Chikako Kishi

Nephrogenic Systemic Fibrosis with Multiple Calcification and Osseous Metaplasia

We describe here a 50-year-old Japanese man with nephrogenic systemic fibrosis. He had been suffering from chronic renal insufficiency and had been treated with haemodialysis. He had undergone magnetic resonance angiography using gadodiamide 7 years previously. One month after magnetic resonance angiography, he noted swelling, hotness, induration and pain in his left arm. The same symptoms gradually spread over his lower extremities, resulting in flexion contractures with limited range of motion. Physical examination revealed skin sclerosis on his extremities with a glossy brownish skin surface. Histologically, increased collagen fibres with high cellularity were seen in the dermis and subcutaneous septa. Thickened fascia was also noted, as well as osseous metaplasia under the fascia. Computed tomography of the whole body revealed multiple calcification of the fascia in many muscles. Treatment with intravenous sodium thiosulphate did not result in any clinical improvement.

Acquired reactive perforating collagenosis in dermatomyositis

1 Gardner FH, Diamond LK. Autoerythrocyte sensitization: a form of purpura producing painful bruising following autosensitization to red blood cells in certain women. Blood 1955; 10: 675–690. 2 Agle DP, Ratnoff OD. Purpura as a psychosomatic entity. A psychiatric study of autoerythrocyte sensitization. Arch Intern Med 1962; 109: 685–694. 3 Kirscher MH, Hofmann GO, Markewitz A et al. Osteosynthetic reconstruction in a patient with Gardner– Diamond syndrome. J Trauma 1995; 38: 392–395. 4 Ratnoff OD. Psychogenic purpura (autoerythrocyte sensitization): an unsolved dilemma. Am J Med 1989; 87: 16N–21N. 5 McDuffie FC, McGuire FC. Clinical and psychological patterns in autoerythrocyte sensitivity. Ann Intern Med 1965; 63: 255–265. 6 Panconesi E, Hautmann G. Stress, stigmatization and psychosomatic purpuras. Int Angiol 1995; 14: 130– 137. 7 Settle EC Jr. Autoerythrocyte sensitization successfully treated with antidepressants. JAMA 1983; 250: 1749– 1750. 8 Lotti T, Benci M, Sarti MG et al. Psychogenic purpura with abnormally increased tPA dependent cutaneous fibrinolytic activity. Int J Dermatol 1993; 32: 521–523. 9 Fey MF, Beck EA. Psychogenic purpura, idiopathic thrombocytopenic purpura, and platelet dysfunction in the same patient. J Clin Psychiatry 1986; 47: 386– 387. 10 Sudy E, Urbina F, Vasquez P. Autoerythrocyte sensitization syndrome with positive anticardiolipin antibodies. Br J Dermatol 1998; 138: 367–368.

Plasmodium berghei infection ameliorates atopic dermatitis‐like skin lesions in NC/Nga mice

Atopic diseases are more prevalent in industrialized countries than in developing countries. In addition, significant differences in the prevalence of allergic diseases are observed between rural and urban areas within the same country. This difference in prevalence has been attributed to what is called the ‘hygiene hypothesis’. Although parasitic infections are known to protect against allergic reactions, the mechanism is still unknown. The aim of this study was to investigate whether or not malarial infections can inhibit atopic dermatitis (AD)‐like skin lesions in a mouse model of AD.

Microsporum canis infection mimics pemphigus erythematosus.

We report a 55-year-old Japanese woman with a two-month history of multiple pruritic erythema and erosion on her face and neck. Based on the clinical appearance, we initially diagnosed her as having pemphigus erythematosus. However, the results of a histopathological examination and a direct immunofluorescence study did not support the initial diagnosis. Additionally, anti-desmoglein 1 and 3 antibodies were all negative. Subsequently, a microscopic examination of scales revealed filaments of fungi and a fungal culture was negative for macroconidium. Using molecular biology techniques, we identified the fungus as Microsporum canis, which causes a zoonotic infection. The immune reaction to the fungi could be drastic and therefore, the eruption sometimes displays atypical clinical manifestations.

Elevated serum MFG-E8 level is possibly associated with the presence of high-intensity cerebral lesions on magnetic resonance imaging in patients with systemic lupus erythematosus

