An 18-year-old male patient with a history of developmental delay, autoimmune thrombocytopenia, diffuse diffuse granulomatous pneumonia, phospholipid syndrome, inverse psycho-motor thrombocytopenia, central obesity and granulomatous lymphopenia.
Medicated with alendronate sodium, haloperidol, losartan, lophenoxylic acid, calcium carbonate and oral iron.
She was referred to the urology department due to a progressive increase in the volume of the external genitalia.
She reported widespread and diffuse swelling of the skin, conditioning voiding difficulty, walking and poor hygiene.
She had a history of genital, inguinal or pelvic surgery.
No history of inguinal or pelvic radiotherapy.
She reported having been in associated countries with genital and inguinal erysipelas.
The objective examination showed marked thickening of the skin and tunics of the external genitals, hardened, painless, with ill-defined limits, involving the entire circumference and even penile edema, mimicking more confidence.
Apparently, the dorsal aspect of the skin due to lack of affection was permeated by the process.
The fixation of the tunics was perfectly mobile in relation to the deep planes, such as rigid bodies and glans, which did not present alterations.
Additionally, he presented psoriatic lesions in the internal region of the thighs and peri-umbilical, with important maceration and exudate aspects.
Fixation of inguinal regions showed no evidence of somatic adenopathy.
Lower limbs without edema.
Rectal examination showed no abnormal masses.
No peripheral lymphadenopathy.
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In analytical terms, only a mild thrombocytopenia (123,000/mL) was evident, with no other alteration recorded such as leukocytosis or eosinophilia.
To rule out pelvic or inguinal neoformations/adenopathy, a pelvic CT scan was requested, which did not reveal any abnormality, except for a marked thickening of the skin and genital tunics.
The diagnosis of genital idiopathic lymphedema, in this case praecox lymphedema, given the age of presentation, was made with no antecedents that would guide another type of diagnosis.
Percutaneous cystostomy was performed with the intention of improving circumventing skin deformity and urinary symptoms. The patient underwent antibiotic and antifungal therapy for two weeks.
There was a marked clinical improvement in exudative skin lesions, with no change in the areas affected by the disease.
At that time, the situation was surgically treated.
This consisted of exeresis of all macroscopically affected skin, placement of skin grafts to cover the penis and making neoscrotum with neighboring skin cutaneous flaps.
First, a circumcision was performed, followed by a longitudinal dorsal penile incision of the skin and tunics up to the depth of the fascia of Buck, with circumferential dissection of the entire base of the penis reaching.
Then an arciform incision was made at the level of both inguinal regions, with the base of the penis as the center.
From it were isolated the inguinal cords, exteriorized the testicles and performed inversion of the tunica vaginalis.
Under control of all important structures, the excision of the affected skin was performed.
Once the dorsal decubitus of the skin was not affected due to the apparently unaffected skin (as with the rest is typical in lymphedema situations), it was not removed, thus allowing sufficient anterior rotation to produce the same neoscrotum.
The inguinal incisions and scrotum were closed after the placement of aspiration drains.
To cover the penis, we proceeded to the collection of partial skin skin skin grafts at the thigh level, which were placed on the Buck fascia, in a helix disposition.
The drains were removed on the second postoperative day and the compressive dressing on the fifth day, verifying the total viability of the injected material.
During surgery, marked gelatinous consistency of the tunics was evident, with apparent translucent appearance and marked exudation.
The surgical specimen comprising penile and affected skin totaled 1.05 Kg.
The anatomopathological result showed a hypocellular myxoid tumor with abundant stroma and spindle cells, with absence of cellular atypia and mytosis ectasia, with prominent component of varied caliber vessels.
Immunohistochemistry: vimentin +; CD 34 +; desmin and actin negative.
Conclusion: aspects compatible with aggressive angiomyxoma.
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After 13 months of follow-up, the patient is well, with no clinical evidence of recurrence and satisfied with aesthetics.
