A case of Wong‐type dermatomyositis treated with rituximab and IVIG

A 37‐year‐old woman presented to our rheumatology–dermatology clinic with a rash, muscle weakness and fatigue. She has had prior diagnoses of cutaneous lupus and lichen planus based on skin biopsies. She did not respond to topical steroids, hydroxychloroquine and dapsone. Clinically, she had sharply demarcated photo‐distributed erythema over the upper back, chest and upper arms, along with hyperkeratotic follicular papules on bilateral upper arms, shoulders, posterior neck, behind the ears, chest including breasts, abdomen and right buttock. Investigations revealed a high titre ANA, elevated creatinine kinase, aldolase and positive anti‐MJ/nuclear matrix protein 2 (NXP‐2). A skin biopsy showed findings of connective tissue disease. The diagnosis of Wong‐type dermatomyositis was made. She responded to therapy with mycophenolate mofetil, rituximab and IVIG.     

Wells' syndrome related to tetanus vaccine

A 69-year-old woman was admitted to the Surgery Department of our hospital in November 1993 because of persistent erythematous skin lesions suspected clinically to be infectious cellulitis- Two months before admission she had noticed a red itchy pruriginous plaque, neasuring 10 × 5 cm, on her left buttock that had become larger in spite of treatment with oral antimicrobials. One month before entry, two more lesions flared up on her right buttock and left groin that soon became infiltrated. She did not notice any systemic symptoms. Past history of urticaria, atopy, tinea infection, or insect bites was not recalled. Two months before the first skin lesion erupted, the second dose of a tetanus vaccine had been given to the patient. The patient's temperature was 36.5°C on admission. On physical examination the patient appeared well and there were no systemic abnormalities. Examination of the skin revealed a red indurated plaque covering her left buttock and another one measuring 4 × 5 cm on the right buttock. A red nodular lesion, 3 × 2 cm in size, on her left groin was also noticed (Fig. 1). Laboratory investigation revealed a total white count of 9.7 × l0³mm³, with 68% neutrophils and 22% lymphocytes. Erythrocyte sedimentation rate was 110/h. Her erythrocyte count was 3.33 × 10⁶mm³. Blood glucose, kidney and liver function tests, and complement were all normal, and a polyclonal hypergammaglobulinemia of 23 g/L was seen on immunoelectrophoresis. Antinuclear antibodies (ANA) and alpha-1-antitrypsin antibodies were negative. Parenteral antibiotic therapy with penicillin and clindamycin was started with no improvement. After surgical debridement, no purulent exudate was found. A skin biopsy specimen of an infiltrated plaque was taken, showing an inflammatory infiltrate of lymphocytes and eosinophils, mostly perivascular, in the superficial and deep dermis, but especially in the deep subcutaneous tissue, with histiocytes and clusters of eosinophilic granules on the collagen fibers. Eosinophilic cellulitis (Wells' syndrome) was diagnosed and therapy with 20 mg/day of prednisone was started immediately. The skin lesions improved in a few days and the dose was tapered 3 weeks after starting therapy with oral steroids. She has not experienced any recurrence.     

