A case of factitious subcutaneous emphysema
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Abstract
A 13-year-old girl presented with a 2-month history of recurrent soft tissue swelling of her right upper extremity. Dermatological examination revealed soft tissue crepitation and a small ulcer on the dorsum of her right hand. After several investigations including radiographic and systemic blood tests the diagnosis of factitious subcutaneous emphysema was made and the patient was referred to psychiatry for proper management.
Introduction
Dermatitis artefacta is a well-known but fortunately rare condition [1, 2, 3, 4]. It is a diagnosis of exclusion because the clinical picture is not compatible with known dermatological disorders, and neither blood tests nor histological investigations support a specific disease [5]. The diagnosis of such a condition is difficult because of multiple clinical aspects and the fear of missing an organic disease [6].
Clinical synopsis
A 13-year-old girl came to our department with a 2-month history of recurrent ulcers and soft tissue swelling on her right upper extremity. She mentioned that the ulcers and swellings occurred on the right hand and forearm every 2 weeks and resolved in a week. The patient did not have a history of associated systemic symptoms complained only about a subtle pain limited to these lesions.
On the examination , swelling and a small ulcer were observed on the dorsum of her right hand; crepitation of this tissue was present on palpitation (Figs. 1 and 2).
This tissue emphysema extended up to the distal parts of the forearm, however no signs indicating inflammation were found. In the preceding location of the lesion, there were three lesions in the form of small atrophic scars on the forearm (Fig. 3). Radiography confirmed the presence of subcutaneous emphysema (Figs. 4, 5).
Systemic laboratory tests were completely normal. With these findings and, in order not to miss gas-producing infections, a course of oral antibiotic was prescribed and the patient was asked to return some days later.
On second visit , the swelling around the lesion had considerably subsided. Considering the differential diagnosis associated with subcutaneous emphysema, no evidence supporting diagnoses other than factitious was observed during the clinical and laboratory investigations. Because the location and pattern of the lesions was similar to needle-prick marks, and because the affected areas were not in contact with the digestive and respiratory systems, suspicions were raised regarding the factitious nature of the emphysema. The patient's mother was consulted; she said that the patient had problems attending school, had disagreements with the family, and had access to syringes at home. Furthermore, the patient was left handed it was always the right upper extremity that was affected. On examination, it appeared that the patient was depressed mood and did not like to speak with the examiner.
The mother was advised that the patient had no serious disease, however, they should return for followup with any new lesions. The patient was also referred for consultation to the psychiatric department.
Discussion
Subcutaneous emphysema is the condition in which air or other gases penetrate the skin and submucosa resulting in soft-tissue distention. Among the causes of subcutaneous emphysema are trauma, iatrogenic factors, spontaneous pneumomediastinum, intra-abdominal disease, infectious disease, ulcers of the elbow, knee, and foot and factitious disease [7].
Subcutaneous gas, recognized by the presence of cutaneous crepitations, results from an infection with gas-producing organisms or an injury to the integrity of internal viscera that contain air (such as the lung and gastrointestinal tract) [8]. Subcutaneous emphysema of the hand can be benign and noninfectious in origin. Emphysema from gas-forming organisms is associated with systemic symptoms, whereas benign subcutaneous emphysema is not [9] .
The most common cause of subcutaneous emphysema is trauma. Spontaneous air dissection may result when the pulmonary alveolar or gastrointestinal continuity is violated; when an infection with gas-producing organisms, such as gas gangrene, is present; when air is pumped into subcutaneous tissue through existing cutaneous ulcers; or when air is injected into subcutaneous tissue [10]. Radiographic studies may demonstrate the presence, spread, and at times even the source of air [11].
Self-inflicted skin changes are rare but well known to dermatologists. They often go undiagnosed for quite some time until the clinical look of bizarre or unusual skin changes combined with non-specific histology and normal blood tests lead to a diagnosis.
Dermatitis artefacta (factitious dermatitis, pathomimia) patients can be difficult to diagnose as the patients initially deny any relation to self-infliction, but claim other factors to be of importance [5].
