Melanosis ; Vitiligo

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

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

BACKGROUND: Mosquito bite is a common dermatological complaint with various treatments but with no gold standard treatment regimen. OBJECTIVE: To compare the safety and efficacy of 0.025% capsaicin cream against 1% hydrocortisone cream and placebo as anti-inflammatory and anti-pruritic preparation for mosquito bites. METHODS: Seventy-five volunteers were enrolled in the placebo-controlled trial and randomized into three treatment groups. They were exposed to laboratory-reared mosquitoes to incur bites and to apply the designated cream. Anti-inflammatory and anti-pruritic effects were determined by mean lesion size, physician's global assessment, pruritus intensity score and 100mm visual analog scale. RESULTS: The mean lesion size and physician's global assessment, pruritus intensity score and visual analog scale showed no significant difference between 0.025% capsaicin and 1% hydrocortisone and they were both found to be superior to placebo (p-value< 0.001 using repeated measures of ANOVA; Kruskal-Wallis test). CONCLUSION: 0.025% capsaicin is comparable to 1% hydrocortisone as an anti-inflammatory and anti-pruritic preparation for mosquito bites.

Human ; Animal ; Male ; Female ; Analysis Of Variance ; Capsaicin ; Cortisone ; Culicidae ; Hydrocortisone ; Insect Bites And Stings ; Pruritus ; Visual Analog Scale ; Volunteers

COVID-19 Vaccines ; Biological Therapy ; Immunotherapy

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

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

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