Soo-Chan Kim

Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia.

Paraneoplastic pemphigus is a newly recognized disease that occurs in some patients with lymphoproliferative neoplasms and occasionally, solid tumors. Patients present with an acute illness of the mucosa and skin that shares clinical and histologic features with erythema multiforme, toxic epidermal necrolysis, and pemphigus vulgaris. These patients have antibodies against a complex of epithelial proteins that are present in desmosomes and hemidesmosomes. The course is usually fatal, except in some patients who undergo total resection of their neoplasm.

Epidermolysis bullosa acquisita: a retrospective clinical analysis of 30 cases.

Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune blistering disorder caused by autoantibody production against type VII collagen. The aim of this study was to examine the clinical types, treatments, and outcomes of 30 patients with EBA. In our cohort, the median age of onset was 44.0 years, with a similar incidence for both genders (46.7% male, 53.3% female). The majority of patients had classic type (36.7%) and bullous pemphigoid (BP)-like type (46.7%) EBA. The remaining patients had mucous membrane pemphigoid-like (6.7%), Brunsting-Perry pemphigoid-like (6.7%), and linear IgA bullous dermatosis-like type (3.3%) EBA. All patients were treated initially with a combination of methylprednisolone, dapsone and colchicine. No significant differences in time to remission were identified between patients with classic vs. BP-like EBA. In a second subset analysis of 19 patients, a group treated with high-dose (> 8 mg) methylprednisolone achieved remission earlier (median time to remission: 3 months) than a group treated with low-dose (≤ 8 mg) methylprednisolone (median time to remission: 12 months), irrespective of clinical type (p = 0.003).

Sensitive and specific assays for routine serological diagnosis of epidermolysis bullosa acquisita

BACKGROUND Epidermolysis bullosa acquisita (EBA) is a severe autoimmune subepidermal blistering disease characterized by autoantibodies against the N-terminal collagenous domain (NC1) of type VII collagen (Col VII). OBJECTIVE Development of reliable assays for the detection of anti-Col VII-NC1 antibodies. METHODS NC1 was expressed in human HEK293 cells and used as target antigen in an enzyme-linked immunosorbent assay (ELISA) and in an immunofluorescence assay (IFA). These two assays were probed in a large cohort of patients with EBA (n = 73), bullous pemphigoid (BP, n = 72), anti-p200 pemphigoid (n = 24), anti-laminin 332 mucous membrane pemphigoid (MMP, n = 15), pemphigus vulgaris (PV, n = 24), and healthy control subjects (n = 254). RESULTS The cut-off for the ELISA was optimized for accuracy by receiver-operating characteristics (area under the curve [AUC] = 0.9952). IgG reactivity against NC1 was detected in 69 of 73 EBA (94.5%) and 5 control sera (2 healthy controls and 3 BP patients), resulting in a specificity of 98.7%. The IFA showed a sensitivity of 91.8% and specificity of 99.8%. Reproducibility of the ELISA was demonstrated by an intra-class correlation coefficient of 0.97. IgG subclass analyses by ELISA revealed IgG1, IgG2, IgG3, and IgG4 anti-NC1 reactivity in 83.6%, 85.3%, 37.7%, and 83.6% of EBA sera, respectively. LIMITATIONS The novel assays were not evaluated prospectively and their use in monitoring serum levels during the disease course was not tested. CONCLUSION The two assays are highly specific and sensitive to diagnose EBA. Their diagnostic competence was demonstrated in a large cohort of well-characterized EBA sera.

Definitions and outcome measures for mucous membrane pemphigoid: Recommendations of an international panel of experts

Mucous membrane pemphigoid encompasses a group of autoimmune bullous diseases with a similar phenotype characterized by subepithelial blisters, erosions, and scarring of mucous membranes, skin, or both. Although knowledge about autoimmune bullous disease is increasing, there is often a lack of clear definitions of disease, outcome measures, and therapeutic end points. With clearer definitions and outcome measures, it is possible to directly compare the results and data from various studies using meta-analyses. This consensus statement provides accurate and reproducible definitions for disease extent, activity, outcome measures, end points, and therapeutic response for mucous membrane pemphigoid and proposes a disease extent score, the Mucous Membrane Pemphigoid Disease Area Index.

