Oki Suwarsa

Stevens-Johnson syndrome and toxic epidermal necrolysis in Dr. Hasan Sadikin General Hospital Bandung, Indonesia from 2009-2013

Background Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions (SCAR) with high mortality and have a significant public health impact because of high mortality and morbidity. Objective To describe data the epidemiological features, etiology, and treatment of retrospectively reviewed data of all patients with SJS and TEN. Methods Retrospective study was conducted in patients with SJS and TEN treated from January 1, 2009 to December 31, 2013 in Dr. Hasan Sadikin General Hospital Bandung, Indonesia. Results A total of 57 patients were enrolled in the study. Thirty-nine cases of SJS (21 males and 18 females), 7 cases of SJS overlapping TEN (4 males and 3 females), and 11 cases of TEN (5 males and 6 females) were reported. All cases of SJS and TEN were caused by drugs, such as paracetamol (16.56%), carbamazepine (7%), amoxicillin (5.73%), ibuprofen (4.46%), rifampicin (3.18%), and trihexyphenidyl (3.18%). All cases were treated systemically with corticosteroid alone (100%). Seven from 57 patients (12,28%) died; 5 cases developed sepsis and 2 cases developed respiratory failure. The mortality rate was 7.69% in SJS, 0% in SJS/TEN overlap, and 36.36% in TEN. Conclusion The role of systemic corticosteroids in SJS and TEN are still controversial, but with a prompt and earlier treatment reduces mortality and improves outcomes of SJS and TEN patients.

Reactive perforating leprosy, erythema multiforme-like reactions, sweet's syndrome-like reactions as atypical clinical manifestations of Type 2 leprosy reaction

Type 2 leprosy reactions commonly known as erythema nodosum leprosum, but various clinical manifestations of type 2 leprosy reaction were exist. The highlight of this case series was to report various atypical clinical manifestations of type 2 leprosy reaction such as reactive perforating leprosy, erythema multiforme-like reaction, and sweets syndrome (SS)-like reaction.

Clinical pilot study: Clarithromycin efficacy in multibacillary leprosy therapy

Background: Rifampicin is one of the important components in the multidrug therapy (MDT)-World Health Organization regimen for leprosy. Clarithromycin is one of the alternative therapies of rifampicin. Methods: This clinical pilot study was to compare the efficacy of 2,000 mg clarithromycin to 600 mg rifampicin in combination with dapsone and clofazimine for 3 months in multibacillary (MB) leprosy patients. They were divided into an MDT-MB regimen group that consists of rifampicin-dapsone-clofazimine and clarithromycin-dapsone-clofazimine (CDC) regimen group, each group consisted of seven patients. Results: The morphological index (MI) was reduced insignificantly after 3 months therapy in MDT-MB group (P = 0.248). While in the CDC group, the MI decrement showed a significant result (P = 0.004). The comparison of MI reduction in MDT-MB and CDC groups showed an insignificant difference (P = 0.130). Skin discoloration was occurred in both groups, whereas mild-nausea was presented in the CDC group, in addition, red-colored urine was developed in the MDT-MB group. Conclusion: We concluded that 2,000 mg clarithromycin is as effective as 600 mg rifampicin in combination with dapsone and clofazimine regimen in MB leprosy patients. Hence, clarithromycin can be considered as an alternative therapy for leprosy patients who resistance and/or allergy to rifampicin.

Systemic contact dermatitis due to corrosion of titanium-coated nickel and cobalt bone plate fixation: A case report

Rationale: Corrosion refers to the degradation of a material that occurs following its interaction with other substances in the environment. Corrosion of metallic substances into tissues may lead to inflammatory responses such as systemic contact dermatitis (SCD), a skin condition where an individual who has previously been sensitized to a particular allergen via the cutaneous route will subsequently react to same allergen via the systemic route. This condition occurs following exposure to allergens such as metals, medications, and certain food substances. In recent years, the use of metal plates for internal fixation has become increasingly common in bone fracture repairs. Patient concerns: A 34-year-old Indonesian male presented with systemic erythema with itching 7 days following a bone plate fixation as a management for mandibular fracture. Diagnoses: Physical examination showed pruritic red macules, papules, and scales on almost his entire body, along with facial swelling. The patch test results revealed a positive reaction to nickel and cobalt. Therefore, the patient was diagnosed with SCD. Interventions: The patient was treated with topical and systemic corticosteroids as well as bone plate removal. Outcome: After treatment, the eruption turned brown, the itching was resolved, and there were no facial swelling as well. Lessons: This case report highlights the need to consider the occurrence of SCD in patients following bone plate fixation.

Swan Neck Deformity Mimicking Claw Hand Caused by Arthritis in Leprosy

Swan neck deformity is a hyperextension of the proximal interphalangeal (PIP) joints and flexion of the distal interphalangeal (DIP) joints. Claw hand is a hyperextension of the metacarpal joints and flexion of the PIP joints, accompanied by reduced motor strength. A 23-year-old female, who was released from leprosy treatment, presented with a bend of the second to fifth fingers of both hands. There was hyperextension of the PIP joints and flexion of the DIP joints from the second to fifth fingers of both hands, thickening of the ulnar nerves, and hypoesthesia without motor impairment of the fourth and fifth fingers of both hands. Radiograph examination revealed cupping of the base of the proximal phalangeal joints of the second, third, and fifth fingers of the left hand and of the second and fifth fingers of the right hand. Additionally, narrowing of the metacarpophalangeal joints of the second, third, and fifth fingers of the right hand and sclerosis of the second and fifth fingers of the right hand were also observed. Claw hand is frequently reported in leprosy, while swan neck deformity is frequently reported in rheumatoid arthritis. To our knowledge, this is the first reported case with swan neck deformity caused by arthritis in leprosy.

