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Laryngeal edema in a patient with dermatomyositis
Otolaryngology Case Reports 2 (2017) 19e21
Contents lists available at ScienceDirect
Otolaryngology Case Reports journal homepage: www.otolaryngologycasereports.com
Laryngeal edema in a patient with dermatomyositis Ryuzaburo Higo, M.D. a, *, Ayumi Yabe, M.D. a, Takuo Haruyama, M.D., Ph.D. a, Kouichi Yamauchi, M.D., Ph.D. a, Hiroshi Yoshikawa, M.D., Ph.D. a, Katsuhisa Ikeda, M.D., Ph.D. b a b
Department of Otorhinolaryngology, Head and Neck Surgery, Juntendo University, Urayasu Hospital, Urayasu-city, Japan Department of Otorhinolaryngology, Head and Neck Surgery, Juntendo University Hospital, Tokyo, Japan
a r t i c l e i n f o
a b s t r a c t
Article history: Received 29 November 2016 Received in revised form 3 January 2017 Accepted 4 January 2017 Available online 9 February 2017
Dermatomyositis (DM) is a multisystem connective tissue disease, characterized by inflammatory changes and degeneration of the skin and muscle. Laryngeal edema coinciding with DM is quite rare. We report a DM case with laryngeal edema that was first suspected to be caused by hypopharyngeal carcinoma but as then considered to represent a unique symptom preceding DM. A 61-year-old man with neck swelling and edema of the left arytenoid was referred to our institute. Since hypopharyngeal carcinoma was suspected, a biopsy for the left hypoparynx was performed, but the results showed an inflamed squamous epithelium with mild basal cell hyperplasia. After the appearance of muscle weakness extending to the proximal limbs and elevations in the serum CK and ALD levels, DM was suspected. MRI findings of the left upper rim and the result of n-EMG finally led to the diagnosis of DM. To the best of our knowledge, this is a second reported case of laryngeal edema occurring as a symptom that preceded DM. Although inflammation of the adjacent muscle and excessive vascular permability in subcutaneous tissue, due to DM, may be a cause of laryngeal edema, it is also possible that unknown causes related to DM lead to laryngeal edema, which is a novel, unique feature that distinguishes this case from typical DM cases. © 2017 Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/ licenses/by/4.0/).
Keywords: Dermatomyositis Laryngeal edema Neck swelling Hypopharyngeal carcinoma
1. Introduction
2. Case presentation
Dermatomyositis (DM) is a rare, systemic rheumatic disease, characterized by the inflammatory change and degeneration of the skin and muscle. Symptoms are mainly symmetrical proximal muscle weakness, subsequent amyotrophy and a skin sign represented by a heliotrope rash. Visceral disorder and/or malignant tumors often coincide with DM. Among the various symptoms with DM, laryngeal edema is a rare condition. We report a rare case of laryngeal edema in a patient with DM, which was first suspected to be caused by hypopharyngeal carcinoma, but finally determined to represent a unique symptom preceding DM.
A 61-year-old man had a 10-day history of cervical edema. A neck cellulitis was suspected, and he was treated with antibiotics at another hospital. These symptoms did not improve by the initial treatment, and he was referred to our institute. Laboratory data on the first visit were as follows: white blood cell count, 7800/mm2; platelet count, 189000/mm2; C-reactive protein, 1.35 mg/dL; serum levels of creatinine, blood urea nitrogen, and albumin, 0.74mg/dL, 13mg/dL, and 4.2g/dL, respectively; thyroid-stimulating hormone, free T3, free T4, within normal limit; anti-nuclear antibody, antiJo1-antibody, anti Scleroderma antibody, anti-smith antibody, antieSSeA/Ro antibody, antieSSeB/La antibody, and antiribonucleoprotein antibody, all negative; radio-allergosorbent test (RAST) for inhalation and food antigen, all negative. Computed tomography (CT) showed cervical edema without a neck abscess, and a contrasted lesion in the left piryform sinus (Fig. 1). The laryngeal endoscopic examination revealed a tumorous lesion in the left arytenoid, which showed significant inflammation, and the right vocal fold also appeared hypertrophic and ulcerated (Fig. 2). The tumor marker SCC was elevated to 2.0ng/ml. Since
* Corresponding author. Department of Otorhinolaryngology, Head and Neck Surgery, Juntendo University, Urayasu Hospital, Tomioka2-1-1, Urayasu-city, 2790021, Chiba, Japan. E-mail address: [email protected] (R. Higo).
