Leukocytoclastic vasculitis (LCV) is a histopathologic descriptor for a prevalent type of small-vessel vasculitis (SVV) that affects arterioles, capillaries, and postcapillary venules. Although the association between vasculitis and malignancy only accounts for <5% of vasculitis cases, it has been recognized as a true paraneoplastic syndrome in several studies. A 57-year-old Filipino male presented with erythematous, nonblanching macules on his lower extremities, which rapidly progressed to violaceous lesions on his trunk, buttocks, and lower extremities. He also reported significant weight loss, decreased appetite, and vomiting. A skin biopsy confirmed LCV. Initially treated for meningococcemia, his condition did not improve. Abdominal imaging revealed an enlarged heterogeneous liver with retroperitoneal lymphadenopathy and a parenchymal nodule. He was eventually diagnosed with vasculitis secondary to an underlying hepatic malignancy and expired later from multiorgan failure.

Human ; Male ; Middle Aged: 45-64 Yrs Old ; Leukocytoclastic Vasculitis ; Erythema Elevatum Diutinum ; Malignancy ; Neoplasms ; Paraneoplastic Syndromes

Pemphigus foliaceous is a rare autoimmune blistering disease, while psoriasis is a common immune‑mediated inflammatory skin disease. The coexistence of psoriasis and pemphigus foliaceous has rarely been reported. We report a case of a 55‑year‑old Filipino female with an 8‑year history of chronic plaque‑type psoriasis biopsy‑proven. After 5 years, she developed generalized flaccid bullae and crusted erosions over the face, trunk, and extremities, with no mucous membrane involvement. Skin punch biopsy, direct immunofluorescence, and enzyme‑linked immunosorbent assay were consistent with pemphigus foliaceous. The combination of topical corticosteroids and oral methotrexate was selected as the therapeutic approach, leading to a notable improvement in the patient’s condition. This case report underscores the significance of identifying the simultaneous presence of psoriasis alongside autoimmune blistering diseases like pemphigus foliaceous. Examining predisposing and triggering factors, performing re‑biopsy, and further work‑up as the disease evolves may yield more profound insights. Nonetheless, effectively managing this condition poses a significant challenge.
Fluorescent Antibody Technique, Direct ; Methotrexate ; Psoriasis

Bullous pemphigoid (BP) is a rare autoimmune blistering disorder primarily affecting older adults, with limited occurrences in children. BP in children typically manifests as large, tense blisters on the skin, often on flexural areas. It also more often affects the oromucosal areas and the face in children than in adults. Diagnosis involves histopathological examination revealing eosinophilic spongiosis or subepidermal split, immunofluorescence tests highlighting immunoglobulin G (IgG) and C3 depositions, and immunological assays detecting BP180 and BP230 IgG autoantibodies. This report presents two cases of childhood BP (CBP) with atypical immunopathological findings. Clinically, the two cases had generalized plaques and bullae, including the face. The first case exhibited the characteristic linear deposits of IgG and C3 on the basement membrane through direct immunofluorescence (DIF) and revealed negative anti‑BP180 antibodies on enzyme‑linked immunosorbent assay (ELISA). In contrast, the second case showed negative DIF results, despite clinical suspicion, but had positive anti‑BP180 IgG antibodies on ELISA. It is, therefore, crucial to consider the complete clinical presentation of the patient, in conjunction with the histological findings and immunopathologic assessments to diagnose CBP.
Pemphigoid, Bullous

CYLD cutaneous syndrome (CCS) encompasses numerous inherited skin adnexal tumor syndromes, such Brooke–Spiegler Syndrome (BSS), familial cylindromatosis (FC), and multiple familial trichoepitheliomas (MFT). These syndromes arise from germline pathogenic variants in the tumor suppressor gene CYLD that show variable expressivity. With merely 115 cases documented worldwide and a singular reported incidence among Filipinos, they are exceptionally rare. We present two cases of trichoepitheliomas in Filipino patients, encompassing their clinical, histopathological, and genetic findings.

Our first case is of a 22-year-old male presenting with an 8-year history of asymptomatic skin-colored papules on the nasolabial folds. He was initially diagnosed with milia and underwent electrocauterization; however, recurrence was observed shortly thereafter. No family history exhibited similar lesions.

