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Nail involvement in Langerhans cell histiocytosis: Diagnostic and prognostic Clues Sirait, Sondang P.; Rihatmadja, Rahadi; Prayogo, Rizky Lendl; Arisanty, Riesye; Melviana, Gisca; Ohara, Kuniaki
Journal of General - Procedural Dermatology & Venereology Indonesia Vol. 4, No. 2
Publisher : UI Scholars Hub

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Introduction: Despite its rarity, nail involvement in Langerhans cell histiocytosis (LCH) may show various clinical presentations. This study aims to show the roles of nail involvement in LCH patients as the diagnostic and prognostic clues.Case illustrations: We presented four cases of multisystem LCH in children which were already confirmed by skin biopsy with various nail abnormalities. We were able to perform nail biopsy in two patients and confirmed the nail involvement. Histopathological examination showed the infiltration of Langerhans cells characterized by indented/reniform nuclei and CD1a expressions. All patients had high-risk organ involvements. Discussion: Langerhans cells may infiltrate the nail bed, proximal nail fold, and nail matrix. Further infiltration may destruct the nail plate. Hypothetically, we suggest that the nail bed as the initial infiltration site of Langerhans cells. The different sites of involvement lead to different clinical presentation. Nail abnormalities may predict a poorer prognosis, as they mostly occur in patients with multisystem disease. Conclusions: Nails should be routinely inspected in the suspicion of LCH. The presence of nail abnormalities in LCH patients may predict a poorer prognosis.
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
Publisher : UI Scholars Hub

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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
Publisher : UI Scholars Hub

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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
Publisher : UI Scholars Hub

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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.
KASUS RECESSIVE DYSTROPHIC EPIDERMOLYSIS BULLOSA-MITIS YANG TERDIAGNOSIS PADA SAAT LANJUT USIA Inadia Putri Chairista; Shannaz Nadia Yusharyahya; Lili Legiawati; Rahadi Rihatmadja; Rinadewi Astriningrum
Media Dermato-Venereologica Indonesiana Vol 50 No 1 (2023): Media Dermato-Venereologica Indonesiana
Publisher : Perhimpunan Dokter Spesialis Kulit dan Kelamin Indonesia (PERDOSKI)

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.33820/mdvi.v50i1.402

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Introduction: Recessive dystrophic epidermolysis bullosa (RDEB) is a rare bullous disease caused by mutations in the COL7A1 gene encoding anchoring fibril collagen that maintains skin integrity. Case: Male, 67-year-old, presented with blisters and wound on various parts of the body, worsening in the recent year. They had been known to easily appear after trauma since. Vesicles-bullae, erosions-excoriations, milia, and atrophic scars were found on the axillae, groins, and limbs, as well as hyperkeratosis and anonychia of the hands and. Histopathological examination showed subepidermal cleft, milia and fibrosis, without immunoglobulin deposits on immunofluorescence. Discussion: Contrary to classical RDEB, blisters in mitis variant do not always cause impairment. The pronounced symptoms in advanced age might be mistaken for epidermolysis bullosa acquisita. Negative immunoglobulin deposition is the diagnostic key, confirming that blister does not result from an autoimmune process, but rather, from trauma to the abnormal skin, more so by flattening of the rete ridges in the elderly. In the absence of definitive therapy, treatment is symptomatic. Conclusion: The diagnosis RDEB-mitis might be missed in geriatric. Correct diagnosis has implication on management because not only does RDEB-mitis require no immunosuppressant, measures to minimize blister formation is more important to save patient from complication.
Microbioma in Aging Skin Nugraheni Pasaribu, Uly Aanda Maria; Rihatmadja, Rahadi; Yusharyahya, Shannaz Nadia; Widaty, Sandra; Legiawati, Lili; Pandjaitan-Sirait, Sondang MHA
Journal La Medihealtico Vol. 5 No. 3 (2024): Journal La Medihealtico
Publisher : Newinera Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37899/journallamedihealtico.v5i3.1315

Abstract

Microbiota is inseparable to the skin. Just as in the gastrointestinal tract, the skin microbiome reflects the health of the organ where it resides. It contributes to skin barrier function and ensures its homeostasis. However relatively stable over time, microbiome composition and number may be influenced by various aging -related changes − dryness, blemish, wrinkles and alteration of sebaceous gland activity impacting the skin ecological conditions − and vice versa. Several generations have been reported to promote the process while others may play a more protective role in this regard.
Steatocystoma multiplex suppurativa: a case report Rainamira, Arlene; Krisnanti, Inge Ade; Rihatmadja, Rahadi; Suprapto, Novita; Surya, Danny
Bali Dermatology Venereology and Aesthetic Journal BDVAJ - Volume 6, Issue 2 (December 2023)
Publisher : Explorer Front