Factitious disorders are characterized by physical or psychological symptoms that are intentionally produced or feigned in order to assume the sick role. Factitious disorders should be distinguished from malingering, also in which the symptoms are produced intentionally but the individual has an obvious goal to achieve, such as to avoid standing trial or essential services and sometimes to receive compensation. In contrast, in a factitious disorder the motivation is a psychological need to assume the sick role and there are no external incentives for the behavior. Munchausen syndrome refers to a special type of factitious illness meeting DSM-IV criteria for chronic factitious illness with physical signs and symptoms [13, 14].
In dermatitis artefacta, the patient creates skin lesions to satisfy an internal psychological need, usually a need to be taken care of. The clinical presentation is characteristic, and differs from that of neurotic excoriations, delusional disorders, malingering, and Munchausen syndrome [2, 12, 16]. In dermatitis artefacta, female-to-male ratios range from 3:1 to 20:1, with the highest incidence of onset in late adolescence to early adult life [2].
Our patient's right hand and forearm were inflicted, explained by the fact that she was left-hand dominant. In a study conducted by Nielsen K et al., the anatomical regions of skin involved were the face and neck, hands, arms, legs, and trunk, which were equally affected in about 40 percent of cases, whereas the scalp and genital area were only affected in 12 and 3 percent, respectively. It was noteworthy that only 12 percent had a solitary lesion, 88 percent had multiple lesions. The three most common objective lesions were skin ulcers (72 %), excoriations (46 %) and erythema (30 %). The subjective complaints were pain (59 %) and itching (37 %), with no gender difference. The patients attributed the lesions to unknown (49 %), trauma (18 %) , allergy (16 %), self-infliction (9 %), insect bites (5 %) , and infection (4 %) [5].
Most patients have a personality disorder and borderline features are common. The patients' denial of psychiatric distress, and the arousal of negative feelings in healthcare personnel make management difficult [2]. What is remarkable is the high percentage of patient denial of self-infliction and the patients' lack of interest in talking with a psychiatrist [5]. When psychiatric referral is refused by the patient, the presciption of psychotropic drugs by dermatologists is helpful and appropriate. The upper dose range of selective serotonin reuptake inhibitors, or low dose atypical antipsychotic agents, may be effective. Except in mild transient cases triggered by an immediate stress, the prognosis for cure is poor. The condition tends to wax and wane with the circumstances of the patient's life [2].
Conclusion
When a patient presents with unexplained recurrent subcutaneous emphysema, suspect self-infliction and examine for punctuate marks. A factitious disorder is always a sign of a psychopathy. In this disorder, the patient has voluntary control over his or her behavior. Patients generally plan the full course of their factitious illness. As in obsessive-compulsive disorder, patients may accept the behavior but cannot control it. Childhood deprivation and rejection play a significant etiologic role [15]. The personality of these individuals shows a vulnerability to life events. Although the true prevalence of this entity is not known, the diagnosis of factitious disorder should be considered when an exhaustive investigation fails to reveal any explanation for patients symptoms.
References
1. Joe EK, Li VW, Magro CM, Arndt KA, Bowers KE. Diagnostic clues to dermatitis artefacta. Cutis. 1999 Apr;63(4):209-14. PubMed
2. Koblenzer CS. Dermatitis artefacta. Clinical features and approaches to treatment. Am J Clin Dermatol. 2000 Jan-Feb;1(1):47-55. PubMed
3. Harth, W., Mayer, K. and Linse, R. The borderline syndrome in psychosomatic dermatology. Overview and case report., J Eur Acad Dermatol Venereol,2004;18, pp. 503?07.PubMed
4. Wang, C. K. and Lee, J. Y. Monosymptomatic hypochondrial psychosis complicated by self-inflicted skin ulceration, skull defect and brain abcess., Br J Dermatol,1997;137, pp. 299?02.PubMed
5. Nielsen K, Jeppesen M, Simmelsgaard L, Rasmussen M, Thestrup-Pedersen K. Self-inflicted skin diseases. A retrospective analysis of 57 patients with dermatitis artefacta seen in a dermatology department. Acta Derm Venereol. 2005;85(6):512-5. PubMed
6. Megarbane H, Tomb R, Makhoul E, Halaby E. Dermatitis artefacta. Report of seven cases. J Med Liban. 2003 Jan-Mar;51(1):9-14. PubMed
7. Torgay A, Aydin E, Cilasun U, Durmaz L, Arslan G. Subcutaneous emphysema after dental treatment: a case report. Paediatr Anaesth. 2006 Mar;16(3):314-7. PubMed
8. De M, Stevenson J. Subcutaneous emphysema of upper limb. Emerg Med J. 2001 Nov;18(6):522. PubMed
9. van der Molen AB, Birndorf M, Dzwierzynski WW, Sanger JR. Subcutaneous tissue emphysema of the hand secondary to noninfectious etiology: a report of two cases. J Hand Surg [Am]. 1999 May;24(3):638-41. PubMed
