1.A case of pemphigus foliaceus in a 40-year-old female successfully treated with doxycycline and prednisone combination therapy
Jobelline Mae C. Fernandez ; Mae Ramirez-Quizon
Journal of the Philippine Medical Association 2024;102(2):77-80
Here, we present a 40-year-old
female with multiple pruritic occasionally
painful vesicles, papules, and plaques in a
circinate pattern on seborrheic areas, progressing
to erosions and scales. Clinical findings led to the
diagnosis of pemphigus foliaceus (PF). Initial
treatment with prednisone and clobetasol
ointment, however, did not fully suppress blister
formation and healing of erosions. Skin punch
biopsy revealed a subcorneal split and
intracorneal neutrophilic infiltrates, while enzymelinked immunoassay (ELISA) revealed elevated
anti-desmoglein 1 (Dsgl), consistent with PF.
Doxycycline was then added to the previous
regimen, resulting in remission. We discuss the
role of doxycycline as a cost-effective adjunctive
treatment in patients with refractory PF.
Pemphigus
;
Clobetasol
;
Enzyme-Linked Immunosorbent Assay
2.Bejeweled: Chronic bullous disease of childhood in a 2-year old treated with colchicine: A case report.
Danielle Nicolle Dionisio Mejia ; Mae Ramirez-Quizon
Journal of the Philippine Medical Association 2019;97(2):47-51
Linear IgA bullous dermatosis, also known as chronic bullous disease of childhood when present in the pediatric
age group, is a rare blistering disease more predominantly seen in females less than five years old. This case describes a 2-year old girl who presented with scattered, tense vesicles and bullae on an erythematous base forming
the classic “cluster of jewels” appearance. This clinical picture is often mistaken as bullous impetigo, commonly
seen in children, delaying diagnosis and prompt treatment. Histopathologic examination showed subepidermal
blistering with a predominantly neutrophilic inflammatory infiltrate. The direct immunofluorescence studies revealed a linear band of IgA deposition in the basement membrane zone consistent with the diagnosis of CBDC.
The patient was started on colchicine and oral prednisone at 1 mg/kg/day and complete resolution was achieved
within two weeks of therapy.
Colchicine
3.Epidermolysis Bullosa Pruriginosa in a 12-year-old male: A case report
Agustin Miguel P. Soriano ; Mae Ramirez-Quizon
Journal of the Philippine Medical Association 2024;102(2):81-88
Introduction:
Epidermolysis Bullosa Pruriginosa (EBP) is a rare subtype of the inherited Dystrophic ~ Epidermolysis Bullosa spectrum of diseases and results from a gene mutation in COL7AL Though predominantly an autosomal dominant disease, autosomal recessive and even sporadic have been reported.
Case Summary:
Case Summary:We report a case of a 12-year-old Filipino male presenting with a chronic history of numerous scratching-induced blisters predominantly distributed on the extensor aspect of his arms and legs without concomitant oral lesions, nail dystrophy, or hair findings, and without a family history of similar lesions. Histopathologic assessment, Direct Immunofluorescence (DIF), and Indirect Immunofiuorescence (IIF) showed a subepidermal split with scant inflammatory infiltrates, no immunofluorescence, and absent userrated linear immunofluorescence at the dermal-side of the Salt Split Skin slide, respectively, which were all consistent with EBP. Enzyme-Linked Immunosorbent Assay (ELISA) for Anti-Collagen VII antibodies was slightly elevated, which may suggest an alternative diagnosis of Epidermolysis Bullosa Acquisita (EBA). This slight elevation may be due to the mutated Collagen Vil protein becoming antigenic and therefore provoking an immune response. To conclusively distinguish EBP from EBA, a COL7AI gene mutation analysis was recommended. With a diagnosis of EBP cannot totally rule out EBA, the patient was initially managed with dapsone monotherapy, counseled regarding behavioral modification to reduce scratching and trauma, advised wound care and close monitoring for the development of oropharyngeal lesions, and recommended for COL7A1 genetic mutation analysis.
Conclusion
This report demonstrates a case of EBP
with elevated Anti-Collagen VII antibodies. The
diistinction between EBP and EBA is important
because this changes the management: EBP is
largely supportive, while EBA may benefit from
immunosuppressive therapy.
Epidermolysis Bullosa Pruriginosa
;
Enzyme-Linked Immunosorbent Assay
;
Epidermolysis Bullosa Acquisita
4.Present tense: A peculiar case of Pemphigus Vulgaris presenting with tense blisters during the COVID-19 pandemic
Dianne Katherine R. Salazar-Paras ; Mae N. Ramirez-Quizon
Acta Medica Philippina 2021;55(5):569-573
Pemphigus vulgaris is an autoimmune bullous dermatosis presenting with flaccid blisters and erosions. The morphology of pemphigus reflects the more superficial intraepidermal level of split seen histologically in contrast with pemphigoid, where the level of split is deep below the epidermis. This is a case of a 58-year-old male clinically presenting with arcuate tense bullae, which are more characteristic of the pemphigoid group of disorders, which revealed an intraepidermal split and tombstoning pattern of the basal epidermis on histopathology. Direct immunofluorescence revealed intercellular IgG and C3 distribution. Although this patient presented clinically with tense bullae, the histopathology and direct immunofluorescence results were consistent with pemphigus vulgaris.
