Patient Presentation
A 32-year-old Caucasian woman presented to our ambulatory dermatology clinic with recent onset of a pruritic eruption involving her thighs, legs, arms and abdomen. She was a primigravida at 22 weeks gestation. She was taking no medications other than prenatal vitamins and denied any medication allergies. Furthermore, she had no prior medical conditions and no prior surgeries.
Physical examination revealed multiple scattered, flaccid bullae on an erythematous base and multiple erythematous plaques. These were located primarily on her legs with less involvement of her arms and abdomen. Punch biopsies were obtained, which revealed a subepidermal bullae. Direct immunofluorescence demonstrated linear deposition of C3.
What is Your Diagnosis?
Diagnosis:Pemphigoid Gestationis
Pemphigoid gestationis (PG), previously known as herpes gestationis, is a rare autoimmune bullous dermatosis of pregnancy. It typically presents with intense pruritus and urticaria-like papules beginning around the umbilicus or less commonly on the extremities during or immediately following pregnancy.1-3
The term “herpes gestationis” was labeled by Milton in 1872. The utility of immunofluorescent techniques in the diagnosis of PG helped to define this entity.4 Antibodies develop against two proteins of the hemidesmosome, namely BP antigen 1 (BP180, 180 kD) and BP antigen 2 (BP230, 230 kD). Both BP180 and BP230 are of the IgG1 subclass. The lesions of PG may progress to the palms and soles and have rarely been reported to involve the face and mucous membranes. Lesions tend to present in the second or third trimester and may progress and/or flare during the peripartum period.
Maternal health is largely spared with only a slightly increased frequency of Grave’s disease in PG patients, necessitating long-term surveillance for thyroid disease. There have been rare reports of choriocarcinoma in this patient population as well.5 The incidence of PG may be as high as 1 in 50,000 births.3 The newborn has approximately a 10% chance of transient bullae but no long- term sequelae.
Differential Diagnosis
The differential diagnoses include contact dermatitis, pruritic urticarial papules and plaques of pregnancy (PUPPP), bullous pemphigoid (BP) and impetigo herpetiformis.
Immunofluorescence is a useful method to differentiate among the aforementioned diagnoses. PG is identified by the deposition of C3 along the basement membrane zone, with a majority of cases positive for IgG1 as well. The autoantibody is selective for the N-terminus domain of BP180, a transmembrane hemidesmosomal glycoprotein also known as collagen type XVII.
Diagnosing PG
Diagnosis is made by clinical examination and histologic findings of a subepidermal bullous disease and confirmed by direct immunofluorescence (DIF).
Histologic findings include a subepidermal blister with eosinophilic infiltrates localized to the dermal-epidermal junction. Characteristic DIF findings include a linear band of C3 deposition along the basement membrane.6 Indirect immunofluoroscence can also be used to detect circulating antibodies. HLA-DR3/DR4 is increased in this patient population to approximately 45% compared to only 3% in the general population.6 Diagnosis in the current case was made by a combination of clinical examination, histologic findings and DIF.
Treatment
The goal of treatment is relief of symptoms while preventing infection and scarring, which is uncommon. Topical steroids and antihistamines can be considered as first-line treatment but are often ineffective in alleviating symptoms. In such cases, systemic corticosteroids (0.5 mg/kg/day) are the treatment of choice. Many patients’ symptoms may regress with systemic treatment but require additional treatment at delivery due to reoccurrence.
As always, when giving corticosteroids during pregnancy, infants should be evaluated for adrenal insufficiency. Should systemic corticosteroids be required, the treating physician should communicate with the patient’s obstetrician to keep him or her updated with the patient’s progress and corticosteroid requirements.
Additional therapeutic options documented in this patient population include dapsone and cyclosporine, with careful attention to the potential risks versus benefits.
Possible Complications for Infants
Neonatal herpes gestationis has been reported to present with cutaneous involvement in less than 10% of infants born to mothers with documented cases of PG. These lesions resolve spontaneously without treatment in days to weeks as maternal antibodies are destroyed with no long-term sequelae. In reported cases, infants present with papulovesicular eruptions and pruritus similar to the lesions reported in the mother.7 Subclinical disease is common in newborns and confirmed only by biopsy of the skin and the presence of complement deposition on immunoflourescence.