10. Gershwin ME, Gude JK, Petralli J. Factitious subcutaneous emphysema. Ann Intern Med. 1971 Oct;75(4):585-7.
11. Craig RP, Boffard KD. Factitious subcutaneous emphysema: a case report. Injury. 1977 May;8(4):307-9.
12. Haenel T, Rauchfleisch U, Schuppli R, Battegay R. The psychiatric significance of dermatitis artefacta. Eur Arch Psychiatry Neurol Sci. 1984;234(1):38-41. PubMed
13. American Psychiatric Association, Diagnostic & Statistical Manual of Mental Disorders. Ed. 4, Washington DC, 1994.
14. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders(DSM-IV), 4th edn. Text revision. Washington DC:American Psychiatric Association,2000:517.
15. YUCEL, EMRE A. MD; KELES, NESIL MD; ARAMI, KIANOUSH MD; ORHAN, SERKAN MD; DEGER, KEMAL MD. Munchausen's syndrome: A case report with multiple symptoms. Otolaryngol Head Neck Surg. 2001 May;124(5):595-7. PubMed
16. Polat A, Kulaksizoglu IB, Vatansever S. An adolescent in Istanbul with Munchausen's syndrome. Int J Psychiatry Med. 2002;32(3):311-5. PubMed(Mostafa Mirshams Shahshah)
A 13-year-old girl presented with a 2-month history of recurrent soft tissue swelling of her right upper extremity. Dermatological examination revealed soft tissue crepitation and a small ulcer on the dorsum of her right hand. After several investigations including radiographic and systemic blood tests the diagnosis of factitious subcutaneous emphysema was made and the patient was referred to psychiatry for proper management.
Introduction
Dermatitis artefacta is a well-known but fortunately rare condition [1, 2, 3, 4]. It is a diagnosis of exclusion because the clinical picture is not compatible with known dermatological disorders, and neither blood tests nor histological investigations support a specific disease [5]. The diagnosis of such a condition is difficult because of multiple clinical aspects and the fear of missing an organic disease [6].
Clinical synopsis
A 13-year-old girl came to our department with a 2-month history of recurrent ulcers and soft tissue swelling on her right upper extremity. She mentioned that the ulcers and swellings occurred on the right hand and forearm every 2 weeks and resolved in a week. The patient did not have a history of associated systemic symptoms complained only about a subtle pain limited to these lesions.
On the examination , swelling and a small ulcer were observed on the dorsum of her right hand; crepitation of this tissue was present on palpitation (Figs. 1 and 2).
This tissue emphysema extended up to the distal parts of the forearm, however no signs indicating inflammation were found. In the preceding location of the lesion, there were three lesions in the form of small atrophic scars on the forearm (Fig. 3). Radiography confirmed the presence of subcutaneous emphysema (Figs. 4, 5).
Systemic laboratory tests were completely normal. With these findings and, in order not to miss gas-producing infections, a course of oral antibiotic was prescribed and the patient was asked to return some days later.
On second visit , the swelling around the lesion had considerably subsided. Considering the differential diagnosis associated with subcutaneous emphysema, no evidence supporting diagnoses other than factitious was observed during the clinical and laboratory investigations. Because the location and pattern of the lesions was similar to needle-prick marks, and because the affected areas were not in contact with the digestive and respiratory systems, suspicions were raised regarding the factitious nature of the emphysema. The patient's mother was consulted; she said that the patient had problems attending school, had disagreements with the family, and had access to syringes at home. Furthermore, the patient was left handed it was always the right upper extremity that was affected. On examination, it appeared that the patient was depressed mood and did not like to speak with the examiner.
