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.A case of mucous membrane pemphigoid in a 73-year-old female
Rommela Angeli Quiampang ; Jamaine Melisse Cruz-Regalado ; Marie Len Camaclang-Balmores ; Vilma C. Ramilo
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):10-10
MMP is a rare blistering disease manifesting with painful erosions or blisters on one or more mucosal surfaces. An estimate of one to two cases per million annually are diagnosed, with females more affected than males.
We present a case of a 73-year-old Filipino female with recurrent multiple mucosal lesions involving the oral mucosa and genitals, ocular symptoms of foreign-body sensation, and erosions on her scalp.
On physical exam, there were multiple well-defined white plaques on an erythematous base on the oral mucosa and the labia minora. There were multiple well-defined erythematous alopecic patches with erosions and milia on the scalp. Histopathology revealed subepidermal split, while DIF findings were consistent with the pemphigoid group. ELISA showed increased anti-BP180, the most common target antigen for MMP. The patient was managed as a case of MMP. Prednisone 25 mg/day was given, which improved her lesions and decreased her MMPDAI score from 44 (moderate) to 10 (mild). She is currently maintained on azathioprine 50 mg/tablet.
Mucous membrane pemphigoid, being rare, is often overlooked as a differential diagnosis. When presented with a patient with predominantly mucosal lesions, it is important to consider MMP to prevent consequences from delayed diagnosis.
Human ; Female ; Aged: 65-79 Yrs Old
3.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
4.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
5.“Born with bubbles” A case of congenital syphilis in a newborn Filipino male
Criselda L. David ; Wilsie Salas-Walinsundin ; Yzabel Vergel de Dios ; Romella Angeli Quiampang ; Sarah Grace Tan-Desierto ; Vilma C. Ramilo
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):19-19
Congenital syphilis is a worldwide public health concern. This occurs when an infected mother transmits the infection to the fetus during pregnancy or at birth.
We present a case of a 6-day-old male, term, born to a mother with secondary syphilis, via normal spontaneous delivery. Upon birth, patient was well and not in cardiorespiratory distress. However, cutaneous examination revealed multiple, well-defined vesicles and pustules on an erythematous background, some topped with erosions and crusts on the scalp, face, extremities, and trunk. Laboratory work-up and imaging were done which revealed congenital syphilis. He was managed with intravenous Penicillin (100,000iu) 160,000 IV for ten days, and wound healing was hastened by use of a coconut-based cellulose wound dressing on the erosions. He was then referred to a multispecialty team to assess and co-manage possible complications. Regular interval follow-up and repeat laboratory tests were advised for observation and for monitoring.
Congenital syphilis is caused by the bacterium Treponema pallidum. Sequelae include preterm birth, low birth weight, skin lesions, bone deformities, hepatosplenomegaly, anemia, and neurological problems. Diagnosis can be made on clinical suspicion combined with Rapid Plasma Reagin (RPR) and Venereal Disease Research Laboratory (VDRL). Aside from Penicillin G, wound care, nutritional build up, and close monitoring of growth and development with regular follow-ups are essential aspects in the management of congenital syphilis. With timely and adequate treatment, infants have a higher likelihood of complete resolution of symptoms, prevention of long-term complications, and improved overall health outcomes.
Human ; Male ; Infant Newborn: First 28 Days After Birth ; Syphilis, Congenital ; Syphilis
6.Shell to success: A journey through rare ostraceous psoriasis in pediatrics and the impact of targeted therapies
May G. Silva ; Wilsie M. Salas-Walinsundin ; Marie Len Camaclang-Balmores ; Criselda L. David ; Vilma C. Ramilo
Journal of the Philippine Dermatological Society 2024;33(Suppl 1):20-21
Psoriasis may manifest as severe hyperkeratotic lesions resembling an oyster shell called ostraceous psoriasis. This type of psoriasis is extremely rare and is often associated with psoriatic arthritis. Psoriatic arthritis is a chronic inflammatory disease of the joints presenting with pain, stiffness, swelling, tenderness, and limited movements. This is a case of a 16-year-old Filipino female presenting with pruritic erythematous plaques topped with thick adherent ostraceous scales associated with bilateral knee and elbow pains. Laboratory tests and biopsy were done. Histopathology is consistent with psoriasiform dermatitis and psoriatic arthritis is established through Early Psoriatic Arthritis (EARP) Screening Questionnaire and Classification Criteria for Psoriatic Arthritis (CASPAR). Patient was started on potent topical corticosteroids and Narrow-band Ultraviolet B (NB-UVB) phototherapy with minimal improvement. Hence, Secukinumab was initiated which showed significant improvement on the skin lesions and joint pains 7 days after the first dose of Secukinumab. Ostraceous psoriasis is rarely found in pediatric population. It is commonly associated with psoriatic arthritis that should be screened during routine consult. Its characteristic firmly adherent thick scales are resistant to topical treatments. One of the therapeutic options is Secukinumab, an IL-17A selective inhibitor targeting the release of proinflammatory cytokines, chemokines and mediators of tissue damage. It demonstrates immediate effect, significant and sustained improvement in the skin lesions, minimal adverse reactions, as well as improved quality of life and physical function. It is therefore a preferred treatment for patients with moderate to severe psoriasis and psoriatic arthritis requiring rapid clearance.
Human ; Female ; Adolescent: 13-18 Yrs Old ; Arthritis, Psoriatic ; Secukinumab
7.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
8.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