Indian J Sex Transm Dis Indian J Sex Transm Dis
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  Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 43  |  Issue : 2  |  Page : 194-195
 

Erythema nodosum leprosum with genital ulceration - A rare and interesting case report


Department of Dermatology, Venereology and Leprosy, Tirunelveli Medical College and Hospital, Tirunelveli, Tamil Nadu, India

Date of Submission07-Apr-2021
Date of Decision06-Apr-2022
Date of Acceptance08-Apr-2022
Date of Web Publication17-Nov-2022

Correspondence Address:
Dr. Palanivel Nirmaladevi
Department of Dermatology, Venereology and Leprosy, Tirunelveli Medical College and Hospital, Tirunelveli - 627 011, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijstd.ijstd_31_21

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   Abstract 

Erythema nodosum leprosum (ENL) is a type III hypersensitivity phenomenon seen in the lepromatous spectrum of leprosy. Involvement of external genitalia is very rare, and genital mucosal involvement is still rare. We report a 39-year-old man with episodic fever, red raised skin lesions predominantly in the lower extremities, and painful genital ulcers for 1-month duration. Genital examination revealed two irregularly shaped, mildly indurated, punched-out tender ulcers with yellowish slough on an erythematous base. Bilateral ulnar and common peroneal nerves were moderately thickened, more on the left side with bilateral stocking type of anesthesia associated with the left side fixed ulnar claw hand and left foot drop. By appropriate investigations and therapeutic elimination, the patient was diagnosed as borderline lepromatous Hansen's disease with type 2 reaction and grade 2 deformity. This case is reported for the rare occurrence of ENL lesions involving the genital mucosa in the form of ulceration.


Keywords: Erythema nodosum leprosum, genital ulcer, glans penis, leprosy, nonvenereal


How to cite this article:
Rupan A, Sivanu S, Nirmaladevi P. Erythema nodosum leprosum with genital ulceration - A rare and interesting case report. Indian J Sex Transm Dis 2022;43:194-5

How to cite this URL:
Rupan A, Sivanu S, Nirmaladevi P. Erythema nodosum leprosum with genital ulceration - A rare and interesting case report. Indian J Sex Transm Dis [serial online] 2022 [cited 2022 Dec 7];43:194-5. Available from: https://www.ijstd.org/text.asp?2022/43/2/194/361316





   Introduction Top


Erythema nodosum leprosum (ENL) is a type III hypersensitivity phenomenon (immune complex reaction involving complement) affecting approximately 50% of patients with lepromatous leprosy (LL) and 10% of borderline lepromatous (BL) leprosy.[1] It classically presents with erythematous evanescent tender papules, nodules, or plaques involving extremities, face, and trunk. Mucosal involvement is uncommon, and its occurrence in the glans penis is extremely rare. Only one case has been reported from New Delhi in 2015 by Kothiwala et al.[2]


   Case Report Top


A 39-year-old married man, farmer by occupation, presented to our skin outpatient department on September 2019, with painful red raised skin lesions over both forearms, thighs, and legs associated with fever for 3 days. He also complained of red skin lesions over the lower extremities and chest, diminished sensation over both hands and feet, and nasal stuffiness for 1 month. The patient developed painful genital ulcers, which started as a small one along with the skin lesions and progressively increased in size to attain the present size. The patient had swelling of both feet for 10 days. He had difficulty in buttoning the shirts and slippage of foot wear while walking for 1 month. There was no history of similar painful nodules or asymptomatic patches in the past. He denied premarital and extramarital sexual contact. Last marital contact was 2 months before the presentation and was unprotected.

On examination, the patient was febrile and had bilateral tender pitting pedal edema. Multiple, well-defined, discrete, scattered erythematous, round-to-oval, scaly dry patches of varying sizes from 1 cm × 1 cm to 6 cm × 5 cm were distributed predominantly over limbs – both thighs, gluteal region, and legs – in a fairly symmetrical pattern with impaired fine touch and pain sensation. Few well-defined, shiny, erythematous, round-to-oval nodules and plaques of varying sizes from 1 cm × 1 cm to 2 cm × 1 cm were also present over extensor aspect of both forearms, medial aspect of the left arm, and right lower chest with intact sensation [Figure 1].
Figure 1: Multiple, well-defined, discrete, scattered erythematous, round-to-oval, scaly dry patches of varying sizes distributed predominantly in the lower extremities - both thighs, gluteal region, and legs - in a fairly symmetrical pattern with impaired fine touch and pain sensation. Irregular, punched-out, multiple, mildly indurated, painful ulcers present over the glans penis. Scanner image (×4) showing multiple nodular collections of inflammatory cells including neutrophils (a). Entire dermis associated with edema and vasodilation (b). Deep dermis and subcutis (c). Periappendageal collection (d). Closer view of perivascular inflammatory cell collection in the upper dermis (×40)

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Genital examination revealed an irregular, punched-out, mildly indurated, tender ulcer of size 1.25 × 0.75 × 0.2 cm with yellowish slough and erythematous borders present over the glans penis near the urethral meatus at 2'o clock position extending to 11'o clock position. Another similar oval ulcer of size 1 cm × 0.6 cm × 0.2 cm was present near the coronal sulcus at 11'o clock position [Figure 1]. On both sides of the groin, single, firm, tender horizontal group of inguinal lymph node of 2 cm diameter was present. Both testes were moderately enlarged and tender. Testicular sensations were normal.

