Impetigo herpetiformis in a 33-year-old Filipino female

@#Introduction: Impetigo herpetiformis is a rare pustular disorder that affects pregnant women. It is also otherwise termed as “pustular psoriasis of pregnancy”, owing to the fact that the pustules are sterile and are not associated with a viral etiology. The classic lesions are erythematous patches or plaques with margins studded with subcorneal pustules spreading centrifugally. A cardinal feature of this disorder is the rapid resolution of lesions after delivery. Case Summary: This is a case report of a 33-year-old female, gravida 3, para 2 (2-0-0-2) at 36 weeks age of gestation who presented with one week history of multiple well defined irregularly shaped erythematous annular patches and plaques with marginal pustules on the trunk and extremities. The lesions began on the trunk spreading centrifugally, sparing the face, palms, soles and mucus membrane. Biopsy showed scale crust in the stratum corneum, the epidermis showed acanthosis, spongiosis and neutrophilic microabscesses with focal vacuolar alteration and small sub-epidermal nests on the basal cell layer. The dermis revealed dilated blood vessels and mild superficial perivascular lymphocytic infiltrates. Patient was given Prednisone and Cetirizine with noted resolution of lesions. Conclusion: Recurrences of impetigo herpetiformis in subsequent pregnancies are common with earlier onset in gestation and are characteristically more severe. The complications are placental insufficiency, stillbirth or neonatal death. Early detection is of utmost importance. Management must be multidisciplinary involving a dermatologist, obstetrician and pediatrician.

Dermatitis herpetiformis-like Linear IgA bullous disease in a Filipino

@#INTRODUCTION: Linear IgA bullous disease (LABD) is a rare autoimmune blistering disease characterized by subepithelial bullae and linear IgA deposition along the basement membrane zone of the epidermis. Lesions present as pruritic papulovesicles and tense bullae which may coalesce forming annular or polycyclic urticarial plaques with blistering on the edge of the lesions forming the classic “string of pearls” sign. Lesions may affect the face, trunk, and extensor extremities. Incidence rates range from 0.5 to 2.3 cases per million individuals per year. Due to its rare occurrence, there are only a fewdocumented reports on cases of LABD, particularly in the Filipino population. CASE REPORT: A 33 year-old Filipino female consulted because of a 3-week history of severely pruritic vesicles and crusts on the face, trunk, and arms. Patient noted no gastrointestinal symptoms on consultation. Skin punch biopsy revealed subepidermal blisters with collection of neutrophils at the dermal papillae. Direct immunofluorescence showed strong (+2) deposits of linear IgA at the dermo- epidermal junction in perilesional skin thus validating the diagnosis. The patient’s serum was negative for IgA anti-tissue transglutaminase and IgA antiendomysial antibodies. Patient was treated with topical corticosteroids and Dapsone 50 mgs OD with remarkable improvement. CONCLUSION: Linear IgA bullous disease has very few reported cases especially in the Philippine setting. Dapsone is considered the first-line treatment. The disease may persist for a decade or longer, and relapses may occur. Careful history-taking accompanied by the histological, immunofluorescence, and serological findings must be done to ensure proper treatment and good prognosis.

Epidermolysis Bullosa Simplex in a 13-year-old Filipina

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.

A case of disseminated granuloma annulare successfully treated with acitretin and narrowband UV-B phototherapy

Introduction@#Granuloma annulare(GA) is a benign, inflammatory skin condition characterized by asymptomatic, flesh colored or erythematous papules. The disseminated variant of granuloma annulare is uncommon, usually affecting women older than 40 years.@*Case Summary@#We report a case of a 52-year-old female, who presented with a 4-year history of skin colored to erythematous papules and annular plaques covering the entire skin surface. Correlation of the clinical presentation, histopathology and dermoscopy findings established the diagnosis of disseminated granuloma annulare. The patient was treated with 25 sessions of narrowband ultraviolet-B (NB-UVB) phototherapy and acitretin 20mg/day for 5 months.@*Conclusion@#Both histopathology and dermoscopy were noted to be valuable tools in the diagnosis of disseminated GA, as well as in monitoring response to the combined therapy.