Generalized linear porokeratosis

A 23-year-old woman was seen for widespread skin lesions present since the age of 2.5 years. Twenty years ago, she developed a brown macular lesion on her right buttock. The lesion became hyperkeratotic and subsequently spread through the posterior aspect of her right leg. It later spread to the right side of the trunk and to the right arm. When she was 9 years old, she developed similar lesions on her left arm and leg. After she was 13 years old, no new skin lesions appeared. There was no family history of similar lesions. On examination, there were numerous linear and whorled, reddish-brown, hyperkeratotic plaques, with central atrophy and raised borders, following Blaschko's lines on all of the extremities. These lesions on the extremities extended to the dorsum of the hands and feet (Fig. 1). She had hyperkeratotic lesions on the pressure points of both of the soles, but no palm involvement. The number of lesions on the right side was greater than that on the left. Reddish-brown annular plaques with central atrophy and raised borders, appearing in zosteriform configuration, and numerous individual 2-3-mm erythematous lichenoid papules were observed on the right side of the thorax and the right inguinal region (Fig. 2). No face, scalp, or mucous membrane involvement was seen. The nails of the second and fifth fingers of the right hand and the nail of the third finger of the left hand showed nail dystrophy with longitudinal ridges and pterygium. All the nails of the right foot and the nails of the first and fifth toes of the left foot showed dystrophic changes with subungual keratosis. The patient was otherwise in good health. Two biopsy specimens taken from a hyperkeratotic plaque and a lichenoid papule showed an epidermal invagination with angulated parakeratotic tier, denoting cornoid lamella. The epidermis just underneath the cornoid lamella displayed vacuolization and the granular layer was absent. The adjacent epidermis was atrophic, and hydropic degeneration within the basal cell layer was seen. In the dermis, a nonspecific, mild, chronic, inflammatory cell infiltrate, telangiectatic vessels, and pigment-laden macrophages were present. These findings were consistent with linear porokeratosis (Fig. 3). Microscopic examinations and mycologic cultures of the nails were negative. We decided to treat our case systemically with retinoids, but the patient refused this therapy. So, topical tretinoin 0.05% was started once a day. A marked improvement was observed in hyperkeratosis through the first 4 weeks of treatment and plateaued at 8 weeks. After 10 weeks, the lesions had almost disappeared. We planned to continue the applications every other day. One year later, she remains stable with application of topical tretinoin 0.05% twice a week and is satisfied with the final appearance. She is under regular follow-up.     

Electronmicroscopic Observation of the Basement Membrane Zone in Focal Dermal Hypoplasia

A 3-month-old boy had reticulated achromic and atrophic skin lesions on the right buttock and thigh, as well as partial syndactyly of the third and fourth toes of the right foot. With hematoxylin and eosin stain, an epidermal defect, abnormally located fat cell lobules, and absence of the upper and midportions of the dermis were observed, conforming to a typical histopathologic picture of focal dermal hypoplasia. Electron microscopic examination of the atrophic site showed loose collagen bundles, collagen fibers with loss of regular bands, abnormal fibroblasts, and disruption of the basement membrane zone. These defects in the basement membrane zone strongly suggest that abnormal formation of type IV collagen is associated with focal dermal hypoplasia, and that this abnormal formation of collagen is correlated with the clinical sign of skin atrophy.     

Leg ulcers and hydroxyurea: report of three cases with essential thrombocythemia

CASE 1: A 65-year-old woman with essential thrombocythemia (ET) had been taking oral hydroxyurea (HU), 1,000 mg daily, for 7 years. Six months ago, she developed an ulcer on the outer part of her left ankle, which healed spontaneously within 2 months. She presented with a new, tender, shallow ulcer, 2 cm x 2 cm in size, at the same site. Doppler examination revealed thrombosis of the left common femoral vein and a calcified atheroma plaque of the left common femoral artery. The dosage of HU was decreased to 500 mg daily when the platelet counts were found to be within normal levels. The ulcer completely healed within 2 months with occlusive wound dressings, and has not recurred within the follow-up period of 1 year. CASE 2: A 56-year-old women presented with multiple, painful, leg ulcers of 1 year duration. She had been diagnosed as having ET and had been on HU therapy, 1,500 mg/day, for the past 5 years. Interferon-alpha-2b was started 3 months ago, in addition to HU, which was tapered to 1,000 mg daily. She had suffered from hypertension for 20 years treated with nifedipine and enalapril, and had recently been diagnosed with diabetes mellitus which was controlled by diet. Examination revealed three ulcers located on the lateral aspects of both ankles and right distal toe. Arterial and venous Doppler examinations were within normal limits. Histopathology of the ulcer revealed nonspecific changes with a mixed inflammatory cell infiltrate around dermal vessels. The ulcers completely healed within 10 weeks with topical hydrocolloid dressings. After healing, she was lost to follow-up. A year later, it was learned that she had developed a new ulcer at her right heel, 3 months after her last visit (by phone call). This ulcer persisted for 8 months until HU was withdrawn. CASE 3: A 64-year-old woman with ET presented with a painful leg ulcer of 6 months' duration. She had been taking oral HU for 5 years. She had a 20-year history of hypertension treated with lisinopril. Examination revealed a punched-out ulcer of 2 cm x 2 cm over the right lateral malleolus. Doppler examination of the veins revealed insufficiency of the right greater saphenous and femoral veins. Angiography showed multiple stenoses of the right popliteal and femoral arteries. As her platelet count remained high, HU was continued. During the follow-up period of 13 months, the ulcer showed only partial improvement with local wound care.     