http://dx.doi.org/10.1016/j.xocr.2017.01.003 2468-5488/© 2017 Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
20
R. Higo et al. / Otolaryngology Case Reports 2 (2017) 19e21
proximal right arm. The serum CK and ALD levels were elevated to 4583 IU/L and 25.3 IU/L, respectively. The patient was diagnosed with dermatomyositis (DM). Since hypopharyngeal carcinoma had already been excluded, a general surveillance for other malignancy was performed. Tumor markers, such as CEA, pro-GRP and CA-125, were within normal limits. Upper and lower gastrointestinal endoscopy, and whole body CT were also performed, and no malignant tumors were detected. After intravenous administration of 500 mg of methylprednisolone sodium succinate for three days, the cervical swelling markedly improved, and erythema of the chest, abdomen, and head also improved. Then, the treatment was changed to oral prednisolone (PSL) at 80 mg and PSL was gradually tapered. Three months after the initial therapy, the laryngeal edema also improved (Fig. 3). Serum CK level improved from 4583 to 44 IU/L in three months. One year after the initial therapy, the patient continues to take 30 mg of PSL, without skin or muscle symptoms, and the laryngeal edema has not reappeared.
3. Discussion
Fig. 1. Contrasted lesion in the left piryform sinus (arrow).
hypopharyngeal carcinoma was suspected, a biopsy for the left hypoparynx was performed, but the results showed an inflamed squamous epithelium with mild basal cell hyperplasia. The swelling of the cervical neck remained for two weeks, and inflammation of the scalp newly developed. Erythema spread through the neck, the precordial region, the abdomen, and the right forearm. Muscle weakness in the left thigh appeared one month after neck and laryngeal swelling onset, and the muscle weakness gradually extended to the proximal limbs. T2 weighted and gadolinium contrasting T1 weighted MRI showed inflammation in the proximal right upper arm. Skin biopsy revealed perivascular dermatitis with interface damage. The needle electromyogram (nEMG) revealed acute denervation and a myogenic change in the
Fig. 2. Laryngeal edema and tumorous lesion of the left arytenoid.
It is quite rare for laryngeal edema to precede DM. One DM case with facial and laryngeal edema [1] was reported. Since a tumorous lesion was detected by the CT and endoscopic examinations, a biopsy was performed for the differential diagnosis of cancer. The results showed inflammatory changes without malignancy. Other factors, such as allergic reaction or hypothyroidism, were also excluded by the laboratory data, and several auto-antibody studies for connective tissue disease were, at first, all negative. It was only after the appearance of muscle weakness extending to the proximal limbs and elevations in the serum CK and ALD levels that DM was suspected. Finally, MRI findings of the left upper rim and the result of n-EMG finally led to the diagnosis of DM. To the best of our knowledge, this is a second reported case of laryngeal edema occurring as a symptom that preceded DM. The cause of laryngeal edema in patients with DM is unclear. Several reported cases of generalized edema in patients with DM suggested that inflammation of adjacent muscle tissue or excessive vascular permeability in muscle and subcutaneous tissue, due to
Fig. 3. Laryngeal endoscopic finding three month after initial therapy. Left arytenoid edema markedly improved.
