1.A case of pemphigus vulgaris with chronic hepatitis B treated with rituximab in a 42-year-old Filipino male
Jannine A. Galimba ; Jamaine Melisse Cruz-Regalado ; Andrea Marie Bernales-Mendoza ; Vilma C. Ramilo
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):9-9
Pemphigus is a rare, chronic, life-threatening autoimmune blistering disease characterized by blisters and erosions of the skin and mucous membranes. Rituximab has been approved as a first-line treatment for moderate to severe pemphigus vulgaris. Despite of its efficacy in achieving remission, Rituximab can also lead to serious complications such as Hepatitis B reactivation.
We present a case of a 42-year-old Filipino male with severe pemphigus vulgaris on chronic immunosuppressive therapy. He had a 10-month history of multiple bullae and crusted erosions associated with pruritus and burning pain on the mouth resulting to odynophagia and dysphagia. He is a known case of chronic Hepatitis B with unrecalled vaccination status. On physical examination, Nikolsky and Asboe-Hansen signs were positive. Histopathology show intraepidermal split and row of suprabasal keratinocytes pattern. ELISA showed very high levels (>200 RU/ml) for both anti-desmoglein 1 & 3. DIF was positive for IgG & C3. Prior to Rituximab administration, Tenofovir 300 mg/tab/day was started as pre-emptive therapy. To lessen the dependence on systemic corticosteroids, two infusions of Rituximab 1g 2 weeks apart were then given. Notable improvement was seen, evidenced by absence of new bullae, reduction of affected BSA, from 19% to 5.3% and decreased PDAI (78 to 1) and ABSIS (46.5 to 2.75) four months after treatment. Maintained remission and undetectable Hepatitis B viral load 4 months following the last dose of Rituximab were noted, indicating a positive treatment response to both Rituximab and Tenofovir.
Rituximab represents a viable treatment option even for patients with chronic Hepatitis B. Pre-emptive therapy may be done prior to infusion to prevent hepatitis reactivation. Clinical evidence supports the efficacy and safety of Rituximab in this case where preventive measures are taken.
Human ; Male ; Adult: 25-44 Yrs Old ; Hepatitis B ; Pemphigus Vulgaris ; Pemphigus ; Rituximab
2.Double trouble: A case of acute generalized exanthematous pustulosis on a 36-year-old Filipino female with psoriasis vulgaris
Paloma Alexandra rojas ; Maria Rosa Noliza F. Encarnacion ; Andrea Marie Bernales Mendoza ; Vilma C. Ramilo
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):10-11
Over-the-counter medications are readily available and convenient. However, intake may result in cutaneous adverse reactions such as acute generalized exanthematous pustulosis (AGEP). The need to differentiate the disease to similar pustular diseases such as pustular psoriasis and subcorneal pustular dermatosis is essential, to give way to proper patient management. Its appearance with psoriasis vulgaris is uncommon.
We highlight a 36-year-old Filipino female with a known case of psoriasis vulgaris, undergoing phototherapy with good compliance and response, who took one dose of mefenamic acid due to headache. Three days after, she presented with multiple, pin-point pustules surrounded by multiple, erythematous plaques and desquamative scales over the body, including non-psoriatic areas.
A skin punch biopsy on the left arm revealed that the epidermis shows a subcorneal pustule with spongiosis and focal vacuolar alteration at the base. The dermis showed edema and was infiltrated mainly of lymphohistocytes and eosinophils, consistent with acute generalized exanthematous pustulosis.
Treatment with cyclosporine of 3.0 mkd was given, with topical corticosteroids of clobetasol 0.05% ointment mixed with petroleum jelly. Gradual tapering every two weeks was done, with 90% improvement. Blood pressure monitoring was done while on treatment. No recurrence of pustular lesions seen thereafter.
Apart from NSAIDs, beta-lactams, and beta-blockers are common causes of AGEP. There have been few published case reports about concomitant psoriasis vulgaris and acute generalized exanthematous pustulosis. To ascertain the diagnosis among subcorneal pustular dermatosis, pustular psoriasis, acute generalized exanthematous pustulosis, clinical and histopathologic correlation should be done.
