Michiyuki Koga

Changes in Plasma Serotonin Concentration and Acceleration Plethysmograms in Patients with Raynaud's Phenomenon after Long‐term Treatment with a 5‐HT2 Receptor Antagonist

High blood serotonin concentrations have been reported in patients with Raynauds phenomenon and a relationship has been suggested. Because of the difficulty in evaluating Raynauds phenomenon objectively, a possible correlation between blood serotonin concentrations and clinical findings has not yet been evaluated. We measured plasma serotonin concentrations and acceleration plethysmograms (APG) before and one year after administration of a serotonin receptor antagonist. Twenty‐seven patients with either collagen disease or diseases associated with Raynauds phenomenon were given a combined 5‐HT2 and serotonin receptor antagonist, sarpogrelate hydrochloride. Plasma serotonin concentrations were determined before and after administration and the APG d/a value was measured as an index of peripheral hemodynamics. These values were compared with the clinical symptoms of the patients. After one year of treatment, the subjective symptoms improved in 59.3% of patients who has Raynauds phenomenon. The pre‐treatment plasma serotonin concentrations of the study patients were significantly higher than those of the normal controls, but became significantly decreased following 5‐HT2 administration. However, there was no clear‐cut relationship with the clinical symptoms. The pre‐treatment APG d/a value of the patients was significantly lower than that of the normal controls, although there was no significant difference after administration when analyzed as an entire group or in a subset of patients whose symptoms did not subjectively improve or worsened after one year of treatment. In the subset in whom the subjective symptoms improved, however, the value significantly increased following administration, suggesting an improvement in peripheral hemodynamics. These results suggest the possibility that APG can be used as an objective index of peripheral hemodynamics.

The occurrence of various collagen diseases in one family: a sister with ISSc, PBC, APS, and SS and a brother with systemic lupus erythematosus.

We encountered siblings who had collagen diseases and related symptoms. Case 1 was a 53‐year‐old woman who had limited cutaneous systemic sclerosis (ISSc) associated with primary biliary cirrhosis (PBC), antiphospholipid antibody syndrome (APS), and subclinical Sjögrens syndrome (SS). Case 2 was a 48‐year‐old man, her younger brother, with systemic lupus erythematosus (SLE) that developed at 32 years of age. Investigation of their family revealed that their mother had Raynauds phenomenon, arthritis, and subclinical Sjögrens syndrome, and that another younger brother of Cases 1 and 2 had Raynauds phenomenon and general fatigue. HLA analysis revealed that the sister and brother had some identical HLA antigens in common, including A2, A33 (19), B67, B44 (12), Cw7, DR2, DR6, DR52, and DQ1. The sister, brother and their mother had common HLA antigens including A2, B67, Cw7, DR2, and DQ1. Although Cases 1 and 2 shared the same HLA system, they presented different phenotypes of collagen disease.

A Case of Eruptive Collagenoma Localized on the Neck and Shoulders

A 78‐year‐old woman, who had first noticed asymptomatic eruptions on her neck and shoulders eight years earlier, presented with papules and nodules 2 to 20 mm in diameter that had a normal to white hue and were flatly elevated. These lesions were scattered and multiple, some forming confluent plaques. Histopathologically, the epidermis was slightly atrophied, and collagen fibers in the dermis were coarse and proliferated. In addition, the number of elastic fibers was slightly decreased. No complications were evident. Based on these findings, the patient was given a diagnosis of mild eruptive collagenoma, a type of connective tissue nevus according to the classification of Uitto. This case is unique in that onset was at an advanced age and that distribution was localized on the neck and shoulders.