Human milk fat globule‐EGF factor 8 (MFG‐E8), also known as lactadherin, is a secreted glycoprotein that plays essential roles in the clearance of apoptotic cells and angiogenesis. It has been reported that serum MFG‐E8 levels are higher in systemic lupus erythematosus (SLE) patients compared with in healthy controls; however, a previous study reported no correlation between serum MFG‐E8 levels and SLE disease activity. The objective of this study was to assess serum MFG‐E8 levels and their clinical associations in patients with SLE. Serum MFG‐E8 levels in 49 Japanese patients with SLE, eight with cutaneous LE, and 28 healthy controls were examined. Serum MFG‐E8 levels in SLE patients were significantly higher than those in cutaneous LE patients and healthy individuals. In addition, serum MFG‐E8 levels were positively correlated with the SLE Disease Activity Index score, which reflects the disease activity of SLE. Notably, the frequency of the presence of high‐intensity cerebral lesions on MRI in the SLE patients with elevated serum MFG‐E8 levels was significantly higher than that in SLE patients with normal serum MFG‐E8 levels. These findings suggest that elevated serum MFG‐E8 levels may be associated with cerebrovascular diseases or neuropsychiatric SLE in patients with SLE, and that the measurement of serum MFG‐E8 levels in SLE patients is useful for risk stratification of cerebrovascular disease or cerebrovascular disease‐related neuropsychiatric SLE.

Localized cutaneous immunoglobulin light chain kappa-positive amyloidosis associated with juvenile dermatomyositis

Dear Editor, A 19-year-old Japanese woman was referred to our department with a 17-year history of erythema on the face, neck, hand and back. When she was 2 years old, she was diagnosed with infantile dermatomyositis based on skin manifestations and muscle weakness. Systemic corticosteroid (20 mg/day) was started. Because symptoms were improved, systemic corticosteroid was discontinued at 6 years old. Although topical corticosteroid was applied for several years, her skin lesions did not improve. Physical examination revealed dark diffuse erythema in the face, posterior neck and lower back (Fig. 1a,b). Hypopigmented areas were associated with the lichenoid eruption. Periungual erythema, Gottron’s papules and Gottron’s sign were present (Fig. 1c). Muscle weakness and pain were not detected. Results of laboratory examination included normal levels of creatine kinase and C-reactive protein. KL-6, a lung fibrosis marker, was 197 U/mL (normal, <500 U/mL). Antinuclear antibody was negative. Anti-TIF1 antibody was detected. AntiRNP, anti-Sm anti-dsDNA, anti-SS-A/Ro, anti-SS-B/La, antiJo-1, anti-aminoacyl-transfer RNA synthetase autoantibodies and anti-melanoma differentiation-associated gene 5 antibody were not detected. No interstitial lung disease and internal malignancy were detected. Histopathological examination of the posterior neck showed hyperkeratosis, acanthosis and thick deposits of an eosinophilic, homogeneous substance in the papillary dermis (Fig. 1d), which were positively stained with Congo red and direct fast scarlet (Fig. 1e). Immunohistochemical staining of the amyloid deposited lesion resulted in positive staining with anti-immunoglobulin light chain kappa (Fig. 1f) and antiserum amyloid P antibodies, but negative staining with antikeratin (Fig. 1g) and anti-transthyretin antibodies. Direct immunofluorescence was negative. Multiple myeloma was excluded by normal levels of urinary Bence Jones protein and the ratio of j/k immunoglobulin free light chain (0.52; normal, 0.31–1.56), no serum M-protein, and no submucosal amyloid deposits by rectal biopsy. Diagnosis was established of secondary localized cutaneous amyloidosis associated with juvenile dermatomyositis. Localized cutaneous amyloidosis includes primary cutaneous amyloidosis, which is the absence of systemic involvement, and secondary cutaneous amyloidosis, which is accompanied by skin neoplasms and systemic diseases, such

Distinct clinical and histological features in dermatomyositis with anti-aminoacyl-tRNA synthetase antibodies.

pacitating right foot pain. She presented with bullous eruption with red halo on her nose, anterior chest and abdomen (Fig. 1a). Also, her right thigh was swelling with a band of bullous eruption into near-circumferential erythema (Fig. 1b). She was diagnosed as having disseminated herpes zoster with atypical features. Computed tomography showed no abnormality although she complained of acute pain in her right upper thigh. Clinical laboratory investigation revealed no inflammatory response. She was treated with oral valacyclovir 500 mg three times daily for 7 days. The serological study of anti-varicella zoster virus (VZV) antibody during the acute stage revealed slight elevation of immunoglobulin (Ig)M (3.02 mL; normal, <0.8 mL) and high elevation of IgG (>128.0 mL; normal, <2.0 mL). Fifteen days after treatment, she complained of soreness and numbness in her left leg. We consulted an orthopedic surgeon for advice on her complaint. The magnetic resonance imaging (MRI) found that she suffered from a ruptured disk in the lower spine (Fig. 1c,d). Her symptoms were those of lumbar disc hernia (LDH) in the left L4 and L5 dermatomes. Herein, we reported a rare case of LDH in the left L4 and L5 dermatomes after herpes zoster infection in the right L2 and L3 dermatomes. It was reported that intervertebral disc degeneration may be caused by VZV. It seemed that our patient had pre-existing spinal canal stenosis between L3 and L4, and also L4 and L5 spinal column, by using X ray and MRI of the spine. She was affected by the reactivation of latent VZV in the right L2 lesion in this course. The distance between the L2 and L3 posterior root ganglia decreased because she had spinal canal stenosis in the region innervated by nerves running from the spinal column. As a result, L2 nerves inflamed by VZV spread to the L3 nerves lesion. Therefore, the swelling on the right side of the inflamed nerves increased stress on the left side of the spine. As a result, the right upper side of the spine was overloaded with the left lower side of the spine. For these reasons, we considered that LDH in the left L4 and L5 dermatomes was caused by herpes zoster infection in the right L2 and L3 dermatomes.