Human ; Female ; Adult: 25-44 Yrs Old ; Acute Generalized Exanthematous Pustulosis
3.Acute generalized exanthematous pustulosis overlapping Stevens-Johnson Syndrome in a 53-year-old Filipino female: A case report
Hans Elmund F. Alitin ; Jamaine Melisse Cruz-Regalado ; Andrea Marie Bernales-Mendoza ; Vilma C. Ramilo
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):12-12
Acute generalized exanthematous pustulosis (AGEP) and Stevens-Johnson Syndrome (SJS) are uncommon, severe cutaneous drug eruptions with distinct clinical and histopathological features. AGEP-SJS overlap is a rare and complicated cutaneous drug eruption. Neutrophilia, leukocytosis, and elevated liver enzymes can be seen in these patients. Currently, there are no available dermoscopic studies on AGEP overlapping SJS. The pathophysiology of overlapping drug reaction are mediated by T cells and delayed-type hypersensitivity. Management includes removal of offending drug and giving supportive measures like pain management, moist dressing and fluids.
Human ; Female ; Middle Aged: 45-64 Yrs Old ; Acute Generalized Exanthematous Pustulosis ; Stevens-johnson Syndrome
4.A case of lupus vulgaris masquerading as pyoderma gangrenosum in a 17-year-old Filipino male
Ma. Princess Alloue V. Gonzales ; Frederica Veronica Marquez-Protacio ; Wilsie Salas-Walinsundin ; Andrea Marie Bernales-Mendoza
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):14-14
Cutaneous tuberculosis is a rare form of extrapulmonary tuberculosis seen in 1% of all cases. Lupus vulgaris is one of the most common types of cutaneous tuberculosis. Its cutaneous patterns may mimic other dermatologic conditions such as pyoderma gangrenosum. Clinical, diagnostics and histopathologic correlation is important in diagnosing lupus vulgaris.
This is a case of a 17-year-old Filipino male with multi-drug resistant Pulmonary Tuberculosis who presented with three-months history of erythematous papule that gradually progressed into plaque on the scalp, abdomen and left popliteal area with associated pruritus 5/10. The initial working diagnosis was cutaneous tuberculosis versus pyoderma gangrenosum. Incision biopsy revealed a granulomatous dermatitis surrounded by a dense mixed cell infiltrate of lymphohistiocytes from superficial to mid dermis suggestive of an infectious process. Chest radiograph confirmed pulmonary tuberculosis, interferon gamma detection by enzyme linked immunosorbent assay (Quantiferon TB Gold Plus) and nucleic acid amplification test (GeneXpert TB) further verified the presence of Mycobacterium tuberculosis (MTB). The patient was managed as lupus vulgaris, plaque type and started on second line anti-Koch’s medications. Excellent clinical response was seen after 3 months of treatment.
Lupus vulgaris is a challenging disease and may mimic a myriad of other cutaneous disorders, in this case pyoderma gangrenosum. This case highlights a high index of suspicion, trained clinical eye and multi-specialty care to diagnose and treat complicated cutaneous tuberculosis cases. In geographic locations where MTB is still endemic and drug resistance burdens pose complications in treatment, second line pharmacologic interventions for MTB treatment is a viable option.
Human ; Male ; Adolescent: 13-18 Yrs Old ; Lupus Vulgaris ; Pyoderma Gangrenosum
5.A case of langerhans cell histiocytosis in a 3-year-old Filipino male
Kristine Bernadette D. Cunanan ; Maria Rosa Noliza F. Encarnacion ; Andrea Marie Bernales-Mendoza ; Marie Len Camaclang-Balmores ; Paloma Alexandra Rojas
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):21-21
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia affecting children 2-5 years old. The clinical presentation ranges from self-resolving localized disease to fulminant, fatal disseminated disease. While the most common presentation of LCH are small, translucent crusted papules on the trunk, intertriginous areas, and scalp, it may present as crusted plaques and alopecia. A 3-year-old male presented with a 4-month history of solitary, well-defined, hyperpigmented plaque with yellow-brown crust on the left parieto-occipital area of the scalp measuring 1.5 x 1.5 cm and a solitary, well-defined, hairless patch with areas of erythema on the left parieto-occipital area measuring 5.0 x 6.0 cm. Scalp biopsy revealed diffuse collection of lymphohistiocytes interspersed with distinct kidney bean-shaped cells. CD1a is positive for cells of interest. Skeletal survey revealed lytic lesions involving the skull, thoracic cage, spine, pelvis, and upper and lower extremities. The rest of the physical examination findings revealed lymphadenopathy, crackles, globular abdomen with right and left upper quadrant dullness. The patient had episodes of fever, difficulty of breathing, and abdominal pain. The patient received chemotherapy as multisystem LCH based on prednisone and vinblastine. Following 3 courses of chemotherapy, there is noted hair regrowth and sloughing off of crust.