The Rare Case of Pemphigus Vegetans in Association with Malnutrition Children in the Multidisciplinary Management

Pemphigus vegetans is a clinical variant of pemphigus vulgaris, accounting for 1–2% of all very rare pemphigus cases in children. The involvement of the oral mucosa in this disease is usually accompanied by severe pain that aggravates the patient’s malnourished condition. Conversely, malnutrition may also reduce vulnerability towards autoimmune diseases. Although pemphigus vegetans has never been reported to develop in a child with marasmus before, we encountered a case of pemphigus vegetans in a severely malnourished patient. A 12-year-old boy in marasmic condition presented with painful, clear, fluid-filled blisters, accompanied by erosions, crusts, and vegetative lesions on almost all parts of the body. Histopathological examination of the lesions revealed a suprabasal cleft, and direct immunofluorescence staining showed deposits of immunoglobulin G in the epidermal intracellular spaces. The patient was treated with a multidisciplinary approach, and intravenous corticosteroid was administered for 2 weeks with an appropriate diet. There were significant improvements in the skin lesions and his nutritional status. Although pemphigus vegetans may occur in children with malnutrition, the underlying mechanism for the development of autoimmune diseases in malnutrition remains unclear.

Increased expression of interleukin-17A in the lesional skin indicates increase of serum antibody anti-phenolic glycolipid-I in leprosy patients

Background: In lepromatous type of leprosy, the serum titer of the antibody against phenolic glycolipid (PGL)-I-a-specific antigen in leprosy that is produced by B cell is high. Whereas, the interleukin (IL)-17A expressed by T-helper 17 cells could induce B-cell differentiation. The role of IL-17A in leprosy is still unknown. Hence, this study aimed to assess a correlation between IL-17A expression in the lesional skin and anti-PGL-I immunoglobulin (Ig) M serum levels in leprosy patients. Methods: This study was performed in Leprosy Clinic, Dr. Hasan Sadikin Hospital Bandung, Indonesia, using a cross-sectional analytical study. A punch biopsy obtained from 49 leprosy patients was included through consecutive sampling for measurement of IL-17A expression in the skin by immunohistochemistry scoring (histoscore). Furthermore, the anti-PGL-I IgM level in serum is evaluated by enzyme-linked immunosorbent assay. Results: The IL-17A expressions in the skin biopsies of tuberculoid (TT), borderline tuberculoid (BT), mid-borderline (BB), borderline lepromatous (BL), and lepromatous leprosy (LL) patients using histoscore were 1.00, 2.31, 4.63, 5.06, and 10.14, respectively, and they showed significant differences (P = 0.0001). The titer of anti-PGL-I IgM levels was 89 pg/ml, 555 pg/ml, 1.244 pg/ml, 1.920 pg/ml, and 23.591 pg/ml in TT, BT, BB, BL, and LL patients, respectively, and they showed significant differences (P = 0.005). The results of Rank–Spearman correlation analysis between IL-17A expression in the skin and anti-PGL-I IgM serum levels were as follows: r = 0.767 and P = 0.0001. Conclusion: These results suggested that the increase of IL-17A expression in the lesional skin indicates the increase of anti-PGL-I IgM serum levels in leprosy.

Interleukin-18 correlates with interleukin-4 but not interferon-gamma production in the lymphocyte cultures of atopic dermatitis patients after staphylococcal enterotoxin B stimulation.

BACKGROUND Staphylococcus aureus (S. aureus) triggers exacerbation of atopic dermatitis (AD) and causes chronic inflammation through the action of various proteins such as staphylococcal enterotoxin B (SEB). SEB has a role in activating interleukin (IL)-18, an important regulator of interferon (IFN)-γ and IL-4, in regards to a therapeutic strategy. OBJECTIVE To determine the correlation of IL-18 level with the IL-4 and IFN-γ level in lymphocyte cultures from AD patients following SEB stimulation. METHOD Twenty patients with AD based on the Hanifin and Rajka criteria and 20 healthy subjects as a control group were selected. A 5 ml blood sample from each subject was taken for lymphocyte culture. The culture was stimulated with SEB for two days and the outcomes were assessed by enzyme-linked immunosorbent assays (ELISA) to evaluate the levels of IL-18, IL-4, and IFN-γ. RESULTS In the AD group, the levels of IL-18, IL-4, and IFN-γ in lymphocyte cultures with SEB were significantly increased compared with non-SEB exposed cells (each p<0.001); similar results were found in the control group. The level of IL-18 was significantly elevated in lymphocyte cultures with SEB stimulation in AD vs. control (p<0.05) and without SEB in AD vs. control (p<0.05). Furthermore, IL-18 levels were significantly correlated with IL-4 levels and score atopic dermatitis (SCORAD) values in AD patients with SEB (r=0.41, p<0.05; and r=0.70, p<0.05, respectively); on the in contrary, there was no correlation between IL-18 and IFN-γ levels (p=0.469). CONCLUSIONS Our results suggest that IL-18 is correlated with increased of IL-4 levels in SEB-stimulated AD lymphocyte cultures.