The second case involves a 43-year-old female with a 31-year history of asymptomatic skin-colored papules on the nose that progressively disseminated to her forehead and ears that started to obstruct the patient’s airway, prompting her to seek consultation.

Genetic testing conducted on affected patients and their relatives identified mutations in the CYLD gene. The first case underwent CO2 laser treatment, while the second patient underwent excision with reconstructive surgery.

Given the rarity of these disorders and their diverse clinical manifestations, genetic testing serves as an invaluable instrument for the purpose of accurate diagnosis, proactive disease progression surveillance, and family planning efforts. This can also contribute to a more comprehensive understanding of the syndrome and the development of new therapeutic strategies.

Human ; Female ; Adult: 25-44 Yrs Old

BACKGROUND

Mpox, a zoonotic disease identified in central Africa in 1958, is mainly endemic to the Congo basin and West Africa. Collecting demographic and clinical data is crucial for early recognition and differentiation of skin lesions as the Philippines addresses this public health challenge.

OBJECTIVE

We describe the demographics, clinical profile, and outcomes of suspected and confirmed mpox cases in the Philippines from July to November 2022.

METHODS

This case series reviewed suspected and confirmed mpox cases at the Research Institute for Tropical Medicine from July to November 2022. Clinical data, demographics, and outcomes were collected through retrospective chart reviews and summarized using descriptive statistics.

RESULTS

Fifteen cases were reviewed, including 2 confirmed mpox cases, with an average age of 37.8 years and 80% male. The other symptoms associated with rash at presentation included fever (20%) followed by lymphadenopathy, malaise and chills reported in 1 case each. All patients had pustules and erythematous papules, with 67% showing vesicles. Lesions mainly appeared on the extremities (93%), trunk (60%), and head/neck (47%), evolving into crusts within 1–2 days. Non-confirmed cases were often misdiagnosed as varicella (60%), arthropod bites (20%), or folliculitis (7%).

DISCUSSION

Young to middle-aged males with travel histories were common in suspected and confirmed mpox cases. Lesions typically included papules and pustules on the extremities, evolving into crusts and erosions within 1–2 days. Confirmed cases had umbilicated papules and fever, while PCR-negative cases were diagnosed with local skin conditions. Testing is vital for timely detection and management, especially since mpox resembles other conditions.

Human ; Mpox, Monkeypox ; Zoonoses ; Zoonotic Disease

BACKGROUND

Acne vulgaris is a common skin disorder among adolescents and young adults, with a worldwide prevalence of 9.4%. Both 5% dapsone gel and 5% benzoyl peroxide gel (BPO) have been reported in literature to have antimicrobial action against Cutibacterium acnes.

OBJECTIVE

To compare the efficacy and safety of 5% dapsone gel versus 5% BPO gel in Filipino patients with mild to moderate acne vulgaris in a tertiary hospital.

METHODS

Phase 1 involved compounding 5% dapsone gel and assessing its safety in healthy individuals through a repeated open application test in 30 participants. In phase 2, a double- blinded experimental study compared the efficacy and safety of both groups. Participants were randomized through block design. Efficacy and safety was assessed using the Global Acne Assessment Score (GAAS) and lesion count, and monitoring the adverse effects. The Cardiff Acne Disability Index (CADI) questionnaire was utilized to evaluate the impact on quality of life.

RESULTS

In phase 1, 56.7% of the participants were female and the only reported adverse event was pruritus (13.3%). In phase 2, both the BPO and dapsone group had 35 participants enrolled. Most are female (75.7%) with a mean age of 24. Results showed improvement in the GAAS from moderate to mild, a decrease in inflammatory and non-inflammatory lesions, and a reduction in side effects during the succeeding visits in both groups.

CONCLUSION

5% dapsone gel and 5% BPO were both efficacious and safe for mild to moderate acne management.

