Factitious Ulcer Misdiagnosed as Pyoderma Gangrenosum

Dermatitis artefacta may represent a real challenge for the clinician. As the patient does not admit self-inflicting the lesions, misdiagnosis with other diseases, such as pyoderma gangrenosum, is common. Consequently, these patients normally go through unnecessary tests and receive potentially harmful treatments as clinicians determine their diagnosis.The authors present the case of a recurrent factitious abdominal ulcer that was initially diagnosed and treated as pyoderma gangrenosum. This report focuses on the necessity of suspecting dermatitis artefacta when morphology, history, and treatment failures are difficult to explain. It is essential to establish a supportive and confident approach and avoid initial confrontation. In-patient treatment may be useful and long-term follow-up may prevent recurrences.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that may present with cutaneous ulcers. The classic initial lesions are normally described as a small, red papule or pustule changing into a larger, ulcerative lesion, characterized by a deep ulceration with a violaceous border that overhangs the ulcer bed. Pyoderma gangrenosum lesions mainly affect the legs, but they may occur anywhere on the body. A minor trauma may lead to the development of new skin ulcers; consequently, surgical debridement must be avoided. This skin condition is known as pathergy. Systemic illnesses are seen in 50% of patients with PG and may occur prior to, concurrently with, or following the diagnosis. A commonly associated disease is inflammatory bowel disease. The diagnosis of PG is supported by compatible, but nonspecific, clinical and histological features.¹ Consequently, after an initial suspicion of PG, if no clinical response is achieved with an adequate immunosuppression treatment, another differential diagnosis must be considered such as, in this case, dermatitis artefacta (DA).^2,3 Dermatitis artefacta is defined as the deliberate and conscious production of self-inflicted skin lesions, such as ulcers, to satisfy an unconscious psychological or emotional need.⁴ It is also a diagnosis of exclusion, due to the absence of specific diagnostic characteristics. As the patient denies the self-infliction of the lesions, its diagnosis normally represents a challenge for the clinician. Patients with this condition require both dermatological assessment and psychosocial support.^4,5 The authors report the case of a factitious ulcer that was misdiagnosed as PG.

A 62-year-old woman with a history of hypertension, obesity, and anxiety-depressive syndrome who was in treatment with venlafaxine and quetiapine, presented with a 2-week history of a slightly painful abdominal ulcer. She had 2 previous similar episodes, the most recent being 6 months before the current presentation, which had been treated in different hospitals. She associated these recurrent ulcers to a mesh placement after surgery for hydatid cysts 20 years prior. These previous ulcers had resolved with conventional treatment for wound healing which consisted of saline cleansing solution and foam dressings over several months. 

She presented with a 6 cm x 4 cm abdominal ulcer, with well-defined, raised, erythematous borders and a clean, deep wound bed (Figure 1). Blood tests, including immunoglobulins and serum proteinogram, were completely normal. Skin biopsies were taken for both microbiological and pathological studies. Staphylococcus aureus was isolated, and no specific histopathological features were found. The biopsy mainly showed chronic inflammatory changes with dermal fibrosis, with absence of vasculopathy or granulomas. Considering these unspecific findings and the clinical suspicion of PG, treatment with prednisone 1mg/kg/day orally was ordered. The occlusive dressings were used to promote autolytic debridement. Clinical evolution was satisfactory. Prednisone was gradually tapered, and 3 months later, reepithelization was complete. 

Unexpectedly, 2 weeks after the last visit, the patient presented with a 1-week history of a new abdominal lesion, with similar characteristics to the previous one (Figure 2). She expressed frustration with the situation and reported the lesion was more painful. With suspicion of PG relapse, the authors initiated treatment with prednisone 1mg/kg/day, amoxicillin/clavulanic acid,  and occlusive wound dressings. One week later, the lesion had worsened, measuring 8 cm x 6 cm, and was covered with a wide central necrotic eschar (Figure 3). During that week, the patient had gone to a different institution for her wound care. The patient explained that despite the authors’ recommendations, the nurse at that facility had used surgical debridement. The authors associated this evolution with pathergy and added cyclosporine 5 mg/kg/day. During follow-up, new peripheral necrotic lesions appeared and the larger, central lesion did not improve (Figure 4). 

Due to this lack of response to immunosuppression therapy, the initial diagnosis of PG was reconsidered. New blood and skin samples were taken. Elevated C-reactive protein (27.5) was the only remarkable finding. Proteus was isolated, and no specific histologic features were found. Taking into account the patient’s history of anxiety-depressive syndrome, test unspecificity, and unexplainable lesion morphology and evolution, the authors finally suspected a factitious ulcer. The authors indicated inpatient treatment with antibiotic therapy and occlusive dressings to avoid having the patient manipulate the lesions. Three weeks later, she was discharged with great amelioration. Local treatment continued on an outpatient basis, and total reepithelization was achieved 1 month later (Figure 5). 

Both psychiatrists and dermatologists conducted the follow-ups for this patient. Direct confrontation was avoided and Socratic dialogue was used, which involves systematically asking questions that encourage deep thought. The patient revealed she was a survivor of sexual abuse by her father. Moreover, she had a conflict-filled relationship with a son, and desired more attention and affection from her husband. Psychotherapeutic meetings with the psychiatrist focused on establishing and maintaining a relationship with the patient, and her current antidepressant treatment was also modified. 

