Yoshiro Ichiki

A case of dermatomyositis that developed after delivery: the involvement of pregnancy in the induction of dermatomyositis

A relationship between dermatomyositis (DM) and pregnancy has rarely been documented, and most cases have been reported from the viewpoint of the management of high‐risk pregnancy. We report a patient with DM which developed after the delivery of a healthy infant. This case, with support from a literature review, suggests that pregnancy could be a trigger for the development of DM. Furthermore, it is suggested that there are at least two types of pregnancy related DM: in one type, the disease activity is provoked during pregnancy and tends to improve after delivery, while the other type (including the present case) has onset in the postpartum period.

Skin infection caused by Mycobacterium avium.

A patient with skin infection due to Mycobacterium avium is reported. A 9–year–old female had 10 subcutaneous nodules and two ulcers on the abdomen and legs. She had no medical history of systemic disease, skin disease or immunosuppressive therapy. Cultures of a biopsy specimen and of aspirated seropurulent fluid in nodules showed acid–fast bacteria, identified as M. avium by the DNA–DNA hybridization method. We treated her with a combination of surgery and the antibiotics, cycloserine, isoniazid and clarithromycin.

An outbreak of eruptive pseudoangiomatosis-like lesions due to mosquito bites: erythema punctatum Higuchi.

Nonitchy small spotted erythema surrounded by an anemic halo (fig. 1) has been called erythema punctatum Higuchi in Japan for about the past 50 years. The clinical features of the eruptions are described as follows [1–6]. Females are more frequently affected than males. Most of the eruptions develop scatteringly on the extremities, especially in summer. Indurations are associated with erythema and halos at the onset, and vanish in about 3 days. Halos become unclear after 4 or 5 days. Both the erythema and the halo disappear 7–19 days after the onset. On the face, lesions have an unclear halo. The etiology is not well known. Histological studies [3, 4, 6] revealed capillary dilatation and inflammatory cell infiltration in the upper dermis, which are not specific features. Ohara et al. [5] reported 24 cases and suggested that mosquito bites play a significant role in the pathogenesis of erythema punctatum Higuchi. They also observed that the eruption appeared in the skin of a 30-year-old female patient 5 min after the bite of Culex pipiens pallens and saw 3 cases induced by other kinds of mosquitoes. However, to date there have been no other reports about the relationship between mosquitoes and erythema punctatum Higuchi. Since 1990, we have observed several outpatients with similar eruptions in Hashima City Hospital, which is located in a garden city in the Nobi plains near the center of Japan, during every summer. The eruptions do not react to corticosteroid ointments and fade out within 1–3 weeks with or without therapy. Though we had hardly seen the eruptions in inpatients of the hospital, we have recently experienced many inpatients with these eruptions. A great number of C. pipiens pallens attacked Hashima City Hospital in the late autumn of 2001 and flew into the hospital especially at night, though no mosquitoes usually appear in Hashima City in that season. C. pipiens pallens worried the inpatients and the staff of the hospital until the end of December. C. pipiens pallens is common in Japan. When bitten by them, people usually feel an itch. We suspected that the propagation of C. pipiens pallens was caused by drainage works that had been carried out near the hospital in 2001. The mosquitoes breed in the stagnant ditch. C. pipiens pallens passed the winter and appeared again in Hashima City Hospital in the early spring of 2002. While a great number of C. pipiens pallens attacked the hospital, we found many inpatients with erythema punctatum Higuchi: 10 inpatients in the late autumn in 2001, and 16 inpatients in the early spring of 2002 (table 1). They had multiple lesions on the face, neck, hands and forearms. These were not itchy. Most of the patients except for psychiatric inpatients were bedridden. Five inpatients on the same floor developed the eruptions within 10 days. Two of them had stayed in the same room and were found to develop the eruptions on the same day. Some confusion arose on that floor, because most doctors and nurses had no knowledge of erythema punctatum Higu-

Pitting Scars in Progressive Systemic Sclerosis

The digital pitting scar is a common clinical feature in patients with progressive systemic sclerosis (PSS). Its pathogenesis is unclear, but it may result in small ulcerations. The clinical and histological features of these lesions are poorly understood. Eighty-seven patients with PSS were examined at least once per year. Pitting scars were defined as pinhole-sized digital concave depressions with hyperkeratosis. They were seen in 34 cases (39%) and were located not only on the tips of the fingers, but also on the sides, especially on the radial border of the index and middle finger and the ulnar side of the thumb, where they had a linear arrangement. Additional pitting scars were noticed on the dorsal surface of the proximal interphalangeal and metacarpophalangeal joints. Pitting was closely associated with several signs of PSS, e.g. Raynauds phenomenon, skin thickening or articular involvement (e.g. stiffness, swelling, pain). There was no relationship between the number of pitting scars and the duration of PSS. Biopsy samples were taken of the lesions on the lateral sides of fingers in 3 patients. Histologically, there was a plug-like hyperkeratosis with parakeratosis, homogenized collagen fibers and slight perivascular mononuclear cell infiltration. Calcification and altered collagen fibers were observed in the deep dermis. Although pitting scars of the fingertips may be different from those of the lateral sides of the fingers, both histologically and pathogenetically, they were similar clinically. The scar locations on the fingertips and finger joints (proximal interphalangeal and metacarpophalangeal) suggest that exogenous trauma, vibration injury and/or cold exposure may play a role in their pathogenesis.

