Secretan Syndrome: A Fluctuating Case of Factitious Lymphedema
Secretan syndrome (SS) is a recurrent or chronic form of factitious lymphedema that usually affects the dorsal aspect of the hand and is accepted as a subtype of Munchausen syndrome. Secretan syndrome usually is induced by compression of the extremity by tourniquets, ligatures, cords, or similar equipment. This unconsciously motivated and consciously produced lymphedema is an expression of underlying psychiatric disease. Herein, we present a fluctuating case of SS involving the hand, with periods of severe lymphedema and those of complete amelioration, parallel to stressful and peaceful periods in the patient’s personal life. We briefly review the current literature on SS to increase awareness among dermatologists, and we present information on the clinical portrait, diagnosis, and appropriate management of this peculiar and underreported disorder.
Practice Points
- Secretan syndrome is a recurrent or chronic form of factitious lymphedema that usually affects the dorsal aspect of the hand; it is accepted as a subtype of Munchausen syndrome.
- Secretan syndrome usually is induced by compression of the extremity by tourniquets, ligatures, cords, or similar equipment.
- This unconsciously motivated and consciously produced lymphedema is an expression of underlying psychiatric disease.
Secretan syndrome (SS) represents a recurrent or chronic form of factitious lymphedema, usually affecting the dorsal aspect of the hand.1-3 It is accepted as a subtype of Munchausen syndrome whereby the patient self-inflicts and simulates lymphedema.1,2 Historically, many of the cases reported with the term Charcot’s oedème bleu are now believed to represent clinical variants of SS.4-6
Case Report
A 38-year-old Turkish woman presented with progressive swelling of the right hand of 2 years’ duration that had caused difficulty in manual work and reduction in manual dexterity. She previously had sought medical treatment for this condition by visiting several hospitals. According to her medical record, the following laboratory or radiologic tests had revealed negative or normal findings, except for obvious soft-tissue edema: bacterial and fungal cultures, plain radiography, Doppler ultrasonography, lymphoscintigraphy, magnetic resonance imaging, fine needle aspiration, and punch biopsy. Reflex sympathetic dystrophy, compartment syndrome, filariasis, tuberculosis, and lymphatic and venous obstruction were all excluded by appropriate testing. Our patient was in good health prior to onset of this disorder, and her medical history was unremarkable. There was no family history of a similar condition.
Dermatologic examination revealed brawny, soft, pitting edema; erythema; and crusts affecting the dorsal aspect of the right hand and proximal parts of the fingers (Figure 1). The yellow discoloration of the skin and nails was attributed to potassium permanganate wet dressings. Under an elastic bandage at the wrist, which the patient unrolled herself, a sharp line of demarcation was evident, separating the lymphedematous and normal parts of the arm. There was no axillary lymphadenopathy.
The patient’s affect was discordant to the manifestation of the cutaneous findings. She wanted to show every physician in the department how swollen her hand was and seemed to be happy with this condition. Although she displayed no signs of disturbance when the affected extremity was touched or handled, she reported severe pain and tenderness as well as difficulty in housework. She noted that she normally resided in a city and that the swelling had started at the time she had relocated to a rural village to take care of her bedridden mother-in-law. She was under an intensive workload in the village, and the condition of the hand was impeding manual work.
Factitious lymphedema was considered, and hospitalization was recommended. The patient was then lost to follow-up; however, one of her relatives noted that the patient had returned to the city. When she presented again 1 year later, almost all physical signs had disappeared (Figure 2), and a psychiatric referral was recommended. A Minnesota Multiphasic Personality Inventory test yielded an invalid result due to the patient’s exaggeration of her preexisting physical symptoms. Further psychiatric workup was rejected by the patient.
Almost a year after the psychiatric referral, the patient’s follow-up photographs revealed that the lymphedema recurred when she went to visit her mother-in-law in the rural village and that it was completely ameliorated when she returned to the city. Thus, a positive “mother-in-law provocation test” was accepted as final proof of the self-inflicted nature of the condition.
