Secretan Syndrome: A Fluctuating Case of Factitious Lymphedema

Comment

In 1901, Henri Francois Secretan, a Swiss physician, reported workmen who had persistent hard swellings on the dorsal aspect of the hands after minor work-related trauma for which they had compensation claims.⁷ In his original report, Secretan did not suggest self-inflicted trauma in the etiology of this disorder.^5,8,9 In 1890, Jean Martin Charcot, a French neurologist, described oedème bleu, a term that is now believed to denote a condition similar to SS.^4-6 Currently, SS is attributed to self-inflicted injury and is considered a form of factitious lymphedema.⁹ As in dermatitis artefacta, most patients with SS are young women, and male patients with the condition tend to be older.^3,8

The mechanism used to provoke this factitious lymphedema might be of traumatic or obstructive nature. Secretan syndrome either is induced by intermittent or constant application of a tourniquet, ligature, cord, elastic bandage, scarf, kerchief, rubber band, or compress around the affected extremity, or by repetitive blunt trauma, force, or skin irritation.^1,4,5,8-10 There was an underlying psychopathology in all reported cases.^1,8,11 Factitious lymphedema is unconsciously motivated and consciously produced.^4,12 The affected patient often is experiencing a serious emotional conflict and is unlikely to be a malingerer, although exaggeration of symptoms may occur, as in our patient.¹² Psychiatric evaluation in SS may uncover neurosis, hysteria, frank psychosis, schizophrenia, masochism, depression, or an abnormal personality disorder.^1,12

Patients with SS present with recurrent or chronic lymphedema, usually affecting the dominant hand.¹ Involvement usually is unilateral; bilateral cases are rare.^3,6 Secretan syndrome is not solely limited to the hands; it also may involve the upper and lower extremities, including the feet.^3,11 There may be a clear line of demarcation, a ring, sulcus, distinct circumferential linear bands of erythema, discoloration, or ecchymoses, separating the normal and lymphedematous parts of the extremity.^{1,4,6,8-10,12} Patients usually attempt to hide the constricted areas from sight.¹ Over time, flexion contractures may develop due to peritendinous fibrosis.⁶ Histopathology displays a hematoma with adhesions to the extensor tendons; a hematoma surrounded by a thickened scar; or changes similar to ganglion tissue with cystic areas of mucin, fibrosis, and myxoid degeneration.^4,6

Factitious lymphedema can only be definitively diagnosed when the patient confesses or is caught self-inflicting the injury. Nevertheless, a diagnosis by exclusion is possible.⁴ Lymphangiography, lymphoscintigraphy, vascular Doppler ultrasonography, and magnetic resonance imaging may be helpful in excluding congenital and acquired causes of lymphedema and venous obstruction.^1,3,9,11 Magnetic resonance imaging may show soft tissue and tendon edema as well as diffuse peritendinous fibrosis extending to the fascia of the dorsal interosseous muscles.^3,4

Factitious lymphedema should be suspected in all patients with recurrent or chronic unilateral lymphedema without an explicable or apparent predisposing factor.^4,11,12 Patients with SS typically visit several hospitals or institutions; see many physicians; and willingly accept, request, and undergo unnecessary extensive, invasive, and costly diagnostic and therapeutic procedures and prolonged hospitalizations.^1,2,5,12 The disorder promptly responds to immobilization and elevation of the limb.^2,4 Plaster casts may prove useful in prevention of compression and thus amelioration of the lymphedema.^1,4,6 Once the diagnosis is confirmed, direct confrontation should be avoided and ideally the patient should be referred for psychiatric evaluation.^1,2,4,5,8,12 If the patient admits self-inflicting behavior, psychotherapy and/or behavior modification therapy along with psychotropic medications may be helpful to relieve emotional and behavioral symptoms.¹² Unfortunately, if the patient denies a self-inflicting role in the occurrence of lymphedema and persists in self-injurious behavior, psychotherapy or psychotropic medications will be futile.⁹