As the patient would not admit to self-inflicting the lesions, the authors do not know the mechanism she used to produce them.

Several diseases may mimic PG and misdiagnosis is reported in as high as 10% of cases.² Pyoderma gangrenosum is a neutrophilic dermatosis that normally presents with rapidly progressive, painful, suppurative cutaneous ulcers with edematous, boggy, blue, undermined, and necrotic borders. This condition needs to be treated with high-dose prednisone or other immunosuppressive medications.¹ Consequently, the misdiagnosis of PG may worsen infectious or malignant diseases that produce similar ulcerations. As PG is a diagnosis of exclusion, with no pathognomonic laboratory or histologic findings, other diseases should be ruled out before a diagnosis of PG is made. Moreover, diagnosis of PG may be reconsidered when the condition fails to respond to standard treatment. A differential diagnosis should include vascular occlusive or venous disease, vasculitis, cancer, infection, exogenous tissue injury, and other inflammatory disorders. However, some cases such as DA, which is also a diagnosis of exclusion, represent a challenge for the clinician.^2,3

Dermatitis artefacta is a type of factitious disorder defined as any condition involving self-inflicted skin lesions for which the patient denies all responsibility. Skin ulcers are the most frequent presentation.⁶

The production of these lesions is thought to satisfy an unconscious psychological or emotional need, such as receiving medical care. In some patients, DA may be considered part of Munchausen syndrome, presenting with affectation of different organs and repeated visits to different hospitals (ie, “doctor shopping”).³ Psychiatric disorders are normally associated with DA, such as anxiety or depression, emotional deprivation, or a personality disorder with borderline features, which include impulsivity and instability of behaviors, interpersonal relationships, and self-image. Consequently, it is essential to consider any previous psychiatric diagnoses, a history of substance abuse, and any possible psychosocial stressors. A long medical file with numerous consultations may be another instructive clue.

The attitude of patients with DA may vary from great mental calm and complacency about their condition (ie, la belle indifference) to anger and impatience. As an essential characteristic of DA is that patients conceal their role in producing the lesions, it may be easily differentiated from other self-inflicted dermatoses, such as self-mutilations in patients in various states of mental health, neurotic excoriation, and trichotillomania.⁷ The definition of DA excludes cases in which a rational motive exists for the self-infliction of lesions, thereby excluding malingerers.⁴

Factitious ulcers are characteristically resistant to healing in spite of adequate treatment, with an unexpected course. They may be found in different stages (eg, early erosions, eschars, or scars). These ulcers normally present with an odd morphology, either with perfectly regular borders or irregular geometric shapes. Peripheral erosions may be clear-cut signs of exogenous damage, such as caustic burn. Although chemical or thermal burns may cause the lesions, they can also be the result of trauma. In some cases, as in this one, the patient will never reveal the mechanism used to produce the ulcer.^4,5

As DA is a diagnosis of exclusion, an exhaustive workup and elliptical incisional skin biopsy for routine histology and special staining to detect microorganisms; and bacterial, fungal, and mycobacterial cultures are essential to rule out other diagnostic possibilities. These lesions are characterized by neither specific laboratory findings nor histopathological findings.² Clinical features, medical history, and patient attitude may be very helpful to support the diagnosis of DA.

The treatment of DA is often unsatisfactory. The patient does not wish to be cured, but looks to receive continuous medical care. Consequently, relapses are commonly observed as the date for discharge approaches. It is essential to design a follow-up plan to avoid reactive recurrences. The dermatologist should be prepared for a prolonged course of treatment and assume failures. Although the referral to a psychiatrist is essential, and follow-up may be multidisciplinary, the patient may reject this advice and look for another doctor. Consoli⁴ suggests treatment of these patients should be based on restructuring the patient’s personality, drug treatment for the psychiatric condition, and medical treatment of the skin lesions. It is essential to establish a supportive and confident approach and avoid initial confrontation and condemnation.

Depending on the underlying psychiatric conditions, different treatments have been suggested, such as relaxation therapy, anxiolytics, antidepressants, and antipsychotics. Considering skin lesions, occlusive dressings and bandages will both promote healing and represent a sign of great diagnostic value. Possible manipulations of the wound by the patient may be recorded and, if they do not occur, the previously recalcitrant ulcer will surprisingly heal. If inpatient treatment is not possible, close follow-up on an outpatient basis is another option.

Chronic cases of DA have a poor prognosis, with continuous relapses and possible suicide attempts. Self-mutilation episodes represent an important risk for infection or iatrogenic complications due to misdiagnosis, as in the case the authors have presented. Close psychiatric and dermatological follow-up care and monitoring in an outpatient setting are essential to prevent relapse. Considering patients with factitious disorder harm themselves to obtain the gratification of the sick role, it has been suggested that allowing them to assume the sick role on an outpatient basis, without having to provide evidence of illness or injury, may reduce morbidity and mortality. Consequently, these regular and frequent physician consultations may reduce both associated risks and costs.

The authors’ experience with this case demonstrates that implementing a monitoring plan, including inpatient treatment, can also be useful.

From the Hospital Infanta Leonor, Madrid, Spain; and Centro de Especialidades Vicente Soldevilla, Hospital Virgen de la Torre, Madrid, Spain

Address correspondence to:
Elena Conde Montero, MD
Hospital Infanta Leonor
Madrid, Spain
elenacondemontero@gmail.com

Disclosure: The authors disclose no financial or other conflicts of interest.