Biopsy‐Proven Spontaneous Regression of a Rhabdomyomatous Mesenchymal Hamartoma

Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare, benign, congenital tumor of the dermis and subcutaneous tissue comprised of skeletal muscle and adipose and adnexal elements. Although the majority of cases are an incidental finding in otherwise healthy patients, some have been reported in association with other anomalies. We present a full‐term boy evaluated on day 2 of life for two lesions located on the midline of the lower back and right buttock that each appeared clinically as an atrophic, pink plaque. Ultrasound of the midline lesion revealed an underlying lipomyelomeningocele with a tethered cord in the spinal canal. Histopathology of the right buttock cutaneous lesion was consistent with a diagnosis of RMH. Surgical excision was performed on the midline intradural lipoma and the lesion on the buttock was monitored clinically. Repeat biopsy of this site at 1 year of age revealed complete spontaneous regression. This case highlights three interesting features: the association with an occult spinal dysraphism lipomyelomeningocele and tethered cord, the clinical presentation of an atrophic plaque as opposed to the more commonly reported raised lesions, and the phenomenon of spontaneous regression of the lesion. Most importantly, this final feature of regression in our patient suggests that, in the absence of symptoms, clinical observation of RMH lesions is warranted for spontaneous regression for 1 to 2 years provided that no functional deficit is noted and that the cutaneous or deeper lesions are not causing a medical problem.     

累及神经系统的恶性萎缩性丘疹病

患者女,48岁,全身丘疹、斑块6年伴右眼视物不清1年就诊。体检：躯干、四肢可见瓷白色萎缩性丘疹,外周伴红晕及毛细血管扩张。右眼右侧斜视受限,视力减退,视野缺损。左手食指、拇指麻木,右侧胫骨前麻木。腹部皮损组织病理检查：真皮层坏死伴黏液样变性,深部数个小血管周围见淋巴细胞为主的炎细胞浸润,数个小血管腔闭塞。肠镜检查：全结肠散在片状充血、糜烂、浅溃疡形成;肌电图：股四头肌源性损害;粪隐血+++。诊断：恶性萎缩性丘疹病。治疗：口服阿司匹林、双嘧达莫治疗3个月,病情无进展。     

河南不规则毛霉致原发性皮肤毛霉病一例

【摘要】 患者女,50岁。右上肢皮肤结节、斑块、红肿、溃烂6年,右臀部斑块2年,右面颊斑块1年。曾单独口服特比萘芬、伊曲康唑治疗无效。右上肢及臀部皮损组织病理及PAS染色均可见真皮内宽大呈直角分支菌丝。真菌培养及分子生物学鉴定为不规则毛霉。联合伊曲康唑和特比萘芬治疗2个月仍无效,改为静脉滴注两性霉素B（总量1 060 mg）治疗,患者右面颊、右臀部斑块肿胀逐渐消退,右上肢包括右手背斑块、红肿、坏死、黑痂消退,皮损愈合成瘢痕,取右上肢皮肤组织再次行真菌培养阴性。随访1年未复发。     

Fluid-fluid level in a giant epidermal cyst of the buttock

We describe a case of a giant epidermal cyst of the buttock that demonstrated a fluid-fluid level on imaging. There have been no previously reported cases of epidermal cysts containing a fluid-fluid level, so our case was considered to be very exceptional. A 39-year-old man had had a slowly enlarging giant subcutaneous mass in the left buttock for more than 10 years. It was elastic-soft, well-circumscribed and smooth-surfaced. Ultrasonography showed a well-circumscribed hypoechoic mass with a fluid-fluid level in the subcutis of the left buttock. Magnetic resonance imaging (MRI) showed a well-circumscribed cystic mass measuring 8 cm x 5.5 cm x 5.5 cm in the same place that the ultrasonography indicated. It was hypointense on the T1-weighted image, hyperintense on the T2-weighted image, and contained a fluid-fluid level on both the T1- and T2-weighted images. It bordered on the anus, but neither adhered nor formed a fistula with it. A surgical excision was performed. The mass was diagnosed histopathologically as an epidermal cyst. The occurrence of fluid-fluid levels in epidermal cysts should be kept in mind in the differential diagnosis of subcutaneous soft tissue tumors.     