Peripheral nerve examination revealed both ulnar and common peroneal nerves with grade 2 thickening, more on the left side associated with grade 2 tenderness. Posterior tibial nerves were not palpable due to pedal edema, and other peripheral nerves were normal. Sensory examination revealed impaired fine touch and pain sensation over the left ulnar nerve distribution area and bilateral stocking type of anesthesia. Motor examination revealed fixed ulnar claw hand and foot drop both on the left side.

The patient was clinically diagnosed as a case of borderline LL with type 2 lepra reaction and grade 2 deformity. Although the patient denied contact, the genital ulcer was provisionally diagnosed as a case of chancroid. He was empirically treated with tablet erythromycin 500 mg qid for 7 days, while the investigations were going on, but the lesions did not respond.

Gram staining, dark field microscopy, Tzanck smear, and rapid plasma reagin were negative. Treponema pallidum hemagglutination was positive, but fluorescent treponemal antibody absorption was negative. Slit skin smear from both ear lobes and ENL plaque on the right forearm had a bacteriological index (BI) of 4, while that from the genital ulcer and nodule from left forearm had a BI of 3. Histopathology of the ENL nodule from the right forearm showed epidermis with extensive spongiosis, multiple nodular collections of mixed infiltrates including neutrophils in the entire dermis associated with edema and vasodilation, with perineural and periadnexal infiltration by polymorphs, lymphocytes, and histiocytes. The patient was not willing for biopsy from the genital ulcer. Finally, the case was diagnosed as BL Hansen's disease with type 2 reaction and grade 2 deformity with genital ulceration. He was started on multidrug therapy and injection dexamethasone, following which the genital ulcer improved. The patient was not on regular follow-up and stopped taking steroids by himself, and he eventually succumbed to the disease within 2 months.


   Discussion Top


eNL usually presents with skin lesions associated with constitutional symptoms and signs in patients of lepromatous spectrum, and it rarely involves the mucosa.

The genital ulcer in our patient was initially diagnosed as chancroid and was treated with antibiotics. Since there was no improvement and as the patient had ENL lesions in skin, thorough literature search regarding its association with genital ulcer was made. Kumar et al. have reported external genital lesions occurring in 6.6% of leprosy patients, more commonly in BL group in 2001.[3] Kothiwala et al. had reported a similar presentation of genital ulcer as in our case with ENL in 2015. Consequently, we started this patient on steroids following which the genital ulcer reduced in size. It can be easily confused with other genital ulcer diseases, both venereal and nonvenereal.[4] They should be ruled out by necessary investigations before ascribing to ENL. Nonvenereal causes include local manifestations of systemic diseases (Behçet's disease, Crohn's disease, pyoderma gangrenosum), autoimmune blistering diseases (pemphigus, pemphigoid), and drug reactions.[5]

Dermatovenerologists should be aware of genital involvement of ENL, which needs to be differentiated from other conditions that require a different approach and prevent the undue delay in the management. This case is reported for its rare occurrence of ENL involving the genital mucosa as ulceration, mimicking genital ulcer disease, which is the second case reported so far in the literature to the best of our knowledge.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Negera E, Walker SL, Girma S, Doni SN, Tsegaye D, Lambert SM, et al. Clinico-pathological features of erythema nodosum leprosum: A case-control study at ALERT hospital, Ethiopia. PLoS Negl Trop Dis 2017;11:e0006011.  Back to cited text no. 1
    
2.
Kothiwala SK, Khandpur S, Singh MK, Durgapal P. Erythema nodosum leprosum on glans penis: Unusual manifestation of common disease. Int J Dermatol 2015;54:1060-3.  Back to cited text no. 2
    
3.
Kumar B, Kaur I, Rai R, Mandal SK, Sharma VK. Involvement of male genitalia in leprosy. Lepr Rev 2001;72:70-7.  Back to cited text no. 3
    
4.
Roett MA. Genital ulcers: Differential diagnosis and management. Am Fam Physician 2020;101:355-61.  Back to cited text no. 4
    
5.
Huppert JS. Lipschutz ulcers: Evaluation and management of acute genital ulcers in women. Dermatol Ther 2010;23:533-40.  Back to cited text no. 5
    


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