MN TDDSs could provide a more efficient and convenient route to deliver macromolecules. Furthermore, MN TDDSs may allow for safe vaccine delivery without permanent scars. MN TDDSs are a major emerging strategy for delivering novel vaccines and treatments for diseases, including skin diseases, allergic diseases, and so on.

A 14-year-old girl had a history of Graves' disease and received propylthiouracil (PTU) treatment for 2 years before admission to our hospital. She developed skin rash over her, whole body 1 month before admission, and laboratory examination revealed pancytopenia. Positive antinuclear antibodies, antihistone antibodies and reduced complement levels were noted, although anti-double-stranded DNA and anti-Sm antibodies were negative. A biopsy specimen revealed mild epidermal atrophy and peri-adnexal lymphoid cell infiltration. Linear deposition of IgM along the basement membrane zone was noted by direct immunofluorescence array. After discontinuation of PTU and steroid treatment, eruptions and pancytopenia improved, suggesting drug-induced lupus erythematosus.
Adolescent ; Antibodies ; Antibodies, Antinuclear ; Atrophy ; Basement Membrane ; Biopsy ; Complement System Proteins ; DNA ; Exanthema ; Female ; Fluorescent Antibody Technique, Direct ; Graves Disease ; Humans ; Immunoglobulin M ; Lymphocytes ; Pancytopenia ; Propylthiouracil

No abstract available.
Colitis, Ulcerative* ; Ulcer*

BACKGROUND: Although surgical excision is the standard method for the treatment of venous malformations, this procedure often leads to massive bleeding and cosmetic problems. Sclerotherapy for venous malformations has recently been reported, whereas sclerotherapy for varicose veins, leg telangiectasias and hemorrhoids has well been established. PBJECTIVE: To assess the usefulness of sclerotherapy for venous malformations. METHOD: Fourteen patients who had venous malformation were treated with sclerotherapy using sodium tetradecyl sulfate (Thromboject(R), Omega Laboratories, Ltd., Montreal, Canada). Clinical efficacy was evaluated by physical examination and comparison of photographs. RESULTS: In patients with venous malformations, 71.4% of the eases showed moderate to marked improvement. Side effects were noted in 6 patients, however, they were trivial and transient and no treatment was needed. CONCLUSION: Sclerotherapy can be recommended as an effective method for the treatment of venous malformations. It has milder and fewer side effects than other treatment modalities, and it also yields superior cosmetic results.
Hemorrhage ; Hemorrhoids ; Humans ; Leg ; Physical Examination ; Sclerotherapy* ; Sodium Tetradecyl Sulfate ; Telangiectasis ; Varicose Veins

Acrodermatitis continua of Hallopeau (ACH) is a rare form of acropustular eruption characterized by a presence of aseptic pustules on inflammatory periungual or subungual regions. Frequently accompanied by paronychia, atrophic skin changes, onychodystrophy, and osteolysis of distal phalanges of the digits, it is considered to be a variant of pustular psoriasis with a chronic relapsing course and refractoriness to many therapeutic modalities. Here, we present a case of a 45-year-old female who presented with multiple pustules pathologically diagnosed as pustular psoriasis on her left thumb. She suffered from ACH for over a decade, and in the process experienced frequent relapses and showed poor response to numerous treatment modalities such as narrow band UVB, topical steroid, steroid intralesional injection, oral retinoids, 308 nm excimer laser, and oral immune suppressants. However, the patient showed dramatic clinical improvements to administration of etanercept (TNF-alpha antagonist, twice a week) for a period of one month. The cessation of etanercept led to recurrence of symptoms and marked deterioration of the skin lesion within a month again, but the re-initiation of treatment soon relieved the problem. After completion of a three months trial of etanercept, the cutaneous lesion subsided, and the patient is now successfully controlled with topical steroid maintenance therapy. Hereby, we report a patient with ACH successfully treated with etanercept.
Acrodermatitis ; Female ; Humans ; Immunoglobulin G ; Injections, Intralesional ; Lasers, Excimer ; Middle Aged ; Osteolysis ; Paronychia ; Psoriasis ; Receptors, Tumor Necrosis Factor ; Recurrence ; Retinoids ; Skin ; Thumb ; Etanercept