Langerhans cell histiocytosis masquerading as hidradenitis suppurativa

Dear Editor, A 42-year-old Japanese man presented with a 22-year history of painful nodules and ulcers with recurrent draining sinuses on the axillary and perianal regions. He developed a pneumothorax at the age of 25 years, which was diagnosed as eosinophilic granuloma. His axillary and perianal lesions had been treated with surgical drainage of the abscesses in other hospitals. Because the lesions had enlarged, he visited our hospital. A physical examination revealed brownish indurated plaques with reddish-purplish papules, ulcers and fistulas, and active purulent drainage on the bilateral axillaries (Fig. 1a). The perianal region showed multiple ulcers and fistulas with scars due to repeated surgical drainage over the past 20 years (Fig. 1b). At that time, we clinically diagnosed these ulcers and fistulas as hidradenitis suppurativa. Bacterial culture revealed a small number of Staphylococcus aureus and Prevotella disiens. We performed debridement and split-thickness skin grafting on the axillary lesions. A pneumothorax developed on postoperative day 2, which was treated with a partial pneumonectomy. Histological examination revealed sinuses lined with epidermis in the dermis (Fig. 1d) and a dense infiltration of histiocytic mononuclear cells and multinucleated giant cells from the dermis to subcutaneous tissue (Fig. 1e). The histiocytic cell populations expressed CD1a (Fig. 1f) and S-100 proteins (Fig. 1g). The hypermetabolic lesions of F-fluorodeoxyglucose positron emission tomography/computed tomography were confirmed in the soft tissue from the right neck to the back, thyroid (Fig. 1h), perianal region and adductor magnus muscle. The histological findings of the resected lung tissue and needle biopsies from the thyroid and soft tissue of the neck were compatible with those of Langerhans cell histiocytosis (LCH). The results of a plain-film bone survey and bone marrow puncture were normal. Finally, we established the diagnosis of adult-onset LCH with multisystem involvement. He was treated

Case of X‐linked hypohidrotic ectodermal dysplasia with a novel EDA missense mutation

1 Okamura K, Yoshizawa J, Abe Y et al. Oculocutaneous albinism (OCA) in Japanese patients: five novel mutations. J Dermatol Sci 2014; 74: 173–174. 2 Cullinane AR, Curry JA, Carmona-Rivera C et al. A BLOC-1 mutation screen reveals that PLDN is mutated in Hermansky-Pudlak Syndrome type 9. Am J Hum Genet 2011; 88: 778–787. 3 Nazarian R, Falcon-Perez JM, Dell’Angelica EC. Biogenesis of lysosome-related organelles complex 3 (BLOC-3): a complex containing the Hermansky-Pudlak syndrome (HPS) proteins HPS1 and HPS4. Proc Natl Acad Sci USA 2003; 100: 8770–8775. 4 Suzuki T, Ito S, Inagaki K et al. Investigation on the IVS5 + 5G –> a splice site mutation of HPS1 gene found in Japanese patients with Hermansky-Pudlak syndrome. J Dermatol Sci 2004; 36: 106– 108. 5 Natsuga K, Akiyama M, Shimizu T et al. Ultrastructural features of trafficking defects are pronounced in melanocytic nevus in Hermansky-Pudlak syndrome type 1. J Invest Dermatol 2005; 125: 154– 158.

Successful treatment of Raynaud's phenomenon and digital ulcers in systemic sclerosis patients with botulinum toxin B injection: Assessment of peripheral vascular disorder by angiography and dermoscopic image of nail fold capillary

We recently identified the efficacy and safety of a botulinum toxin (BTX)‐A/B in Raynauds phenomenon (RP) and digital ulcers (DU) in Japanese patients with systemic sclerosis (SSc). Detailed assessments of peripheral vascular disorder using angiography and dermoscopic images of nail fold capillaries have not been performed previously. This study aimed to evaluate the effect of BTX‐B on SSc‐associated peripheral vascular disorder. Two SSc patients who suffered with RP and DU were treated with a BTX‐B injection, and thereafter the symptoms of RP were improved and DU healed in both patients. Furthermore, angiography showed an increased blood flow to the palm and fingers, and dermoscopic images of nail fold capillary changes showed improvement. These results suggest that a BTX‐B injection may increase peripheral blood flow and improve RP and DU in SSc patients.