Human ; Male ; Child Preschool: 2-5 Yrs Old ; Alopecia ; Histiocytosis, Langerhans-cell ; Vinblastine
6.Complete remission of palmoplantar psoriasis through phototherapy and topical steroids: A case report
Ana Dominique L. Españ ; a ; Wilsie Salas-Walinsundin ; Andrea Marie Bernales-Mendoza ; Criselda L. David ; Vilma C. Ramilo
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):23-23
Palmoplantar psoriasis is a rare subtype of psoriasis. It is a chronic, relapsing, inflammatory, immunologically-mediated disease affecting the palms and soles.
This is a case of a 58-year-old female with multiple, well-defined, yellowish plaques on slightly erythematous base with some fissures on bilateral palmoplantar areas affecting 4% of body surface area. On Dermatology Life Quality Index, she scored 14. She was advised biopsy but deferred. The lesions were also tested with potassium hydroxide for fungal elements, yielding positive results. She was initially managed as tinea pedis et manuum with three pulse doses of oral antifungal medication for three months. With minimal improvement, patient finally consented for biopsy, confirming diagnosis of psoriasis. She was subsequently treated with potent topical corticosteroids and narrowband-ultraviolet B localized phototherapy, leading to a complete clearance of lesions after 16 weeks of steroid treatment and 52 sessions of phototherapy. There was no more erythema, plaques and fissures, with affected BSA down to 0% and DLQI score to 1. Three months post-treatment, there is still no recurrence of lesions.
Palmoplantar psoriasis is an uncommon variant of psoriasis featuring hyperkeratotic plaques and fissures limited to the palms and soles with associated significant functional impairment. It can be difficult to diagnose, often mistaken for other diseases, and is typically resistant to treatment with poor long-term remission. Biopsy plays a crucial part in the effective management especially among patients with refractory disease. There are numerous treatment modalities but psychosocial needs are equally important to be addressed.
Human ; Female ; Middle Aged: 45-64 Yrs Old ; Biopsy ; Corticosteroid ; Adrenal Cortex Hormones ; Phototherapy
7.Gene mutations meet targeted therapy: Sirolimus therapy for a case of RAD50 and POLE deficient Klippel-Trenaunay syndrome in a Filipino infant
Hans Elmund F. Alitin ; Wilsie Salas-Walinsundin ; Andrea Marie Bernales-Mendoza ; Jay-v James G. Barit ; Vilma C. Ramilo
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):32-32
Klippel-Trenaunay syndrome (KTS) is a rare slow-flow congenital vascular disorder with an incidence of 1:100,000. 1 , 2 KTS is classically characterized by a clinical triad of capillary malformation, venous malformation, and bony or soft tissue hypertrophy. RAD50 and POLE genes act directly on deoxyribonucleicacid (DNA) and genome stability. Although distinct from the more studiedphosphatidylinositol-4,5-bisphosphate3-kinase catalytic subunit alpha (PIK3CA)gene, RAD50 and POLE genes coexist as a deficient gene in few vascular malformations and papillary thyroid carcinoma (PTC).
This is a case of a 7-month-old Filipino female patient clinically and radiologically diagnosed as KTS presenting with multiple capillary malformations and left limb length-girth discrepancies. Dermoscopy showed various vessel patterns in all affected areas. Soft tissue ultrasound and magnetic resonance imaging/angiography (MRI/MRA) of the left extremities revealed subcutaneous capillary malformations, hypertrophy of the subcutaneous structures and compartment muscles. Strong family history of PTC was elicited and genetic sequencing revealed detected RAD50 and POLE genes. She was treated using the mammalian target of rapamycin inhibitor sirolimus with careful monitoring of trough levels and radiographic tests. A significant outcome one year post-sirolimus revealed no abnormal vessels on ultrasound, a lesser degree of hypertrophy and capillary malformations were no longer appreciated in MRI/MRA of left extremities. Port-wine stains (PWS) and affected limbs showed a decrease in erythema and growth rate during the treatment period.
