Dystrophic epidermolysis bullosa (DEB) is characterized by blisters that heal with scarring and milia formation, including nail dystrophy, with an incidence of 2.1 per million live births. DEB is derived from defects of the anchoring fibril which results in sublamina densa separation.

We report a one-month old female who presented with vesicles and erosions on bilateral feet at birth. New vesicles and bullae were noted on other areas of the body such as the scalp, ears, anterior trunk, gluteal area, and extremities which spontaneously ruptured leading to erosions and crusts. Nails were thick and yellowish black. Consult was done at a clinic and was given unrecalled antibiotic, antihistamine, and cream for one week. There was no noted improvement hence she was brought to another clinic, diagnosed as staphylococcal scalded skin syndrome, advised admission and subsequently referred to dermatology service. After clinical assessment, findings of cell poor subepidermal blister on histology, linear C3 on DIF, and positive collagen 7 on ELISA, patient was managed as a case of dystrophic epidermolysis bullosa.

Epidermolysis bullosa is an inherited genetic disease with mutation in COL7A1 gene manifesting as vesicles and bullae on trauma prone areas. The diagnosis is based on clinical, histopathology, immunofluorescence, antigen studies, and electron microscopy. Uniquely, immunofluorescence revealed strong linear deposition of C3 at the basement membrane zone which is more suggestive of bullous pemphigoid than epidermolysis bullosa. This case highlights the importance of early diagnosis and proper management of the disease to limit unnecessary intervention.

Human ; Female ; Infant: 1-23 Months ; Epidermolysis Bullosa Dystrophica ; Infant

Vascular malformations are aberrant proliferations of blood vessels that may present at birth or develop subsequently. Capillary malformations (CMs) represent the most common category of vascular malformations. They generally present as localized discoloration, including port-wine stains (PWSs) and telangiectasia. Certain PWSs, especially those affecting the V2 dermatome, may give rise to pyogenic granulomas or other epithelial or mesenchymal hamartomas.

We present a case of a 46-year-old male with a 5-year history of a solitary, progressively enlarging firm papule on an erythematous patch or “birthmark” on his left cheek. Despite infrequent manipulation, there were no reports of bleeding or any associated symptoms, including pain, pruritus, and thrills. The patient sought consultation for cosmetic reasons and wanted to have the lesion removed. Hence, an excision biopsy revealed a well-defined proliferation of mature thick and thin-walled vessels, lined by a single layer of endothelial cells, surrounded by dysmorphic and irregularly arranged vessels consistent with arteriovenous malformation with capillary malformation.

Capillary malformation (CM) is the most common form of vascular malformation. Fifty percent of port-wine stains (PWSs), which is the most common (CM) in children, are in the face area innervated by the second branch of the trigeminal nerve. Arteriovenous malformations manifest in the advanced stages of PWSs and, alongside pyogenic granuloma, represent underreported histological changes inside mature PWSs, especially in the face area innervated by the second branch of the trigeminal nerve (V2).

Human ; Male ; Middle Aged: 45-64 Yrs Old ; Port-wine Stain ; Arteriovenous Malformations

Most medical complications following acute stroke are preventable (such as cardiac events, pneumonia, bed sores and venous thrombosis). This was a study on the frequency of medical complications and their association with key performance indicators. Methods: The study used a cross-sectional baseline audit of stroke care practices. The audit captured details on the nature of the stroke, patient demographics, characteristics of hospital care, and compliance with six key quality indicators in Philippine Academy of Rehabilitation Medicine Clinical Practice Guideline on Stroke Rehabilitation. Patient records were retrospectively consecutively sampled. Results: A total of 1,683 patients were included in the audit which came from 49 hospitals. Medical complications were seen in 182 patients (11.2%). Pneumonia contributed to half the medical complications (50%), followed by respiratory failure (7.7%) and gastrointestinal bleeding (3.8%). Presence of medical complications were associated with in-patient mortality (OR 3.3 (95% CI 2.1-5.3)) and prolonged hospital stay (16.1 ± 20.7 days vs 9.6 + 10.9 days). The best predictor model for pneumonia included variables of not having a swallow screen within the first 24 hours, having a nasogastric tube inserted, not achieving medical stability, not having a stroke unit in the admitting hospital, having suffered a previous stroke and being older. Conclusion: Non-adherence to evidence-based stroke care rehabilitation guidelines contributed significantly to medical complications in an audit of Filipino stroke patients