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.51559/rzd0ak48

Abstract

Introduction: Steatocystoma multiplex (SM) suppurativa is an inflammatory variant of SM, a benign hamartomatous disorder of pilosebaceous unit that usually occurs in early adulthood. Treatment responses are often disappointing due to widespread lesions and late diagnosis. This case report aimed to describe a male diagnosed with SM suppurativa to increase the knowledge and management of SM suppurativa. Case: A 23-year-old male came with multiple lumps on his neck, chest, back, and extremities over the last four years. On dermatological examination, yellow to skin-colored papules, nodules, and cysts, 0.3 to 2 cm in diameter, were observed with erythematous-to-hyperpigmented macules and scars over the lesions. Histopathological examination of the lesion showed cysts with pilosebaceous-like lining and sebaceous glands adhered to the cyst’s wall. The patient diagnosed with steatocystoma multiplex SM suppurativa was treated only with a topical antibiotic and corticosteroid. Discussions: Although the histopathological findings showed pathognomonic findings for SM, SM suppurativa was established as the working diagnosis based on the clinical and dermoscopic findings of inflammatory lesions. The biopsy of noninflammatory lesions might cause no sign of inflammation in the histopathological findings. Conclusions: Dermoscopic findings showed a yellow structureless area with diffuse erythematous borders and histopathological findings of a pilosebaceous-like layer with sebaceous glands adhering to the cyst wall and chronic inflammation is the hallmark of SM suppurativa.
VASKULITIS LEUKOSITOKLASTIK ATIPIKAL: KASUS DENGAN PRESENTASI ANULAR MENYERUPAI REAKSI ID PADA TINEA KORPORIS Halim, Melissa; Miranda, Eliza; Rihatmadja, Rahadi; Fitri, Eyleny Meisyah; Andrea, Valerie
Media Dermato-Venereologica Indonesiana Vol 52 No 3 (2025): Media Dermato Venereologica Indonesiana
Publisher : Perhimpunan Dokter Spesialis Kulit dan Kelamin Indonesia (PERDOSKI)

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.33820/mdvi.v52i3.491

Abstract

Pendahuluan: Dermatofitosis (tinea) biasanya ditandai lesi anular atau serpiginosa dengan skuama di tepi. Pada sebagian kasus, infeksi ini dapat memicu erupsi inflamasi sekunder di lokasi jauh dari fokus primer, disebut reaksi id. Salah satu manifestasi jarang sebagai respons imun sekunder adalah vaskulitis leukositoklastik, yaitu peradangan pembuluh darah kecil dengan ciri histopatologis deposit fibrin, kerusakan dinding vaskular, dan debris inti. Kasus: Laki-laki 47 tahun datang dengan bercak dan bintik kemerahan di kedua tungkai bawah sejak satu tahun. Pemeriksaan menunjukkan papul-plak eritematosa hingga hiperpigmentasi multipel, sebagian anular dengan tepi meninggi, dan skuama. Pemeriksaan mikroskopik jamur dari lesi representatif di pedis dekstra memperlihatkan hifa dan artrospora yang sesuai dengan tinea korporis, sedangkan hasil dari pedis sinistra negatif. Setelah empat minggu terapi antijamur, infeksi sembuh, tetapi muncul lesi anular baru pada lutut dan kaki bilateral disertai palpable purpura. Biopsi kulit menunjukkan vaskulitis leukositoklastik. Diskusi: Reaksi id awalnya dipertimbangkan karena memenuhi kriteria diagnosis untuk reaksi id, tetapi lesi tidak menghilang walaupun tinea korporis sembuh. Diagnosis vaskulitis leukositoklastik anular ditegakkan berdasarkan pemeriksaan klinis dan histopatologi. Pasien mendapat kortikosteroid sistemik tapering hingga tercapai remisi. Kesimpulan: Kasus ini menyoroti presentasi jarang vaskulitis leukositoklastik anular yang menyerupai reaksi id pada tinea korporis. Studi lebih lanjut diperlukan untuk memperjelas hubungan antara tinea, reaksi id, dan vaskulitis, serta menentukan terapi optimal.
Diagnosis dan Tata Laksana Hydroa Vacciniforme Reshinta, Reisa; Rahmayunita, Githa; Rihatmadja, Rahadi; Astriningrum, Rinadewi
Cermin Dunia Kedokteran Vol 51 No 5 (2024): Kedokteran Umum
Publisher : PT Kalbe Farma Tbk.

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.55175/cdk.v51i5.902

Abstract

Hydroa vacciniforme (HV) is a rare photodermatoses with the most frequent onset in childhood. The exact pathogenesis of HV is still not known. This disease is a spectrum with almost similar clinical symptoms, but with a very different prognosis. Classic HV has the best prognosis, but affects the quality of life due to scar formation, while hydroa vacciniforme-like lymphoma (HVLL) is the variant with the worst prognosis. HV needs to be considered as a differential diagnosis, especially in childhood-onset photodermatoses. Classical HV must be distinguishes from hydroa vacciniforme-like lymphoproliferative disorders (HVLPD) because it is related to prognosis. There is still no consensus or guidelines on diagnosis and management for HV.