Neurologic variant of epidermal nevus syndrome with a facial lipoma.

A 1-year-old girl presented for evaluation of a linear plaque on her forehead. She was born at 36 weeks' gestation following an uncomplicated pregnancy and delivery. At birth, she was noted to have an enlarged right cheek. She had no seizure history, but developed grand mal seizures 1 year later. On examination, she had a yellow plaque on her forehead which extended onto her nose. Under her right jaw, extending onto her anterior neck, there was a café-au-lait macule within which there was a yellow plaque which followed the lines of Blaschko. Her right cheek was enlarged and was erythematous (Fig. 1). Magnetic resonance imaging (MRI) of her face showed a mass in the right cheek deep to the subcutaneous fat tissue layer. The signal from the mass was identical to that from the fat, indicating that the mass represented a lipoma. This was later excised surgically and was histologically a lipoma. MRI of the brain demonstrated enlargement of the right lateral ventricle in addition to enlargement of the right cerebral hemisphere. There was also evidence of abnormal gyral architecture. Computerized tomography (CT) three-dimensional reconstruction of the skull demonstrated overgrowth of the right maxilla, right mandible, and right orbit (Fig. 2).     

A case of trichoadenoma arising in the buttock.

A 41-year-old Japanese man with trichoadenoma on his buttock was described. Histological examination showed numerous horn cysts and some tumor islands consisting entirely of eosinophilic epithelial cells. A review of 24 reported cases of trichoadenoma revealed that most of them developed on the facial region (14 of 24 cases, 58%); the buttock (6 of 24, 25%) was the next most common site.     

Unusual clinical features of cutaneous tuberculosis in an immune compromised patient

We report a case of an unusual form of cutaneous tuberculosis in an 82-year-old woman. She visited our hospital because of an intractable ulcer on the fifth finger of her right hand. While examining the ulcer surrounding half of her right fifth finger and covered with necrotic tissue, we also perceived a nodule with crust on the forearm, multiple subcutaneous nodules on the right forearm and upper arm, and a hen's-egg-sized agglomerative nodule on the axilla. All the lesions were located on her right arm. Skin biopsy specimens showed granulomatous tissue with necrosis in the lesions. Mycobacterium tuberculosis was identified by culture of a biopsied specimen, so the diagnosis was confirmed. Further examination revealed that she also had pulmonary tuberculosis. Cutaneous tuberculoses are classified morphologically with reference to host immune status, but no satisfactory classification exists. The present case can't be classified into any of the types which have been proposed so far. She is elderly and suffers from liver cirrhosis, hepatocellular carcinoma and myelodysplastic syndrome. The resulting acquired immunosuppression may have caused a unique form of cutaneous tuberculosis.     

儿童皮肤非结核分枝杆菌感染1例

患儿女,3岁6个月。右臀部皮疹3年,局部曾有蚊虫叮咬史。皮损特征为边界清楚的紫红色溃疡性斑块。病理显示为结核样肉芽肿改变,抗酸染色阳性。诊断:非结核分枝杆菌感染。予口服利福平联合克拉霉素治疗3个月,皮损愈合。     

Atrophic nodular cutaneous amyloidosis

Primary cutaneous amyloidosis is limited to the skin without involving any other tissue. Nodular amyloidosis is rare, and atrophic nodular cutaneous amyloidosis is even rarer. We describe the fourth case of atrophic nodular cutaneous amyloidosis by searching PubMed databases. A 52-year-old female presented to our hospital with a 2-year history of orange papules and nodules without subjective symptom on her right abdomen. Review of systems was negative. Atrophic nodular amyloidosis may progress to primary systemic disease in up to 7% of cases. Because our patient had no systemic involvement, she was diagnosed with atrophic nodular cutaneous amyloidosis based on characteristic symptoms and histopathologic examination. Routine follow-up for this patient is necessary to detect any potential disease progression.     