Ulcerative Sarcoidosis: Case Report and Review of the Japanese Literature

,Sarcoidosis is a chronic multi-system disease of unknown origin involving the eyes, skin, lungs, heart, bones and nerves. Skin lesions are relatively common, and are clas-sified as either specific or non-specific (1). Specific le-sions are those that show the typical sarcoid granulomas, including lupus pernio, infiltrated plaques, maculopapular eruptions, subcutaneous nodules and infiltration of old scars (1). The most typical non-specific skin lesion is erythema nodosum. The patient described here had skin lesions that first suggested a diagnosis of erythema indu-ratum of Bazin, but we ultimately diagnosed sarcoidosis based on other findings, including uveitis, bilateral hilar lymphadenopathy, an increased level of serum angio-tensin-converting enzymes (ACE) and a negative response to purified protein derivative of tuberculin (PPD).CASE REPORT

Surfactant Protein D (SP-D) and Systemic Scleroderma (SSc)

We measured serum levels of SP‐D in collagen diseases (110 cases) such as systemic scleroderma (SSc), scleroderma spectrum disorders (SSD), systemic lupus erythematodes (SLE), Sjögren syndrome (Sjs), dermatomyositis (DM), rheumatoid arthritis (RA), and dermatitis (DE) (109 cases) as a control. Additionally, we performad a correlation analysis to determine how these levels were related to pulmonary fibrosis and function test (vital capacity, %DLco). The serum levels of SP‐D increased in SSc patients with Barnett type III more than in SSc patients with Barnett type I or II, while they increased slightly in SSD (incomplete type of SSc) patients. The differences in these figures were statistically significant between the SSc (SSc & SSD) and non‐SSc (SLE, DM, Sjs & RA) groups (p<0.005). The serum levels of SP‐D in SSc patients with anti‐topoisomerase I antibodies were statistically higher than those in SSc patients with other types of anti‐nuclear antibodies. There was a statistically significant correlation between the severity of pulmonary fibrosis and the serum levels of SP‐D, and a statistically negative correlation between SP‐D levels and vital capacity or %DLco, but there was no proportional correlation with the forced expiatory volume (FEV1.0%). There was no statistical relationship between pre‐ and post‐therapy with photopheresis; however, there was a statistical correlation between the serum levels of SP‐D and KL‐6. In the group of collagen diseases, plasma levels of SP‐D were higher than serum levels of SP‐D. Patients with SSc possess higher levels of SP‐D than do those with other collagen diseases and dermatitis, which may correspond to the severity of pulmonary fibrosis.

A Novel Nonsense Mutation at E106 of the 2B Rod Domain of Keratin 14 Causes Dominant Epidermolysis Bullosa Simplex

Epidermolysis bullosa simplex (EBS) is classified into three main types and is caused, in most cases, by missense mutations in the genes encoding keratin (K) 5 and K14. In this study, we clinically, ultrastructurally, immunohistochemically, and molecularly studied a patient with a dominant EBS, Köbner type. Using sequence analysis of genomic DNA, a novel K14 nonsense mutation was identified. A heterozygous mutation G1231T of KRT14 was found to be associated with the disease in the patient. The mutation created a premature stop codon (amino acid codon 411, residue 106 of the 2B helix) in the K14 molecule. This residue lies in a highly conserved region and was recently found to be absolutely required for molecular stability and intermediate filament assembly in K5 and K14. The E411X (E106X) heterozygous ablation, missing the last 16 amino acid residues of the 2B and the entire tail domain of K14, led to disease but did not result in clumping of keratin filaments. It is the first premature stop codon mutation of K14 found in dominant EBS.

Aberrant expression of p16 and RB protein in eccrine porocarcinoma

Background:  Eccrine porocarcinoma is a rare malignant tumor of the sweat gland and very little is known about its etiology and molecular basis.

PREVALENCE OF RAYNAUD'S PHENOMENON AND SPECIFIC CLINICAL SIGNS RELATED TO PROGRESSIVE SYSTEMIC SCLEROSIS IN THE GENERAL POPULATION OF JAPAN

Background. In order to evaluate the prevalence rates of Raynauds phenomenon (RP) and specific clinical signs related to progressive systemic sclerosis (PSS) in the general population of Japan, inquiries were made concerning RP in the hands and dermatologic examinations were also conducted.

A trial of acupuncture for progressive systemic sclerosis.

Thirty four cases of PSS were treated once or twice a week by a low frequency electrical acupuncture method for periods of 3 months to 9 years and 2 months. On clinically worse side of arm, two meredian points, Hatija and Shokai , were used for the electrical stimulation. As the result, various degrees of improvement were obtained in ulcerations on the fingertips or toetips, arthralgia, cutaneous sclerosis, hypo‐ and hyperpigmentation and sensory disturbance etc. and in respiratory functions, plethysmography, capillary microscopic features of the nail bed and histopathologic features biopsied from the forearms. Laboratory data showed remarkable improvement in the inflammatory reactions. However, the improvement in immune reactions was poor paralleling the poorness of clinical improvement and prognosis.