R. Higo et al. / Otolaryngology Case Reports 2 (2017) 19e21
immune complex-mediated vasculopathy, may cause subcutaneous edema [2,3]. Although inflammation of the adjacent muscle and excessive vascular permability in subcutaneous tissue may also be a cause of laryngeal edema, it is possible that unknown causes related to DM led to laryngeal edema, which is a novel, unique feature distinguishing this case from typical DM cases. Hypopharyngeal carcinoma was suspected in this case, but the biopsy revealed only inflammatory changes. It is well-known that DM is highly associated with malignancy, and the percentage of DM cases that develop malignancy is 40% [4]. Malignancies associated with DM included lung cancer, gastric cancer, breast cancer, and ovarian cancer in Europe and America. In contrast, nasopharyngeal cancer is the most commonly associated malignancy in Southeast Asia. Hypopharyngeal cancer in patients with DM is quite rare in the English literature. In order not to avoid overlooking malignancies, CT of the lung and abdomen, abdominal ultrasonography, and upper and lower gastrointestinal endoscopy are recommended. Mammography, CT of the pelvis, cytologic examination for the uterine cervix and uterus are also recommended for female patients. It is necessary to follow up for possible malignancies for 3e5 years after the DM onset. 4. Conclusion We report a rare case of laryngeal edema in a patient with DM, which was first suspected to be caused by hypopharyngeal carcinoma, but finally determined to represent a unique symptom preceding DM. Laryngeal edema is a very rare manifestation that can occur as the initial symptom of DM, although the cause of laryngeal edema in patients with DM is unclear. Additional cases
21
are needed to clarify the pathogenetic mechanism of laryngeal edema with DM. Statement of human rights All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent Informed consent was obtained from all individual participants included in the study. Conflict of interest The authors declare that they have no conflict of interest. References [1] Yoshida N, Kaieda S, Yoshimura S, Ida H. Facial and laryngeal edema in a patient with dermatomyositis. Intern Med 2014;53:921. [2] Werner de Castro GR, Appenzeller S, Bertolo MB, Costallat LT. Acute DM with subcutaneous generalized edema. Clin Rheumatol 2006;25:898e900. [3] O1 Gorelik, Almoznino-Sarafian D, Alon I, Rapoport MJ, Goltsman G, Herbert M, et al. Acute inflammatory myopathy with severe subcutaneous edema, a new variant? Report of two cases and review of the literature. Rheumatol Int 2001;20:163e6. [4] Callen JP. The value of malignancy evaluation in patients with dermatomyositis. J Am Acad Dermatol 1982;6:253e9.
Contents lists available at ScienceDirect
Otolaryngology Case Reports journal homepage: www.otolaryngologycasereports.com
Laryngeal edema in a patient with dermatomyositis Ryuzaburo Higo, M.D. a, *, Ayumi Yabe, M.D. a, Takuo Haruyama, M.D., Ph.D. a, Kouichi Yamauchi, M.D., Ph.D. a, Hiroshi Yoshikawa, M.D., Ph.D. a, Katsuhisa Ikeda, M.D., Ph.D. b a b
Department of Otorhinolaryngology, Head and Neck Surgery, Juntendo University, Urayasu Hospital, Urayasu-city, Japan Department of Otorhinolaryngology, Head and Neck Surgery, Juntendo University Hospital, Tokyo, Japan
a r t i c l e i n f o
a b s t r a c t
Article history: Received 29 November 2016 Received in revised form 3 January 2017 Accepted 4 January 2017 Available online 9 February 2017
Dermatomyositis (DM) is a multisystem connective tissue disease, characterized by inflammatory changes and degeneration of the skin and muscle. Laryngeal edema coinciding with DM is quite rare. We report a DM case with laryngeal edema that was first suspected to be caused by hypopharyngeal carcinoma but as then considered to represent a unique symptom preceding DM. A 61-year-old man with neck swelling and edema of the left arytenoid was referred to our institute. Since hypopharyngeal carcinoma was suspected, a biopsy for the left hypoparynx was performed, but the results showed an inflamed squamous epithelium with mild basal cell hyperplasia. After the appearance of muscle weakness extending to the proximal limbs and elevations in the serum CK and ALD levels, DM was suspected. MRI findings of the left upper rim and the result of n-EMG finally led to the diagnosis of DM. To the best of our knowledge, this is a second reported case of laryngeal edema occurring as a symptom that preceded DM. Although inflammation of the adjacent muscle and excessive vascular permability in subcutaneous tissue, due to DM, may be a cause of laryngeal edema, it is also possible that unknown causes related to DM lead to laryngeal edema, which is a novel, unique feature that distinguishes this case from typical DM cases. © 2017 Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/ licenses/by/4.0/).