Fatty acid analysis of transplanted adipose tissue

OBJECTIVE: To determine whether autologously transferred human adipose tissue maintains viability in vivo for prolonged periods. DESIGN: Six healthy female patients (mean age, 61.5 years; mean body mass index, 23.4 kg/m2) received autologous fat transplants from the gluteus to the nasolabial folds. Subcutaneous fat was sampled from facial and gluteal sites 4 times in 1 year. SETTING: Private practice, basic science research center. INTERVENTION: After local anesthesia, 10 g of subcutaneous adipose tissue was harvested from the right buttock of each patient. Ten milligrams of adipose tissue was aspirated from the right nasolabial fold. Five grams of gluteal fat was then injected into each nasolabial fold using a uniform monolayer threading technique with no overcorrection. As controls, 10 mg of adipose tissue was obtained from the opposite left buttock and left cheek. Adipose tissue from the transplanted and control facial and gluteal sites was sampled at 4, 6, and 12 months after transplantation. MAIN OUTCOME MEASUREMENTS: Gluteal fat has more monounsaturated fatty acids and less saturated fatty acids than facial fat. This unique site-specific fatty acid pattern was used to assess the course of the survival of transplanted adipose tissue in the nasolabial region. In all fat samples, the percent area (weight percentage) was obtained for each fatty acid (C12:0 to C22:6 omega-3) using capillary gas chromatography. Clinical results were also analyzed by macrophotographs. RESULTS: As expected, gluteal fat had significantly more monounsaturated fatty acids and less saturated fatty acids than facial fat. In 5 of 6 patients, at 4, 6, and 12 months after transplantation, the fatty acid pattern at the transplanted recipient site was similar to the pattern of the control facial site. However, at 4 months, 1 patient had a fatty acid pattern in the transplant recipient site that was similar to the pattern of her gluteal fat. This pattern persisted for 1 year. Fat retention at the transplant site was corroborated by photographic assessment. CONCLUSIONS: Long-term adipocyte survival is an achievable goal following fat transfer. The importance of harvesting and injection techniques as well as adipose tissue characteristics require further study.     

Progesterone-induced urticaria: response to buserelin

Summary We report a 41-year-old Caucasian woman with polycystic liver and kidney disease, and a 9-year history of severe cyclical urticaria related to her menses. The urticaria was not adequately controlled by antihistamines or Prempak-C® (conjugated oestrogens and norgestrel). Intradermal testing with progesterone was strongly positive at 30 min and 18 h. Buserelin, administered intranasally at doses of up to 800 μg daily, controlled the urticaria within 4 weeks, and she was completely symptom-free thereafter. She had therapy-induced amenorrhoea and occasional hot flushes. Unfortunately, her liver cysts progressively enlarged, and a right hepatectomy was performed in June 1992, but she died after complications 3 weeks later. Prior to this she had been free of urticaria for 6 months after starting buserelin. Buserelin, a gonadotrophin-releasing hormone analogue, may he useful in the management of progesterone-induced urticaria, in patients in whom conjugated oestrogens are contraindicated or unhelpful.     

Lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy

An 11-year-old girl presented with a one-year history of multiple, hard, slightly painful subcutaneous nodules on her right cheek, upper arms, and buttock. Histology of a skin biopsy specimen showed a lobular panniculitis. Laboratory studies revealed positive ANA, anti-double strand DNA, and elevated muscle enzymes. She was diagnosed as having lupus panniculitis. During hydroxychloroquine treatment, erythema over knuckle joints developed. These unusual clinical and laboratory findings of panniculitis associated connective tissue diseases made it difficult to make a precise diagnosis. We report this unusual case of lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy.     