Although it was previously believed that PG during pregnancy leads to an increase in premature births and stillbirth, no study has been able to confirm these conclusions. According to a study of 28 women with PG, there was no evidence of increased fetal morbidity or mortality.3
Patient Presentation
A 32-year-old Caucasian woman presented to our ambulatory dermatology clinic with recent onset of a pruritic eruption involving her thighs, legs, arms and abdomen. She was a primigravida at 22 weeks gestation. She was taking no medications other than prenatal vitamins and denied any medication allergies. Furthermore, she had no prior medical conditions and no prior surgeries.
Physical examination revealed multiple scattered, flaccid bullae on an erythematous base and multiple erythematous plaques. These were located primarily on her legs with less involvement of her arms and abdomen. Punch biopsies were obtained, which revealed a subepidermal bullae. Direct immunofluorescence demonstrated linear deposition of C3.
What is Your Diagnosis?
Diagnosis:Pemphigoid Gestationis
Pemphigoid gestationis (PG), previously known as herpes gestationis, is a rare autoimmune bullous dermatosis of pregnancy. It typically presents with intense pruritus and urticaria-like papules beginning around the umbilicus or less commonly on the extremities during or immediately following pregnancy.1-3
The term “herpes gestationis” was labeled by Milton in 1872. The utility of immunofluorescent techniques in the diagnosis of PG helped to define this entity.4 Antibodies develop against two proteins of the hemidesmosome, namely BP antigen 1 (BP180, 180 kD) and BP antigen 2 (BP230, 230 kD). Both BP180 and BP230 are of the IgG1 subclass. The lesions of PG may progress to the palms and soles and have rarely been reported to involve the face and mucous membranes. Lesions tend to present in the second or third trimester and may progress and/or flare during the peripartum period.
Maternal health is largely spared with only a slightly increased frequency of Grave’s disease in PG patients, necessitating long-term surveillance for thyroid disease. There have been rare reports of choriocarcinoma in this patient population as well.5 The incidence of PG may be as high as 1 in 50,000 births.3 The newborn has approximately a 10% chance of transient bullae but no long- term sequelae.
Differential Diagnosis
The differential diagnoses include contact dermatitis, pruritic urticarial papules and plaques of pregnancy (PUPPP), bullous pemphigoid (BP) and impetigo herpetiformis.
Immunofluorescence is a useful method to differentiate among the aforementioned diagnoses. PG is identified by the deposition of C3 along the basement membrane zone, with a majority of cases positive for IgG1 as well. The autoantibody is selective for the N-terminus domain of BP180, a transmembrane hemidesmosomal glycoprotein also known as collagen type XVII.
Diagnosing PG
Diagnosis is made by clinical examination and histologic findings of a subepidermal bullous disease and confirmed by direct immunofluorescence (DIF).
Histologic findings include a subepidermal blister with eosinophilic infiltrates localized to the dermal-epidermal junction. Characteristic DIF findings include a linear band of C3 deposition along the basement membrane.6 Indirect immunofluoroscence can also be used to detect circulating antibodies. HLA-DR3/DR4 is increased in this patient population to approximately 45% compared to only 3% in the general population.6 Diagnosis in the current case was made by a combination of clinical examination, histologic findings and DIF.
Treatment
The goal of treatment is relief of symptoms while preventing infection and scarring, which is uncommon. Topical steroids and antihistamines can be considered as first-line treatment but are often ineffective in alleviating symptoms. In such cases, systemic corticosteroids (0.5 mg/kg/day) are the treatment of choice. Many patients’ symptoms may regress with systemic treatment but require additional treatment at delivery due to reoccurrence.
As always, when giving corticosteroids during pregnancy, infants should be evaluated for adrenal insufficiency. Should systemic corticosteroids be required, the treating physician should communicate with the patient’s obstetrician to keep him or her updated with the patient’s progress and corticosteroid requirements.
Additional therapeutic options documented in this patient population include dapsone and cyclosporine, with careful attention to the potential risks versus benefits.
Possible Complications for Infants
Neonatal herpes gestationis has been reported to present with cutaneous involvement in less than 10% of infants born to mothers with documented cases of PG. These lesions resolve spontaneously without treatment in days to weeks as maternal antibodies are destroyed with no long-term sequelae. In reported cases, infants present with papulovesicular eruptions and pruritus similar to the lesions reported in the mother.7 Subclinical disease is common in newborns and confirmed only by biopsy of the skin and the presence of complement deposition on immunoflourescence.