Acrokeratoelastoidosis (AKE) is a rare skin disorder initially described by Costa, which is inherited by autosomal dominant, but also may be sporadic. Clinically, it consists of small, firm papules with occasional keratosis or umbilication, characteristically along the margins of hands and feet. Histopathologically, it shows hyperkeratosis, acanthosis in the epidermis and fragmentation and rarefaction of elastic fibers-elastorrhexis-in the dermis. A 32-year-old man presented with multiple papules along the border of the hands and feet, and from histopathology, AKE was diagnosed. Herein we report a case of acrokeratoelastoidosis and review the clinical and histopathologic features, etiology, differential diagnosis and treatment.
Adult ; Dermis ; Diagnosis, Differential ; Epidermis ; Foot ; Hand ; Humans ; Keratosis ; Skin

BACKGROUND: For the differential diagnosis between the various subtypes of muscular dystrophy, the analysis of the protein expression pattern from the biopsied skeletal muscle tissue is essential. Authors performed the immunohistochemical study (IHC) using sets of antibodies for the differentiation of subtypes of muscular dystrophy. METHODS: Antibodies against dystrophin C-terminal, dystrophin rod domain, dystrophin N-terminal, alpha-, beta-, gamma-sarcoglycans, laminin alpha2 chain, dysferlin, and beta-dystroglycan were used for the IHC study in 43 patients with muscular dystrophy. The reactivity against the specific antibodies was graded and the clinical findings were assessed. RESULTS: We found 15 cases of dystrophin deficiency and 7 cases of dysferlin deficiency. Those with dystrophin deficiency were clinically classified previously as follows, 11 cases with Duchenne's muscular dystrophy (DMD), two with congenital muscular dystrophy (CMD), one with Becker's muscular dystrophy (BMD), and a female patient with limb-girdle muscular dystrophy (LGMD). Those with dysferlin deficiency consisted of 4 cases with LGMD phenotype and 3 with distal myopathy. CONCLUSIONS: The results of our study confirm the dystrophin immunostain is essential for the identification of dystrophinopathies among the various subtypes of muscular dystrophy. Also, the identification of 7 cases with dysferlin deficiency suggests dysferlinopathy is the common cause of muscular dystrophy in Korea.
Antibodies ; Diagnosis, Differential ; Distal Myopathies ; Dystroglycans ; Dystrophin ; Female ; Humans ; Immunohistochemistry ; Korea ; Laminin ; Muscle, Skeletal ; Muscular Dystrophies* ; Muscular Dystrophies, Limb-Girdle ; Muscular Dystrophy, Duchenne ; Phenotype ; Sarcoglycans

In most cases, acute diarrhea in childhood heals spontaneously, but it may become the form of chronic diarrhea in immunodeficient children and then cause weight loss, dehydration, malabsorption and malnutrition. The immunodeficient diseases associated with chronic diarrhea include severe combined immunodeficiency syndrome, common variable immunodeficiency, acquired immunodeficiency syndrome, agammaglobulinemia or selective IgA deficiency. IgA deficiency is the most common primary immunodeficiency. Because many IgA deficient individuals seem to have compensated for their deficiency with increased IgM production and various nonimmunologic factors, the incidence of gastrointestinal involvement is not prominent. Some of those with IgA deficiency and recurrent infections have been found to also have IgG subclass deficiency. IgA deficiency with IgG2 and IgG4 subclass deficiency have high susceptability to infection and chronic diarrhea. IgG subclass deficiency, when present, is more likely to be found in association with a partial IgA deficiency rather than complete IgA deficiency. We report a 3-month-old male with intractable diarrhea accompanied by IgA, IgG2, and IgG4 deficiency.
Acquired Immunodeficiency Syndrome ; Agammaglobulinemia ; Child ; Common Variable Immunodeficiency ; Dehydration ; Diarrhea* ; Humans ; IgA Deficiency ; Immunoglobulin A* ; Immunoglobulin G* ; Immunoglobulin M ; Incidence ; Infant ; Male ; Malnutrition ; Severe Combined Immunodeficiency ; Weight Loss