Contact anaphylaxis due to para-aminophenol and para-methylaminophenol in hair dye

Case Report A 61-year-old man was being treated by his primary physician for eczema of the hands. The patient had been using a commercial hair dye about 1¿ a month for the previous 20 years. One day in May 2000, after using the hair dye, he began to develop upper airway stridor and generalized urticaria. The following month, while using the same hair dye (about 30–40 min after starting), the patient had dyspnea and generalized urticaria. He was evaluated in an emergency room, where the physician suspected anaphylaxis due to the hair dye. Open tests and prick tests with the hair dye (1% aq.) and its ingredients (para-phenylenediamine, paraaminophenol, ortho-aminophenol, para-methylaminophenol and sodium methyl oleoyl taurate) were all negative at 1%, 5% and 10% aq. Scratch tests with para-aminophenol (1%, 5% and 10% aq.) and para-methylaminophenol (1%, 5% and 10% aq.) were positive, using histamine dihydrochloride (1% aq.) as positive control and isotonic water as negative control. 10 control subjects also scratch tested with para-aminophenol and para-methylaminophenol were all negative. The patient was diagnosed as having a Type I allergy.

The relationship between angioblastoma (Nakagawa) and tufted angioma: report of four cases with angioblastoma and a literature-based comparison of the two conditions.

We report four recent cases of angioblastoma (Nakagawa). Histopathologic examinations of all cases revealed dispersed islets of clear marginal lobules of varying sizes in the dermis. Neoplastic endothelioid cells with moderate atypia and enlarged capillaries containing erythrocytes were found in the conglomerates. Recently, the features of this disease have been compared to the tufted angioma that has been reported in Europe and the U.S. Our evaluation suggests that these two diseases are very likely the same. We suggest that this disease should be called “angioblastoma” in agreement with the first report of this disease by Nakagawa.

Clinical course of 44 cases of localized type vitiligo.

Vitiligo is often classified into three types, generalized, segmental, and localized, on the basis of their distribution pattern. It is also classified into type A (non‐dermatomal or non‐segmental) and type B (dermatomal or segmental) vitiligo on the basis of both the distribution pattern and physiological function ( 1 ). The natural courses of type A and type B vitiligo are characteristic and quite different from each other. Whereas type A vitiligo appears at any age and progresses throughout the patients life span, type B vitiligo affects the young and stabilizes within a few years ( 2 ). Segmental type vitiligo corresponds to type B, and generalized type vitiligo is the late stage of type A. However, no one has observed the course and character of localized type vitiligo, and its nosological position in A/B classification is unclear. We followed 44 cases of localized type vitiligo for periods of 6 months to 8 years. In 3 of the 44 patients, new white patches developed within the same dermatome as their affected areas in the first 12 months and stabilized in a short period. Therefore, these patients were diagnosed as type B vitiligo. In 15 patients, vitiligo developed in other dermatomal areas; the earliest case at 9 months, and others later on. The new white patches continue to develop for a long period, so these patients were diagnosed with type A vitiligo. In 26 of the 44 patients, the vitiligo remained localized within the period of observation. It is concluded that most localized type vitiligo is the early stage of type A, but a small number of cases belong to the early stage of type B.

Staphylococcal scalded skin syndrome in a healthy adult.

We report a case of staphylococcal scalded skin syndrome (SSSS) in a 65‐year‐old healthy woman. Fever, purulent conjunctivitis, and exfoliation of the skin in the gluteal region were noted. A scarlatiniform rash was observed on the body, and this erythema was followed by generalized desquamation. Staphylococcus aureus was isolated from her eye discharge, posterior nasopharynx, and the erosive surface of the skin. All the investigated strains produced exfoliative toxin B, but none produced toxic shock toxin‐1 (TSST‐1) or enterotoxin. The patient was treated with antibiotics and fluid supplementation, resulting in subsidence. This case is thought to have been caused by an abortive form of SSSS or a scarlatiniform variant, which is very rare in healthy adults.