The mother was advised that the patient had no serious disease, however, they should return for followup with any new lesions. The patient was also referred for consultation to the psychiatric department.
Discussion
Subcutaneous emphysema is the condition in which air or other gases penetrate the skin and submucosa resulting in soft-tissue distention. Among the causes of subcutaneous emphysema are trauma, iatrogenic factors, spontaneous pneumomediastinum, intra-abdominal disease, infectious disease, ulcers of the elbow, knee, and foot and factitious disease [7].
Subcutaneous gas, recognized by the presence of cutaneous crepitations, results from an infection with gas-producing organisms or an injury to the integrity of internal viscera that contain air (such as the lung and gastrointestinal tract) [8]. Subcutaneous emphysema of the hand can be benign and noninfectious in origin. Emphysema from gas-forming organisms is associated with systemic symptoms, whereas benign subcutaneous emphysema is not [9] .
The most common cause of subcutaneous emphysema is trauma. Spontaneous air dissection may result when the pulmonary alveolar or gastrointestinal continuity is violated; when an infection with gas-producing organisms, such as gas gangrene, is present; when air is pumped into subcutaneous tissue through existing cutaneous ulcers; or when air is injected into subcutaneous tissue [10]. Radiographic studies may demonstrate the presence, spread, and at times even the source of air [11].
Self-inflicted skin changes are rare but well known to dermatologists. They often go undiagnosed for quite some time until the clinical look of bizarre or unusual skin changes combined with non-specific histology and normal blood tests lead to a diagnosis.
Dermatitis artefacta (factitious dermatitis, pathomimia) patients can be difficult to diagnose as the patients initially deny any relation to self-infliction, but claim other factors to be of importance [5].
Factitious disorders are characterized by physical or psychological symptoms that are intentionally produced or feigned in order to assume the sick role. Factitious disorders should be distinguished from malingering, also in which the symptoms are produced intentionally but the individual has an obvious goal to achieve, such as to avoid standing trial or essential services and sometimes to receive compensation. In contrast, in a factitious disorder the motivation is a psychological need to assume the sick role and there are no external incentives for the behavior. Munchausen syndrome refers to a special type of factitious illness meeting DSM-IV criteria for chronic factitious illness with physical signs and symptoms [13, 14].
In dermatitis artefacta, the patient creates skin lesions to satisfy an internal psychological need, usually a need to be taken care of. The clinical presentation is characteristic, and differs from that of neurotic excoriations, delusional disorders, malingering, and Munchausen syndrome [2, 12, 16]. In dermatitis artefacta, female-to-male ratios range from 3:1 to 20:1, with the highest incidence of onset in late adolescence to early adult life [2].
Our patient's right hand and forearm were inflicted, explained by the fact that she was left-hand dominant. In a study conducted by Nielsen K et al., the anatomical regions of skin involved were the face and neck, hands, arms, legs, and trunk, which were equally affected in about 40 percent of cases, whereas the scalp and genital area were only affected in 12 and 3 percent, respectively. It was noteworthy that only 12 percent had a solitary lesion, 88 percent had multiple lesions. The three most common objective lesions were skin ulcers (72 %), excoriations (46 %) and erythema (30 %). The subjective complaints were pain (59 %) and itching (37 %), with no gender difference. The patients attributed the lesions to unknown (49 %), trauma (18 %) , allergy (16 %), self-infliction (9 %), insect bites (5 %) , and infection (4 %) [5].
Most patients have a personality disorder and borderline features are common. The patients' denial of psychiatric distress, and the arousal of negative feelings in healthcare personnel make management difficult [2]. What is remarkable is the high percentage of patient denial of self-infliction and the patients' lack of interest in talking with a psychiatrist [5]. When psychiatric referral is refused by the patient, the presciption of psychotropic drugs by dermatologists is helpful and appropriate. The upper dose range of selective serotonin reuptake inhibitors, or low dose atypical antipsychotic agents, may be effective. Except in mild transient cases triggered by an immediate stress, the prognosis for cure is poor. The condition tends to wax and wane with the circumstances of the patient's life [2].
