|Year : 2019 | Volume
| Issue : 2 | Page : 196-198
A Case of hansen's disease masquerading as polymorphous light eruption
Vikas Pathania1, Prerna Shankar2, Divya Shelley3, Sukriti Baveja4, Anwita Sinha4
1 Department of Dermatology, Command Hospital (SC), Pune, Maharashtra, India
2 Station Health Organization, Pune, Maharashtra, India
3 Department of Pathology, AFMC, Pune, Maharashtra, India
4 Department of Dermatology, Command Hospital, Pune, Maharashtra, India
|Date of Submission||03-Nov-2018|
|Date of Acceptance||17-Dec-2018|
|Date of Web Publication||07-Oct-2019|
Lt Col (Dr) Vikas Pathania
Command Hospital (SC), Pune - 411 040, Maharashtra
Source of Support: None, Conflict of Interest: None
Hansen's disease is a chronic, relapsing, infective granulomatous skin disease with protean manifestations imitating many benign dermatological conditions. This not only poses a diagnostic dilemma to the treating physician but also often delays the diagnosis and treatment. We report one such case of Hansen's disease imitating a polymorphous light reaction suspected and detected incidentally on the lack of response to treatment of the “apparently benign photodermatoses.”
Keywords: Hansen's disease, photodermatoses, polymorphous light eruption
|How to cite this article:|
Pathania V, Shankar P, Shelley D, Baveja S, Sinha A. A Case of hansen's disease masquerading as polymorphous light eruption. J Mar Med Soc 2019;21:196-8
| Introduction|| |
Leprosy is a chronic granulomatous condition caused by Mycobacterium leprae and spread most likely by airborne route in close contacts. Although the diagnosis of Hansen's disease is often clinical, at least initially, the cardinal signs for confirming the diagnosis include the presence of an anesthetic skin lesion, peripheral nerve enlargement, and/or demonstration of M. leprae in skin smears. However, clinical presentation can more often than not be varied in different spectrum of the disease including lepra reactions and mimic a large variety of lesions from other dermatoses. The problem is further compounded when the clinical presentation does not conform with the cardinal signs, thereby delaying diagnosis and treatment. We report a case of Hansen's disease in a 42-year-old man simulating polymorphous light eruption.
| Case Report|| |
A 42-year-old male, resident of Maharashtra, presented with complaints of red raised itchy rash over the face of 11-month duration. Initially, he noticed a coin-sized red raised lesion which progressed over a period of 6 weeks to involve the entire left cheek. The patient initially resorted to self-medication in the form of application of various topical gels and creams with some transient improvement. Subsequently, over the next 8 months, he developed similar lesions over the right cheek as well. There was no history of numbness in the lesions, light-colored numb patches elsewhere on the body, redness of eyes, and swelling of hands or feet. The lesions were associated with itching and burning sensation with aggravation of symptoms on sun exposure. There was, however, no history of oral ulcers, fever or joint pains, and swelling. The patient reported to a dermatologist where a skin scraping for KOH mount was found negative for dermatophytosis. An initial skin biopsy done during the same review from the patch over the left cheek was descriptive, and following a negative ANA screen, the patient was managed empirically for polymorphous light eruption with topical steroids, sunscreens, and sun-protective measures. However, after an initial symptomatic improvement, the patient offered no relief with medication. On presenting to us, dermatological examination revealed the involvement of both cheeks (left more than right) in the form of multiple erythematous to violaceous, norm esthetic, normotrichic, polysized papules, and plaques coalescing at places to suggest an arcuate morphology, with the largest plaque over the left cheek measuring 12 cm in diameter, extending from the left angle of mandible to the left angle of mouth horizontally and from the left lower eyelid to the upper one-third of the left side of the neck vertically [Figure 1]. There was no nerve to patch or thickened peripheral nerves. Histopathologic examination of a repeat skin biopsy from the patch over the left cheek revealed an atrophic epidermis with the dermis showing numerous periadnexal, perifollicular, and perineural noncaseating epithelioid granulomas with a rich lymphocytic cuff around them with few giant cells but no acid-fast bacilli (AFB[L]) on the modified Ziehl–Neelsen stain and slit-skin smears [Figure 2] and [Figure 3]. Based on histopathology, a diagnosis of Hansen's disease (borderline tuberculoid) was made. The patient was started on three drugs, multidrug therapy (MDT) in the form of tablet dapsone 100 mg daily, capsule clofazimine 50 mg daily, and capsule rifampicin 500 mg and capsule clofazimine 300 mg monthly supervised to which he responded well in the form of subsidence of lesions within 1 month and is currently on the follow-up [Figure 4].
|Figure 1: Multiple erythematous to violaceous, norm esthetic, normotrichic, polysized papules, and plaques over the left cheek|
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|Figure 2: H and E scanner view showing an atrophic epidermis with dermis showing numerous periadnexal and perineural epithelioid granulomas|
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|Figure 3: H and E high-power view (×10) showing noncaseating epithelioid granulomas with a rich lymphocytic cuff around them with few giant cells|
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|Figure 4: Resolution of lesions over the left cheek after 1 month of multidrug therapy|
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| Discussion|| |
Hansen's disease is a chronic relapsing disease infecting the skin and peripheral nerves. Lesions of leprosy can show a wide range of morphologies such as macules (erythematous or hypopigmented), papules, plaques, and nodules. Infiltrative lesions can further cause surface changes in the form of dryness and hypotrichosis by destruction of appendages of the skin. Acute inflammatory lesions and limb swelling can result from reactionary episodes. Polymorphous light eruption is a common, sunlight-induced photodermatoses characterized with pruritic, erythematous papular, plaque, and vesicular lesions occurring over the photoexposed areas within hours of exposure to ultraviolet radiation and complete resolution over few weeks. Our patient presented with slowly progressive erythematous to violaceous papules and plaques with photoaggravable pruritus over the face. The cardinal signs of Hansen's disease require the presence of hypoesthesia, peripheral nerve thickening, and demonstration of AFB in tissue smears, all of which were lacking in this case. Moreover, the uniform presence of pruritus and consistent history of photoaggravation of symptoms was striking enough to throw the diagnostic algorithm off track, at least initially. Although pruritus and burning in lepromatous lesions have been described in the literature, the occurrence of photoaggravable pruritus over the lesion was novel.,, The absence of AFB (L) in skin smears as well as an inconclusive initial histopathology further delayed the diagnosis. Differential diagnosis of erythematous macules includes early morphea, fixed drug eruption, Lyme's disease, pityriasis rosea, roseolar syphilide, seborrheic dermatitis, and tinea incognito while that for papules and plaques include granuloma annulare, granuloma multiforme, lupus vulgaris, psoriasis, sarcoidosis, secondary syphilis, and urticaria., In this case, we had considered the clinical differential diagnosis of tinea faciei/incognito, polymorphous light eruption, facial psoriasis, cutaneous sarcoidosis, lupus miliaris disseminatus faciei, and leprosy. Other similar mimicking conditions described in literature include angioedema, cutaneous lymphomas, erythema multiforme, single plaque lepromatous leprosy, verrucous lesions, nonhealing ulcer, infiltrated linear lesions, and vesiculobullous lesions.,, The novelty of the case is highlighted by the fact that not only did it pose a diagnostic dilemma by virtue of lacking the cardinal signs of leprosy but also that it closely simulated a totally unrelated, fairly common photodermatoses in its clinical behavior. This reemphasizes the importance of keeping a high index of suspicion for Hansen's disease in otherwise benign photodermatoses but unresponsive to routinely prescribed treatment for the condition.
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]