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Journal : Journal of General-Procedural Dermatology

Lucio’s phenomenon: A report on six patients in a tertiary referral hospital in Indonesia Marissa, Melani; Rihatmadja, Rahadi; Surya, Denny; Lim, Henry W; Menaldi, Sri Linuwih
Journal of General - Procedural Dermatology & Venereology Indonesia Vol. 5, No. 1
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Background: Lucio's phenomenon (LP) is a rare variant of leprosy reaction, with the clinical manifestation of “necrotizing erythema.” LP was observed in patients with lepromatous leprosy who have not received or completed the treatment, and it is especially evident in patients with diffuse lepromatous leprosy (DLL), known as Lucio-Latapi’s leprosy. LP occurs due to M. leprae invading the blood vessels, causing endothelial damage that leads to thrombosis, ischemia, infarction, and tissue necrosis. The clinical features of the disease are erythematous lesions that can be accompanied by vesicles or bullae, with ulcers forming scar tissues. Histopathological examination can help establish the diagnosis of LP. Until now, there is still controversy about LP management. Multidrug therapy for multibacillary leprosy (MDT-MB) is the preferred line of treatment. Lucio-Latapi’s leprosy and LP are commonly found in Mexico and Central America, but rarely reported in Indonesia. Case Illustration: We report here the clinical description and development of six patients with LP observed in the tertiary referral hospital in Indonesia over a five-year period from 2013 to 2017. Discussion: All patients were diagnosed using clinical and histopathological examination, and all of them presented with ulceration and vasculitis. They were treated with MDT-MB WHO regimens and systemic corticosteroids. Five patients were alive, and one died due to extensive cutaneous lesions that lead to sepsis. Conclusion: Early diagnosis and prompt institution of multidrug therapy with systemic corticosteroids may improve the prognosis and outcome of LP.
Purpuric lesion in a patient with leprosy: Was it a Lucio’s phenomenon or an epiphenomenon? Menaldi, Sri Linuwih; Marissa, Melani; Chairista, Inadia Putri; Lim, Henry W.; Rihatmadja, Rahadi
Journal of General - Procedural Dermatology & Venereology Indonesia Vol. 5, No. 1
Publisher : UI Scholars Hub

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Background: Lucio’s phenomenon (LP) is a severe necrotizing cutaneous reaction that occurs in patients with Lucio’s leprosy. The exact pathomechanism is not fully understood, but typically abundant acid-fast bacilli in the walls of blood vessels point to direct perturbation of blood supply to the epidermis. We report a case of LP where epidermal necrosis occurred in the absence of vascular invasion by mycobacteria, raising the question whether this was an epiphenomenon or a true LP. Case Illustration: A 34-year-old male was referred for an episodic swelling of his hands and feet that persisted for two years. There were signs of leprosy (diffuse shiny skin lesions, anesthesia, and anhidrosis of the extremities) with purpuric patches on lower extremities. The slit-skin smear test revealed a high index of acid- fast bacilli. Histopathological examination revealed epidermal necrosis and leukocytoclastic vasculitis without demonstrable bacillus. WHO multidrug regimen for leprosy and corticosteroids successfully cured the patient and prevented ulcer development. Discussion: Despite the presence of classical LP characteristics clinically and histologically, mycobacterium was absent in the vessels’ walls. We hypothesized that, in LP, vascular impairment might also be secondary due to antigen–antibody reaction and hypercoagulable state.Conclusion: Purpuric patches in Lucio’s leprosy might be the first sign of skin necrosis or vascular damage (purpura and ulceration). Besides the antimycobacterial drugs, anti-inflammatory drugs should be administered. Laboratory test for hemostasis might be advised.
Generalized pustular psoriasis and Cushing’s syndrome in childhood due to corticosteroids misuse Azizah, Fitri; Rahman, Yusnita; Rihatmadja, Rahadi; Agustin, Triana; Astriningrum, Rinadewi; Rahmayunita, Githa
Journal of General - Procedural Dermatology & Venereology Indonesia Vol. 5, No. 3
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Background: Generalized pustular psoriasis (GPP) is characterized clinically by yellowish sterile pustules surrounded by erythema. GPP in children is very rare and may appear following steroid withdrawal in psoriasis vulgaris.Case Illustration: A 14-year-old female presented to the emergency unit with generalized erythematous plaques and multiple pustules with lake of pus after abruptly stopped oral and topical corticosteroid for two weeks. The patient had used topical and oral corticosteroids for almost a year to treat psoriasis. Physical examination revealed moderately ill and tachycardic, with short stature, moon face, amenorrhea, and multiple erythematous plaques with multiple pustules and lake of pus along with red-purple striae all over the body.Discussion: Adverse effects of steroid-induced adrenal insufficiency are time-dependent and generally reversible. Gradual discontinuation of steroids must adhere to physiological dose of steroids, mostly in 3.49 ± 2.92 months. Other treatments available are emollients, salicylic acid, calcipotriol, coal tar, topical steroids, phototherapy, retinoids, and cyclosporine. Topical steroids cannot be the main therapy in severe psoriasis due to the possible side effects of excessive usage. Conclusion: Misuse of topical and systemic steroid in children could lead to Cushing’s syndrome, a multisystem disorder resulting from prolonged exposure to excess glucocorticoids, either both systemic or topical. Children could easily develop systemic reactions to topical steroids due to their higher total body surface area to body weight ratio.
Comparison between oral pentoxifylline + corticosteroid andoral corticosteroid alone for severe erythema nodosum leprosum Martinus, Martinus; Rihatmadja, Rahadi; Menaldi, Sri Linuwih
Journal of General - Procedural Dermatology & Venereology Indonesia Vol. 6, No. 1
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Background: Severe erythema nodosum leprosum (ENL) is common but difficult to treat. Long term use of systemic corticosteroid causes side effects. This study compares the use of a combination of pentoxifylline + oral corticosteroids with a single oral corticosteroid in leprosy patients with severe ENL reactions. Parameters measured include skin RSA score, systemic RSA, total corticosteroid dose, resolution time of skin lesions, improvement of pain VAS and treatment side effects.Methods: 29 subjects with severe ENL reactions are allocated randomly into two groups which receive oral pentoxifylline + methylprednisolone, and oral placebo + methylprednisolone for 12 weeks. The starting dose of pentoxifylline are 400 mg thrice daily for 4 weeks, tapered to 400 mg daily every 4 weeks. Methylprednisolone is based on WHO guideline. Results: In the end, the median of cutaneous RSA score in pentoxifylline vs placebo group is 4 (0-5) vs 3 (0-5). The median of systemic RSA score in pentoxifylline vs placebo group is 0 (0-6) vs 0 (0-5). The median of total corticosteroid doses in pentoxifylline vs placebo group is 156 (120-200) mg vs 136 (96-200) mg. The median of resolution time in pentoxifylline vs placebo group is 6 (0-12) weeks vs 6 (0-12) weeks. The median of change of pain VAS score in pentoxifylline vs placebo group is 5 (0-6) vs 3 (-3-6). No statistically significant difference (p>0,05) are found in all parameters, including side effects. Conclusion: Combination of oral pentoxifylline + corticosteroid is not proven to be more effective. Both are safe.