Two Japanese cases of localized involutional lipoatrophy

We present two Japanese cases of involutional lipoatrophy. The first case is that of a 30-year-old woman, who first appeared at our hospital complaining of a localized, well-demarcated depression, approximately 3 x 4 cm in size, normal to slightly erythematous in coloration, on the lateral side of the left upper arm (Fig. 1a). The condition was asymptomatic, and she had noticed this anomaly a month prior to consultation. She received intramuscular injections of corticosteroids of unknown dosage at the affected site for the treatment of allergic rhinitis 4 months prior to her present consultation. The second patient, a 23-year-old woman, appeared at our hospital complaining of a similar macule 4 x 4 cm in size, which she noticed several weeks prior to her most recent consultation. She had no history of injury or injection at the site before the development of the condition (Fig. 1b). She had been under treatment for atopic dermatitis since early childhood and was treated only with topical applications of white petrolatum containing 2% salicylic acid for the past several years. In order to rule out the possibility of acquired partial lipodystrophy associated with localized scleroderma, lupus profundus and the other connective tissue diseases, a histological examination was performed for both patients. Histopathological analysis of the region exhibited a well-defined fat lobule composed of numerous small adipocytes (Fig. 1c) embedded in hyaline connective tissue. Edema and dilated capillaries were noticeable in the subcutaneous tissue surrounding the area. Inflammatory cells were not prominent, although mononuclear cells were observed in both patients. No epidermal change was seen in either patient. Direct and indirect immunofluorescence studies revealed no deposits of immunoreactants in the skin of either patient. Immunohistochemical studies with the antibody against macrophage (anti-CD68 antigen; DAKO.) showed that positive cells were scattered around blood vessels and shrunken lipocytes in the subcutaneous tissues (Fig. 1d). Most of these cells in the fat lobules were also positive for mucin stains such as Alcian blue. No abnormal findings came to light in the ordinary hematological and blood chemistry examinations of both patients. The autoantibody screening tests using antinuclear, anti-DNA, anticentromere, and anti-Scl-70 antibodies were negative in both patients.     

Reactions to Penicillamine: A Case of Cutis Laxa,Elastosis Perforans Serpiginosa and “Pseudo” Pseudoxanthoma

This patient was a 61‐year‐old white female who received several years of penicillamine therapy for the treatment of cystinuria. She subsequently developed penicillamine induced cutis laxa, elastosis perforans serpiginosa, and pseudoxanthoma elasticum like skin lesions. In addition, she suffered from numerous chronic bilateral lower extremity skin ulcerations. Her past medical history was also significant for end stage renal disease requiring hemodialysis and pulmonary fibrosis. She presented to the University of Miami Wound Care Center in 1/04 for treatment of her chronic ulcerations. On physical examination, the patient had multiple large hyperpigmented plaques with central ulcerations on her lower extremities. Some of the ulcers had overlying crust and others were covered with yellow fibrinous tissue. She also had generalized thickened, lax skin with multiple folds. On her neck, thighs, back and arms were violaceous, atrophic, serpiginous plaques with peripheral crusted erosions. A biopsy taken from the patients left thigh revealed dermal elastosis and the features of pseudo‐pseudoxanthoma. Two additional biopsies taken from the left thigh demonstrated elastosis perforans serpiginosa. This case highlights multiple skin manifestations of penicillamine therapy.     

Buschke-Ollendorff syndrome: three generations in a Japanese family

Buschke-Ollendorff syndrome is an autosomal dominant disease characterized by disseminated connective tissue nevi of elastic type and osteopoikilosis. We report a 6-year-old Japanese boy with connective tissue nevi that showed slightly grouped yellowish or skin-colored papules and nodules, distributed from birth over his right thigh, right buttock, and back. Radiologic skeletal survey revealed osteopoikilosis. A skin biopsy specimen obtained from a papule showed that collagen bundles in the dermis were thickened and homogenized. The elastic fibers were not increased but were coarse and clumped. The boy's father, at age 34, has had osteopoikilosis and similar papules and nodules on his left buttock and back for the preceding 18 years. We studied the paternal grandfather, aged 65. He had osteopoikilosis and similar skin lesions on his lumbar region. None of the three had a history of hearing loss or malignant tumor. To our knowledge, this is the first report of three generations of Buschke-Ollendorf syndrome in a Japanese family.