Keywords: Dermatomyositis Laryngeal edema Neck swelling Hypopharyngeal carcinoma
1. Introduction
2. Case presentation
Dermatomyositis (DM) is a rare, systemic rheumatic disease, characterized by the inflammatory change and degeneration of the skin and muscle. Symptoms are mainly symmetrical proximal muscle weakness, subsequent amyotrophy and a skin sign represented by a heliotrope rash. Visceral disorder and/or malignant tumors often coincide with DM. Among the various symptoms with DM, laryngeal edema is a rare condition. We report a rare case of laryngeal edema in a patient with DM, which was first suspected to be caused by hypopharyngeal carcinoma, but finally determined to represent a unique symptom preceding DM.
A 61-year-old man had a 10-day history of cervical edema. A neck cellulitis was suspected, and he was treated with antibiotics at another hospital. These symptoms did not improve by the initial treatment, and he was referred to our institute. Laboratory data on the first visit were as follows: white blood cell count, 7800/mm2; platelet count, 189000/mm2; C-reactive protein, 1.35 mg/dL; serum levels of creatinine, blood urea nitrogen, and albumin, 0.74mg/dL, 13mg/dL, and 4.2g/dL, respectively; thyroid-stimulating hormone, free T3, free T4, within normal limit; anti-nuclear antibody, antiJo1-antibody, anti Scleroderma antibody, anti-smith antibody, antieSSeA/Ro antibody, antieSSeB/La antibody, and antiribonucleoprotein antibody, all negative; radio-allergosorbent test (RAST) for inhalation and food antigen, all negative. Computed tomography (CT) showed cervical edema without a neck abscess, and a contrasted lesion in the left piryform sinus (Fig. 1). The laryngeal endoscopic examination revealed a tumorous lesion in the left arytenoid, which showed significant inflammation, and the right vocal fold also appeared hypertrophic and ulcerated (Fig. 2). The tumor marker SCC was elevated to 2.0ng/ml. Since
* Corresponding author. Department of Otorhinolaryngology, Head and Neck Surgery, Juntendo University, Urayasu Hospital, Tomioka2-1-1, Urayasu-city, 2790021, Chiba, Japan. E-mail address: [email protected] (R. Higo).
http://dx.doi.org/10.1016/j.xocr.2017.01.003 2468-5488/© 2017 Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
20
R. Higo et al. / Otolaryngology Case Reports 2 (2017) 19e21
proximal right arm. The serum CK and ALD levels were elevated to 4583 IU/L and 25.3 IU/L, respectively. The patient was diagnosed with dermatomyositis (DM). Since hypopharyngeal carcinoma had already been excluded, a general surveillance for other malignancy was performed. Tumor markers, such as CEA, pro-GRP and CA-125, were within normal limits. Upper and lower gastrointestinal endoscopy, and whole body CT were also performed, and no malignant tumors were detected. After intravenous administration of 500 mg of methylprednisolone sodium succinate for three days, the cervical swelling markedly improved, and erythema of the chest, abdomen, and head also improved. Then, the treatment was changed to oral prednisolone (PSL) at 80 mg and PSL was gradually tapered. Three months after the initial therapy, the laryngeal edema also improved (Fig. 3). Serum CK level improved from 4583 to 44 IU/L in three months. One year after the initial therapy, the patient continues to take 30 mg of PSL, without skin or muscle symptoms, and the laryngeal edema has not reappeared.
3. Discussion
Fig. 1. Contrasted lesion in the left piryform sinus (arrow).
hypopharyngeal carcinoma was suspected, a biopsy for the left hypoparynx was performed, but the results showed an inflamed squamous epithelium with mild basal cell hyperplasia. The swelling of the cervical neck remained for two weeks, and inflammation of the scalp newly developed. Erythema spread through the neck, the precordial region, the abdomen, and the right forearm. Muscle weakness in the left thigh appeared one month after neck and laryngeal swelling onset, and the muscle weakness gradually extended to the proximal limbs. T2 weighted and gadolinium contrasting T1 weighted MRI showed inflammation in the proximal right upper arm. Skin biopsy revealed perivascular dermatitis with interface damage. The needle electromyogram (nEMG) revealed acute denervation and a myogenic change in the
Fig. 2. Laryngeal edema and tumorous lesion of the left arytenoid.
