Chromoblastomycosis is a chronic fungal infection of the skin and subcutaneous tissue caused by traumatic (often unnoticed or unremembered) inoculation of pigmented fungi which produce muriform bodies and clinically producing slow growing exophytic lesions. The diagnosis is clenched by biopsy. Usually disease is localized but lymphatic and hematogenous dissemination may occur. It is complicated by secondary bacterial infection and later lymphatic stasis occurs as a sequel to infection.
As per history, our patient developed initial lesion over forehead which later disseminated to axilla and lower abdomen. When he presented to us, he had developed complications of chromoblastomycosis such as erysipelas (over abdominal and groin lesion), lymph stasis and consequently regional elephantiasis.
. reported erysipelas as a frequent complication in patients with genital elephantiasis, as the defense mechanism of the skin is impaired due to chronic lymphatic obstruction and compromised blood supply. Repeated episodes of erysipelas may further increase the lymphatic obstruction and elephantiasis. Such patients benefit from long term penicillin therapy and vaccination against streptococci.[1
Genital elephantiasis occurs due to diverse causes like LGV, Donovanosis, tuberculosis, filariasis in endemic areas. The causative organism of LGV, C. trachomatis is a lymphotropic organism which causes thrombo-lymphangitis, peri-lymphangitis and involvement of the draining lymph nodes. Genital elephantiasis in LGV is seen approximately 1-20 yrs after infection, occurring as a combination of chronic edema, sclerosing fibrosis, and active diffuse lympho-granulomatous infiltration in the subcutaneous tissues. Polymerase chain reaction (PCR) has been useful in identification of L1 to L3 serovars of C. trachomatis. According to a study conducted by Younget al
, genital PCR gives better results than routine culture.[2
In donovanosis, genital elephantiasis occurs as a result of constriction of lymphatics (pseudo elephantiasis). Nucleic acid amplification test offers the highest sensitivity for diagnosis of Donovanosis, but is not commercially available. Demonstration of donovan bodies with bipolar staining in large foamy mononuclear cells from the genital ulcers is the mainstay of diagnosis.
Primary elephantiasis can be differentiated from secondary elephantiasis by early onset in prior and lymphangiography. Nelsonet al
. reported penile and scrotal elephantiasis caused by indolent Chlamydia trachomatis, which responded slowly to treatment with doxycycline.[3
. reported a case of granulomatous lymphangitis in an 11-year-old boy, and proposed of it as important differential diagnosis of chronic idiopathic swelling of the genitalia, in younger individuals. Such cases should be followed closely for co existing or subsequent development of Crohn's disease. The overlap between granulomatous lymphangitis of the genitalia, Crohn's disease and orofacial granulomatosis suggests that granulomatous lymphangitis of the genitalia may represent a forme fruste of Crohn's disease.[4
. reported a case of lupus vulgaris presenting with genital elephantiasis and mimicking LGV. The skin biopsy of the genital lesion and chest X-ray confirmed the diagnosis of tuberculosis.[5
The main treatment modality for chromoblastomycosis is antifungal chemotherapy like itraconazole, terbinafine, flucytosine. However, we treated our patient with fluconazole because of easy availability of drug, and the patient responded well. For genital elephantiasis surgical reduction is needed. Our patient was not willing for surgery. Mc Dougal reported no recurrence after a follow up of six months to 10 years in male patients with non STI related lymphedema treated with reduction procedures.[6