KTS detected with RAD50 and POLE genes successfully treated with sirolimus with trough-level monitoring. Radiographic evaluation and regular anthropometric assessment remain valuable in the diagnosis and monitoring.
Human ; Female ; Infant: 1-23 Months ; Klippel-trenaunay-weber Syndrome ; Sirolimus
8.A pilot study on the safety and efficacy of Abelmoschus Esculentus (Okra) 5% extract in the treatment of non-bullous impetigo among paediatric patients at Jose R. Reyes Memorial Medical Center, Department of Dermatology.
Andrea Marie Bernales-Mendoza ; Daisy King-Ismael
Journal of the Philippine Medical Association 2021;100(1):80-86
INTRODUCTION
Impetigo Is a common, contagious,
superficial skin infection most commonly
presenting as erythematous crusting papules and
pustules on the face and or extremities. The
pathogens usually implicated in this skin disease
are gram- positive organisms including
Staphylococcus aureus, and less frequently, group
A β-hemolytic Streptococcus pyogenes. In
JRRMMC, Department of Dermatology, this disease
always ranks in the top 1 O most common skin
diseases annually. Treatment options for this
disease include topical and oral antibiotics
depending on the condition's severity. Mupirocin
and Fusidic acid are considered as gold standard in
the treatment of impetigo.
9.Unmasking tumors: A case of a CD30-Negative MycosisFungoides masquerading as Erythema NodosumLeprosumin a filipino male.
Ma. Cristina Georgina T. Paloma ; Andrea Marie Bernales-Mendoza
Journal of the Philippine Medical Association 2023;101(2):29-32
Mycosis Fungoides is the most common type of primary
cutaneous lymphoma. Early in its course, it usually
presents as erythematous patches and plaques similar
to a lot of cutaneous conditions. A 68-year-old male
presented with a 13-year history of multiple
erythematous patches and plaques on the arms and
trunk. The lesions were pruritic and chronically relapsing
over the years, temporarily relieved by topical
corticosteroids. Thereafter, there was onset of multiple
nodules on the trunk, extremities and face. Initial biopsy
was done by a different dermatologist, revealed
Hansen’s Disease, Borderline Borderline spectrum.
Fitefaraco stain was positive but no bacillary index was
given. The patient was subsequently started on
rifamcpicin 600mg/capsule once a day, Ofloxacin
400mg/tablet once a day, and Minocycline
100mg/capsule once a day. The lesions were noted to
worsen, eventually developing ulcerations over the trunk
and extremities prompting referral to our institution. The
biopsy results were as follows: Cutaneous T-Cell
Lymphoma, diffuse cluster of differentiation (CD) 3
staining, focal CD20 staining, and negative FiteFaraco
stain. The histopathologic findings combined with the
clinical presentation led to the diagnosis of Mycosis
Fungoides. He was then referred to medical oncology
for proper staging and definitive management. The
prognosisof Mycosis Fungoides is generally dependent
on the stage as determined by the extent of skin
involvement as well as presence of lymph node invasion
and/or metastasis.
Mycosis Fungoides
;
Lymphoma
;
T-Cell
;
Cutaneous
10.Concordance of acid-fast stain result and histopathologic vs clinical diagnosis of leprosy: A three-year retrospective study in a Tertiary Government Hospital and Sanitarium in the Philippines
John Benjamin B. Gochoco ; Andrea Marie Bernales-Mendoza
Acta Medica Philippina 2024;58(23):42-48
Objectives:
In the Philippines, there has been a lack of information on the concordance between classifications of Hansen’s disease or leprosy clinically, histopathologically, and with AFS results. The study ultimately aimed to determine the concordance between the clinical diagnosis, histopathological results, and AFS results of patients with leprosy seen at the Dr. Jose N. Rodriguez Memorial Hospital and Sanitarium (DJNRMHS).
Methods
This is a descriptive, retrospective, single-center study conducted at the DJNRMHS, a tertiary government hospital and one of the last remaining sanitaria in the country located in northern Metro Manila in the Philippines. The study reviewed and included all the patient records from the years 2017-2019 which included skin biopsy results and slit-skin smear with AFS. Leprosy patients were then classified based on the following classifications: World Health Organization (WHO) and Ridley-Jopling classifications; and the concordance of clinical diagnosis vs the histopathologic findings and clinical diagnosis vs AFS results were determined using kappa testing.
Leprosy
;
Biopsy