Pemphigus
;
Fluorescent Antibody Technique, Direct
5.Spot the difference: A case of Hailey-Hailey Disease in a 64-year-old Filipino female
Raisa Celine R. Rosete ; Juan Paolo David S. Villena ; Mae N. Ramirez-Quizon
Acta Medica Philippina 2021;55(5):563-568
Hailey-Hailey disease (HHD) is an uncommon acantholytic disorder of the skin. This is a case of a 64-year-old Filipino female with a chronic history of painful and malodorous intertriginous plaques. Histopathologic evaluation showed overlapping features of pemphigus vulgaris and Hailey-Hailey disease. A negative direct immunofluorescence test clinched the diagnosis of Hailey-Hailey disease. The patient was advised regarding preventive measures and treated with topical antibiotics and corticosteroids with improvement of her lesions.
Pemphigus, Benign Familial
;
Fluorescent Antibody Technique, Direct
6.Bullous henoch-schonlein purpura in 9-year old Filipino male: A case report.
Camille Clarisse S. Mundin ; Christene Pearl F. Arandia ; Mae Ramirez-Quizon
Journal of the Philippine Medical Association 2021;99(2):42-45
Henoch- Schonlein purpura (lgA vasculitis)
is the most common vasculitis in the pediatric
population. It usually affects the skin, synovia,
gastrointestinal tract, and kidneys. It usually
presents as a palpable purpura. The occurrence of
hemorrhagic bullae in children with HSP is an
uncommon presentation. We present a case of an
otherwise healthy 9-year-old male with a three-day
history of erythematous maculopapular lesions over
the lower extremItIes which progressed to
violaceous plaques with central hemorrhagic bullae
affecting the bilateral lower extremities, buttocks
and arms. Odynophagia and intermittent abdominal
pain were present. Histopathology revealed small
vessel leukocytoclastic vasculitis and direct
immunofluorescence (DIF) showed granular
deposition of lgA and fibrinogen along the walls
of the papillary dermal blood vessels. The patient
was successfully treated with prednisone at
1 mg/kg/day and showed resolution of lesions
within 1 week of treatment with no recurrence at 1
month follow-up. We stress the importance of
having a high index of suspicion in these atypical
presentations in order to prevent delay in diagnosis
and achieve maximal treatment gains.
7.Bullous Mastocytosis in a Filipino infant: A case study
Blythe N. Ke ; Shahara Abalos-Babaran ; Jay-V James G. Barit ; Mia Katrina R. Gervacio ; Mae N. Ramirez-Quizon
Acta Medica Philippina 2021;55(5):587-591
Introduction:
Mastocytosis is a disease defined by the proliferation of mast cells in organs, most commonly the skin. It may affect any age group but is usually found in children in the first year of life. We present a case of diffuse cutaneous mastocytosis manifesting in the rare bullous form.
Case:
A 4-month-old Filipino male presented with multiple bullae on the head, trunk, and extremities after applying chamomile oil. Biopsy of the skin demonstrated numerous mast cells, confirming the diagnosis of bullous mastocytosis. The patient was treated with oral antihistamines and corticosteroids, which was followed by a good response.
Conclusion
Diagnosis of diffuse cutaneous mastocytosis may be challenging due to its rarity. Proper management requires preventive measures, symptomatic treatment, as well as communication of prognosis with the stakeholders.
Mastocytosis, Cutaneous
8.Subungual squamous cell carcinoma of the great toe presenting as a pyogenic granuloma-like mass in a 64-year-old Filipino male: A case report.
Sher Claranza O. LIQUIDO ; Bernice C. NAVARRO ; Tanya Angela Perez CHUA ; Mae Ramirez QUIZON
Journal of the Philippine Dermatological Society 2022;31(1):46-49
INTRODUCTION: Subungual squamous cell carcinoma is rare, though it is the most common primary malignant neoplasm in the nail unit. Fingernails are more commonly involved than toenails with nonspecific and mild features. Histopathologic presentation may be difficult to distinguish from other tumors. With this, there is often a delay in diagnosis.
CASE REPORT: A 64-year-old male presented with a subungual yellowish granulomatous plaque, eventual dystrophy, and persistent bleeding on the fi rst digit of the right foot of two years' duration. Initially diagnosed as pyogenic granuloma through skin punch biopsy, debridement with ungiectomy was done. Upon recurrence, he underwent wide excision with matricectomy, wherein deeper sections revealed features of basosquamous carcinoma. A positive Epithelial Membrane Antigen and negative BerEP4 staining later confirmed a diagnosis of SCC. Since bone involvement was repeatedly suspected in magnetic resonance imaging after postoperative radiotherapy, amputation was eventually done.
CONCLUSION: We report a case of subungual SCC initially diagnosed as a pyogenic granuloma. Full-thickness biopsy should be done in persistent nail conditions using special stains to confirm the diagnosis. Surgical treatment or radiotherapy with or without systemic therapy is the first line of treatment for subungual SCC. In cases of bone involvement, amputation may be warranted.
KEYWORDS: nail, pyogenic granuloma, squamous cell carcinoma, tumor
Nails ; Granuloma, Pyogenic ; Carcinoma, Squamous Cell ; Neoplasms
9.A literature review and clinical consensus guidelines on the management of Bullous Pemphigoid
Clarisse G. Mendoza ; Josef Symon S. Concha ; Cybill Dianne C. Uy ; Bryan K. Guevara ; Evelyn R. Gonzaga ; Maria Jasmin J. Jamora ; Jamaine L. Cruz‑Regalado ; Katrina C. Estrella ; Melanie Joy D. Ruiz ; Rogelio A. Balagat ; Mae N. Ramirez‑Quizon ; Johanna Pauline L. Dizon ; Marie Eleanore O. Nicolas
Journal of the Philippine Dermatological Society 2023;32(2):63-76
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