It is quite rare for laryngeal edema to precede DM. One DM case with facial and laryngeal edema [1] was reported. Since a tumorous lesion was detected by the CT and endoscopic examinations, a biopsy was performed for the differential diagnosis of cancer. The results showed inflammatory changes without malignancy. Other factors, such as allergic reaction or hypothyroidism, were also excluded by the laboratory data, and several auto-antibody studies for connective tissue disease were, at first, all negative. It was only after the appearance of muscle weakness extending to the proximal limbs and elevations in the serum CK and ALD levels that DM was suspected. Finally, MRI findings of the left upper rim and the result of n-EMG finally led to the diagnosis of DM. To the best of our knowledge, this is a second reported case of laryngeal edema occurring as a symptom that preceded DM. The cause of laryngeal edema in patients with DM is unclear. Several reported cases of generalized edema in patients with DM suggested that inflammation of adjacent muscle tissue or excessive vascular permeability in muscle and subcutaneous tissue, due to
Fig. 3. Laryngeal endoscopic finding three month after initial therapy. Left arytenoid edema markedly improved.
R. Higo et al. / Otolaryngology Case Reports 2 (2017) 19e21
immune complex-mediated vasculopathy, may cause subcutaneous edema [2,3]. Although inflammation of the adjacent muscle and excessive vascular permability in subcutaneous tissue may also be a cause of laryngeal edema, it is possible that unknown causes related to DM led to laryngeal edema, which is a novel, unique feature distinguishing this case from typical DM cases. Hypopharyngeal carcinoma was suspected in this case, but the biopsy revealed only inflammatory changes. It is well-known that DM is highly associated with malignancy, and the percentage of DM cases that develop malignancy is 40% [4]. Malignancies associated with DM included lung cancer, gastric cancer, breast cancer, and ovarian cancer in Europe and America. In contrast, nasopharyngeal cancer is the most commonly associated malignancy in Southeast Asia. Hypopharyngeal cancer in patients with DM is quite rare in the English literature. In order not to avoid overlooking malignancies, CT of the lung and abdomen, abdominal ultrasonography, and upper and lower gastrointestinal endoscopy are recommended. Mammography, CT of the pelvis, cytologic examination for the uterine cervix and uterus are also recommended for female patients. It is necessary to follow up for possible malignancies for 3e5 years after the DM onset. 4. Conclusion We report a rare case of laryngeal edema in a patient with DM, which was first suspected to be caused by hypopharyngeal carcinoma, but finally determined to represent a unique symptom preceding DM. Laryngeal edema is a very rare manifestation that can occur as the initial symptom of DM, although the cause of laryngeal edema in patients with DM is unclear. Additional cases
21
are needed to clarify the pathogenetic mechanism of laryngeal edema with DM. Statement of human rights All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent Informed consent was obtained from all individual participants included in the study. Conflict of interest The authors declare that they have no conflict of interest. References [1] Yoshida N, Kaieda S, Yoshimura S, Ida H. Facial and laryngeal edema in a patient with dermatomyositis. Intern Med 2014;53:921. [2] Werner de Castro GR, Appenzeller S, Bertolo MB, Costallat LT. Acute DM with subcutaneous generalized edema. Clin Rheumatol 2006;25:898e900. [3] O1 Gorelik, Almoznino-Sarafian D, Alon I, Rapoport MJ, Goltsman G, Herbert M, et al. Acute inflammatory myopathy with severe subcutaneous edema, a new variant? Report of two cases and review of the literature. Rheumatol Int 2001;20:163e6. [4] Callen JP. The value of malignancy evaluation in patients with dermatomyositis. J Am Acad Dermatol 1982;6:253e9.