A Case of Dermatomyositis Associated with Mechanic's Hand

A 67‐year‐old man was referred to the Department of Internal Medicine at Tokyo Medical University with interstitial pneumonia in July 1999. He presented with keratotic plaques on both palms and on the ventral and lateral sides of his fingers. Erythematous keratosis was observed on the dosal aspect of his fingers and metatarsophalangeal (MP) joints. Edematous erythema was seen on the patients chest, back, and the extensor surfaces of his arms. Electromyography revealed a myogenic pattern and an increased level of myogenic enzymes was found in the blood. Histological findings of the ventral sides of his fingers showed hyperkeratosis and parakeratosis of the dermal tissue and liquefaction degeneration of the basal layer at the papilla. Based on these findings, the patient was given a diagnosis of dermatomyositis associated with mechanics hand. A systemic examination confirmed interstitial pneumonia and carcinoma of the duodenal papilla. Mechanics hand is a type of dermatitis associated with myopathy first reported by Stahl et al. in patients with collagen disease. We report herein the first documented case of mechanics hand in Asians.

Food-dependent exercise-induced anaphylaxis with a high level of plasma noradrenaline.

Ingesting certain foods sometimes triggers anaphylaxis when followed by exercise (food‐dependent exercise‐induced anaphylaxis, FDEIA). Specific food‐induced mucocutaneous urticaria may also progress to anaphylaxis (oral allergy syndrome, OAS). A positive skin test and/or radioallergosorbent test (RAST) to the foods suggest involvement of immunoglobulin (Ig)E‐anaphylaxis in both disorders. The triggering foods and initial target organs are usually different in each case. In the present study, a 32‐year‐old male reported dyspnea accompanied by wheals, and symptoms of low blood pressure while walking after eating Chinese noodles and donuts. He also reported uncomfortable sensations in his mouth and throat after ingesting melon. Exercise challenge tests were administered. Serum histamine, plasma adrenaline, noradrenaline and dopamine were measured pre‐ and post‐test. No symptoms were induced by exercise or by the ingestion of any single food item before exercise. However, numerous wheals appeared when exercise followed the combined ingestion of foods. Likewise, the sequence of eating pancakes and then exercising resulted in numerous wheals and anaphylaxis. Olopatadine hydrochloride and ketotifen fumarate completely inhibited this anaphylaxis. The skin prick tests resulted in fruit‐induced erythema and wheals. The results of these tests with wheat, butter and sugar were negative, and no symptoms were induced by the exercise test after ingestion of watermelon, melon or apple. The anaphylactoid symptoms were accompanied by a significant increase of plasma noradrenaline. In this case, not only wheat, but sugar and butter may induce the onset of FDEIA. There was no significant correlation between the intensity of the symptoms and the serum histamine levels in the present case. Noradrenaline may be involved in the onset of FDEIA, since noradrenaline may selectively inhibit T‐helper (Th)1 functions while favoring Th2 responses. The tests showed no cross‐reactivity between the causative foods of OAS and FDEIA, indicating that the mechanisms of onset are different between them.

Perforating Pilomatricoma: Transepithelial Elimination or Not

We present a 56‐year‐old woman with a perforating pilomatricoma in the left eyebrow region. Histologically, the tumor consisted mainly of basophilic cells and shadow cells, and the tumor components were being eliminated through an ulcer with damage to the epithelial structures. In past reports of perforating pilomatricoma, this elimination pattern has often been described as transepithelial elimination. In many patients with perforating pilomatricoma, elimination is accompanied by ulceration and epithelial damage. Mehregan recently stated that elimination accompanied by epidermal necrosis and superficial ulceration constituted one form of transepithelial elimination. Epidermal necrosis and ulceration generally constitute severe damage. However, when Mehregan first proposed the concept of transepithelial elimination, it was defined as a phenomenon with relatively little or no damage to the epithelial structures, differentiating it from other types of elimination. This original definition makes transepithelial elimination a unique and interesting phenomenon, and its most important feature is that there is relatively little or no damage to the epithelial structures. Therefore, the terms “epidermal necrosis” and “ulceration” should not be used in association with transepithelial elimination. Hence, in patients with perforating pilomatricoma, the elimination of tumor components from ulcers with damage to the epithelial structures, as seen in the present case, should not be described as transepithelial elimination.