Conclusion
When a patient presents with unexplained recurrent subcutaneous emphysema, suspect self-infliction and examine for punctuate marks. A factitious disorder is always a sign of a psychopathy. In this disorder, the patient has voluntary control over his or her behavior. Patients generally plan the full course of their factitious illness. As in obsessive-compulsive disorder, patients may accept the behavior but cannot control it. Childhood deprivation and rejection play a significant etiologic role [15]. The personality of these individuals shows a vulnerability to life events. Although the true prevalence of this entity is not known, the diagnosis of factitious disorder should be considered when an exhaustive investigation fails to reveal any explanation for patients symptoms.
References
1. Joe EK, Li VW, Magro CM, Arndt KA, Bowers KE. Diagnostic clues to dermatitis artefacta. Cutis. 1999 Apr;63(4):209-14. PubMed
2. Koblenzer CS. Dermatitis artefacta. Clinical features and approaches to treatment. Am J Clin Dermatol. 2000 Jan-Feb;1(1):47-55. PubMed
3. Harth, W., Mayer, K. and Linse, R. The borderline syndrome in psychosomatic dermatology. Overview and case report., J Eur Acad Dermatol Venereol,2004;18, pp. 503?07.PubMed
4. Wang, C. K. and Lee, J. Y. Monosymptomatic hypochondrial psychosis complicated by self-inflicted skin ulceration, skull defect and brain abcess., Br J Dermatol,1997;137, pp. 299?02.PubMed
5. Nielsen K, Jeppesen M, Simmelsgaard L, Rasmussen M, Thestrup-Pedersen K. Self-inflicted skin diseases. A retrospective analysis of 57 patients with dermatitis artefacta seen in a dermatology department. Acta Derm Venereol. 2005;85(6):512-5. PubMed
6. Megarbane H, Tomb R, Makhoul E, Halaby E. Dermatitis artefacta. Report of seven cases. J Med Liban. 2003 Jan-Mar;51(1):9-14. PubMed
7. Torgay A, Aydin E, Cilasun U, Durmaz L, Arslan G. Subcutaneous emphysema after dental treatment: a case report. Paediatr Anaesth. 2006 Mar;16(3):314-7. PubMed
8. De M, Stevenson J. Subcutaneous emphysema of upper limb. Emerg Med J. 2001 Nov;18(6):522. PubMed
9. van der Molen AB, Birndorf M, Dzwierzynski WW, Sanger JR. Subcutaneous tissue emphysema of the hand secondary to noninfectious etiology: a report of two cases. J Hand Surg [Am]. 1999 May;24(3):638-41. PubMed
10. Gershwin ME, Gude JK, Petralli J. Factitious subcutaneous emphysema. Ann Intern Med. 1971 Oct;75(4):585-7.
11. Craig RP, Boffard KD. Factitious subcutaneous emphysema: a case report. Injury. 1977 May;8(4):307-9.
12. Haenel T, Rauchfleisch U, Schuppli R, Battegay R. The psychiatric significance of dermatitis artefacta. Eur Arch Psychiatry Neurol Sci. 1984;234(1):38-41. PubMed
13. American Psychiatric Association, Diagnostic & Statistical Manual of Mental Disorders. Ed. 4, Washington DC, 1994.
14. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders(DSM-IV), 4th edn. Text revision. Washington DC:American Psychiatric Association,2000:517.
15. YUCEL, EMRE A. MD; KELES, NESIL MD; ARAMI, KIANOUSH MD; ORHAN, SERKAN MD; DEGER, KEMAL MD. Munchausen's syndrome: A case report with multiple symptoms. Otolaryngol Head Neck Surg. 2001 May;124(5):595-7. PubMed
16. Polat A, Kulaksizoglu IB, Vatansever S. An adolescent in Istanbul with Munchausen's syndrome. Int J Psychiatry Med. 2002;32(3):311-5. PubMed(Mostafa Mirshams Shahshah)