Human ; Acne ; Acne Vulgaris ; Dapsone ; Benzoyl Peroxide

Bullous pemphigoid (BP) is the most common autoimmune blistering disease primarily characterized by tense blisters and occasionally with urticarial plaques, affecting the skin and mucous membranes. These are caused by autoantibodies against BP180 and BP230 which target antigens on the basement membrane zone. The diagnosis relies on the integration of clinical, histopathological, immunopathological, and serological findings. The management depends on the clinical extent and severity. We present in this article a literature review and the clinical consensus guidelines of the Immunodermatology Subspecialty Core Group of the Philippine Dermatological Society in the management of BP.
Pemphigoid, Bullous

Introduction: Epidermolysis Bullosa (EB) is a rare genodermatosis characterized by fragility of the skin and mucous membranes, manifested by blistering with little or no trauma. There are three subtypes: EB Simplex, Junctional EB, and Dystrophic EB. Each type of EB has its own specific genetic defect. We report a case of a 13-year-old girl who presented with multiple tense blisters and eroded plaques since birth on the entire body. Case summary: This is a 13-year-old-girl who presented with solitary tense blister on her right thigh three days after birth, which gradually affected the scalp, trunk, and upper and lower extremities, particularly on the trauma prone areas. There was nail dystrophy and multiple brownish dental pits at three years of age. A 4 mm lesional skin punch biopsy showed subepidermal blisters containing fibrin, lymphocytes and few red blood cells. PAS showed basement membrane zone beneath the blister, compatible with EB. Immunofluorescence mapping showed decreased immunofluorescence (+1) on keratin 5/6, (+2) on keratin 14, and absence of immunofluorescence on alpha 6 / beta 4 integrins. Final diagnosis is EB Simplex. Conclusion Early detection is important in managing this case, to detect systemic involvement and provide palliative care. Genetic counseling is recommended for prospective parents who have a family history of any form of epidermolysis bullosa. The prognosis of Inherited EB is very variable and the mortality is usually due to complications of systemic involvement. A multidisciplinary approach in the supportive management of this case is necessary as there is still no cure for this condition.
Fluorescent Antibody Technique ; Integrins

Mastocytosis is an uncommon, sporadic, heterogenous illness resulting from hyperplasia of mast cells. Diffuse cutaneous mastocytosis is the rarest subtype of mastocytosis affecting children, with bullous mastocytosis being its least common variety. Systemic manifestations like nausea, vomiting, bone pain, diarrhea, and central nervous system abnormalities are less common in children than adults. We report a four-month old male who presented with a two-month history of generalized yellowish to tan macules, papules and plaques with peau d'orange texture, with some blisters and erosions on the back, abdomen and scalp. Darier's sign was positive. Baseline laboratory workup were negative for systemic involvement. CD117 and Giemsa staining were positive for mast cells. Based on the clinical findings and histopathologic results, a diagnosis of bullous mastocytosis was made. Treatment included ketotifen drops, mupirocin cream and cetirizine drops, which resulted in flattening of most lesions and resolution of blisters and erosions.

Human ; Male ; Infant ; Blister ; Cetirizine ; Diarrhea ; Hyperplasia ; Ketotifen ; Mast Cells ; Mastocytosis ; Mastocytosis, Cutaneous ; Mupirocin ; Nausea ; Vomiting

Morphea, a localized type of scleroderma, is a rare fibrosing disorder of the skin that presents with a variety of clinical manifestations such as linear morphea, plaque morphea, generalized morphea and other miscellaneous groups. It has an incidence rate of 0.4-2.7 cases per 100,000 people. Generalized morphea is defined as four or more plaques larger than 3cm, and/or involving of two or more anatomical sites. Among pediatric population, 5% of the cases present as generalized morphea. Concomitant vitiligo is found in in 7% of morphea cases. We report a case of generalized morphea in a four-year-old boy who presented with a one-year history of multiple, well-defined, indurated, annular, skin-colored to hyperpigmented plaques with central atrophy on the mid to lower back and left cheek. There was also concurrent two-year history of multiple ill-defined vitiliginous patches on the upper back, upper arms, and elbow.

Human ; Male ; Child Preschool ; Arm ; Atrophy ; Cheek ; Elbow ; Elbow Joint ; Incidence ; Scleroderma, Localized ; Scleroderma, Systemic ; Skin ; Vitiligo