Objective evaluation of treatment effects on port-wine stains using L*a*b* color coordinates.

Wide variations in port‐wine stains and their responses to various therapies pose a need for the development of an objective method to evaluate the effects of treatment. Several techniques such as laser Doppler, reflectance spectrometry, and tristimulus colorimetry have been used to evaluate the color of port‐wine stains, but these techniques are limited by cost, small test size area, and other factors. Therefore, we developed a simple and cost‐effective method of evaluating treatment results on port‐wine stains using the L*a*b* color coordinate system in combination with a personal computer. For 22 patients with port‐wine stains, the slide photographs were digitized using a slide scanner. L*a*b* color differences of the normal control and port‐wine stain sites were obtained before and after treatment, and treatment effect (percent) was calculated. By calculating each color difference between the lesion and normal skin both before and after treatment, problems arising from different illuminating conditions during photography were minimized. The results were compared with the visual evaluation conducted by three experienced plastic surgeons. The treatment effects analyzed by L*a*b* color coordinate ranged from 4 to 95 percent, with a mean of 48.1 percent, whereas treatment effects evaluated by the plastic surgeons ranged from 15 to 92 percent, with a mean of 51.1 percent. The subjective clinical grades correlated well with the treatment effects obtained by the proposed color analysis system (correlation coefficient, 0.89). The maximum difference in the effect of treatment for a patient evaluated by the three clinicians was up to 60 percent, which means that visual judgment is very subjective and variable. The color analysis system proposed as a result of this study is very easy, objective, quantitative, cost‐effective, and can be useful for the evaluation of treatment effects on colored skin lesions such as port‐wine stains. (Plast. Reconstr. Surg. 108: 842, 2001.)

Hyperkeratosis of the nipple and areola simultaneously developing with cutaneous T-cell lymphoma

Hyperkeratosis of the nipple and areola is rare. Since its first description by Tauber (cited in Oberste-Lehn’) in 1923, about 20 cases have been reported in the literature.2-4 Clinically the typical lesions are characterized by verrucous thickening and brown pigmentation of the nipple and areola. The histopathologic features are acanthosis, hyperkeratosis, and papillomatosis. To our knowledge this is the first reported case of hyperkeratosis of the nipple and areola that simultaneously developed with cutaneous T-cell lymphoma.

Generalized multinucleate cell angiohistiocytoma

Multinucleate cell angiohistiocytoma is a rare benign fibrohistiocytic vascular condition that mainly affects middle-aged women. Grouped reddish-brown papules are found in the extremities. We describe a case of multinucleate cell angiohistiocytoma occurring in a 24-year-old man who presented with multiple papules on the trunk and extremities. This is the first report of generalized multinucleate cell angiohistiocytoma.

Nevus comedonicus associated with epidermal nevus

A 21-year-old man had a zosteriform nevus comedonicus associated with a surrounding hyperpigmented maculopapular eruption, which had the histologic appearance of epidermal nevus. Scanning electron microscopic examinations of the comedones and the surrounding epidermal nevus showed a common feature, that is, chrysanthemum-like keratotic plugs. The association of a nevus comedonicus with an epidermal nevus and the common scanning electron microscopic findings of both suggest that a nevus comedonicus probably is an uncommon form of epidermal nevus.

Serum levels of anti-type VII collagen antibodies detected by enzyme-linked immunosorbent assay in patients with epidermolysis bullosa acquisita are correlated with the severity of skin lesions.

Background  Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal bullous disease characterized by circulating autoantibodies against type VII collagen. Detecting these autoantibodies is crucial for the diagnosis of this disease, and is also useful for measuring disease activity. Enzyme‐linked immunosorbent assay (ELISA), a quantitative method to measure anti‐type VII collagen antibody levels, is currently available to diagnose EBA.

Perforating plate-like osteoma cutis in a man with solitary morphoea profunda

Summary Solitary morphoea profunda is an unusual form of scleroderma. characterized by marked fibrosis, hyalinization of collagen fibres, and inflammatory cell infiltration in the deep dermal and subcutaneous layers. We describe a 58‐year‐old man showing solitary morphoea profunda. Plate‐like osteoma cutis, with transepidermal elimination of bony material. within the morphoea profunda, was present.