Although it was previously believed that PG during pregnancy leads to an increase in premature births and stillbirth, no study has been able to confirm these conclusions. According to a study of 28 women with PG, there was no evidence of increased fetal morbidity or mortality.3
Patient Presentation
A 32-year-old Caucasian woman presented to our ambulatory dermatology clinic with recent onset of a pruritic eruption involving her thighs, legs, arms and abdomen. She was a primigravida at 22 weeks gestation. She was taking no medications other than prenatal vitamins and denied any medication allergies. Furthermore, she had no prior medical conditions and no prior surgeries.
Physical examination revealed multiple scattered, flaccid bullae on an erythematous base and multiple erythematous plaques. These were located primarily on her legs with less involvement of her arms and abdomen. Punch biopsies were obtained, which revealed a subepidermal bullae. Direct immunofluorescence demonstrated linear deposition of C3.
What is Your Diagnosis?
Diagnosis:Pemphigoid Gestationis
Pemphigoid gestationis (PG), previously known as herpes gestationis, is a rare autoimmune bullous dermatosis of pregnancy. It typically presents with intense pruritus and urticaria-like papules beginning around the umbilicus or less commonly on the extremities during or immediately following pregnancy.1-3
The term “herpes gestationis” was labeled by Milton in 1872. The utility of immunofluorescent techniques in the diagnosis of PG helped to define this entity.4 Antibodies develop against two proteins of the hemidesmosome, namely BP antigen 1 (BP180, 180 kD) and BP antigen 2 (BP230, 230 kD). Both BP180 and BP230 are of the IgG1 subclass. The lesions of PG may progress to the palms and soles and have rarely been reported to involve the face and mucous membranes. Lesions tend to present in the second or third trimester and may progress and/or flare during the peripartum period.
Maternal health is largely spared with only a slightly increased frequency of Grave’s disease in PG patients, necessitating long-term surveillance for thyroid disease. There have been rare reports of choriocarcinoma in this patient population as well.5 The incidence of PG may be as high as 1 in 50,000 births.3 The newborn has approximately a 10% chance of transient bullae but no long- term sequelae.
Differential Diagnosis
The differential diagnoses include contact dermatitis, pruritic urticarial papules and plaques of pregnancy (PUPPP), bullous pemphigoid (BP) and impetigo herpetiformis.
Immunofluorescence is a useful method to differentiate among the aforementioned diagnoses. PG is identified by the deposition of C3 along the basement membrane zone, with a majority of cases positive for IgG1 as well. The autoantibody is selective for the N-terminus domain of BP180, a transmembrane hemidesmosomal glycoprotein also known as collagen type XVII.
Diagnosing PG
Diagnosis is made by clinical examination and histologic findings of a subepidermal bullous disease and confirmed by direct immunofluorescence (DIF).
Histologic findings include a subepidermal blister with eosinophilic infiltrates localized to the dermal-epidermal junction. Characteristic DIF findings include a linear band of C3 deposition along the basement membrane.6 Indirect immunofluoroscence can also be used to detect circulating antibodies. HLA-DR3/DR4 is increased in this patient population to approximately 45% compared to only 3% in the general population.6 Diagnosis in the current case was made by a combination of clinical examination, histologic findings and DIF.
Treatment
The goal of treatment is relief of symptoms while preventing infection and scarring, which is uncommon. Topical steroids and antihistamines can be considered as first-line treatment but are often ineffective in alleviating symptoms. In such cases, systemic corticosteroids (0.5 mg/kg/day) are the treatment of choice. Many patients’ symptoms may regress with systemic treatment but require additional treatment at delivery due to reoccurrence.
As always, when giving corticosteroids during pregnancy, infants should be evaluated for adrenal insufficiency. Should systemic corticosteroids be required, the treating physician should communicate with the patient’s obstetrician to keep him or her updated with the patient’s progress and corticosteroid requirements.
Additional therapeutic options documented in this patient population include dapsone and cyclosporine, with careful attention to the potential risks versus benefits.
Possible Complications for Infants
Neonatal herpes gestationis has been reported to present with cutaneous involvement in less than 10% of infants born to mothers with documented cases of PG. These lesions resolve spontaneously without treatment in days to weeks as maternal antibodies are destroyed with no long-term sequelae. In reported cases, infants present with papulovesicular eruptions and pruritus similar to the lesions reported in the mother.7 Subclinical disease is common in newborns and confirmed only by biopsy of the skin and the presence of complement deposition on immunoflourescence.
Although it was previously believed that PG during pregnancy leads to an increase in premature births and stillbirth, no study has been able to confirm these conclusions. According to a study of 28 women with PG, there was no evidence of increased fetal morbidity or mortality.3
