Patient Presentation
This 85-year-old female presented with an extremely pruritic rash that was unresponsive to a 1-week prednisone taper. It began on her back and within days, new lesions were noted on her scalp, neck, chest, and legs. Calamine lotion temporarily alleviated the pruritus. The patient was otherwise asymptomatic and denied any visual problems. Her medications included an Albuterol inhaler for asthma and Lipitor for hypercholesterolemia.
What’s Your Diagnosis?
Diagnosis: Linear IgA Dermatosis
On physical examination, innumerable erythematous papules and plaques — some with white scale and some with erosions and hemorrhagic crust — were present in a bilateral and symmetrical distribution on the posterior neck, upper chest, back, buttocks, medial knees, and ankles. Of note, several intact vesicles were noted on the mid-back.
Biopsies for hematoxylin and eosin and immunofluorescence were done.Linear immunoglobulin A (IgA) dermatosis (LAD), which was first described as distinct from papillary IgA dermatitis herpetiformis in 1982 under the name adult linear IgA disease, is a rare autoimmune disorder in which IgA antibodies attack various indigenous antigens of the basement membrane zone (BMZ).1,2 Subsequent invasion of neutrophils and complement results in subepidermal separation. This translates clinically to vesicles erupting on the trunk and extremities in adults and on the lower abdomen and anogenital areas in children. Although this immunoglobulin disease can be distinguished by immunofluorescence studies demonstrating build-up of a linear band of IgA in the BMZ, it often presents with a clinical picture similar to that of other autoimmune blistering diseases.2,3
Clinical Features
The characteristic separation of the dermal-epidermal junction produces discrete, clustered or annular vesicles or bullae. The bullae variably rest on normal, erythematous or urticarial skin, and may secondarily become crusted, impetiginized or even necrotic.3 (See photo below.) These lesions are also occasionally engulfed by the presence of telangiectasia.4 In addition to the more common cutaneous distribution, LAD is also frequently associated with oral and ocular mucosal lesions.5
Pathogenesis and Histology
The IgA antibodies of LAD are most commonly confined to the basement membrane and only circulate in approximately 30% of patients.2 Bullous pemphigoid (BP) 180 antigen (BPAG2) and what have been postulated to be its breakdown products, 97 kDa (LABD97) and 120 kDa (LAD-1), appear to be the primary antigens implicated in the IgA autoantibody response.6
Not surprisingly, immunofluorescence provides an excellent tool for characterizing LAD. Indeed, a positive immunofluorescence finding will demonstrate a distinct accumulation of linear IgA along the BMZ. (See photo below.) Similarly, immunoelectron microscopy provides a second avenue for confirming and localizing the presence of an IgA autoimmune response in the BMZ. LAD also has a distinct histological pattern illustrated by an invasion of neutrophils to dermal papillary tips. Eosinophils, in addition to clustering with neutrophils inside the prominent subepidermal blisters, are found diffusely scattered throughout the papillary dermis.2,6,7
Etiology and Prevalence
Although the complete etiology of LAD is largely unknown, a number of autoimmune and inflammatory diseases, cancers, and drug reactions have demonstrated a relationship with the onset of the disease.
Some conditions previously reported to be linked to LAD include systemic lupus erythematosus, rheumatoid arthritis, ulcerative colitis, dermatomyositis, multiple sclerosis, Crohn’s disease, typhoid, tuberculosis, brucella, respiratory infections, gynecological infections, and various cancers. In relation to adverse drug reactions, vancomycin, lithium carbonate, diclofenac, gliberamide, captopril, phenytonin sodium, ampicillin/sulbactam, and penicillin sodium, as well as others, have all been implicated in LAD’s development.3,4,8
LAD tends to affect women slightly more than men. It has a mean childhood onset of 3.3 to 4.5 years, a mean adult onset of 52 years, and has a reported age range of 6 months to 83 years.3 We now report a case of new-onset LAD in an 85-year-old woman.
Diagnosis and Management
LAD and many of the cutaneous autoimmune blistering disorders present with a similar heterogeneous clinical picture. BP and dermatitis herpetiformis are the most common diseases in the differential diagnosis. As mentioned previously, when presented with this differential diagnosis, histology, immunofluorescence, and immunoelectron microscopy allow for a near- definitive diagnosis of LAD.3
If the patient’s LAD is secondary to a drug reaction, the initial treatment involves discontinuing the culprit medication. In idiopathic and unresolved drug-induced LAD, a 50-mg daily treatment of dapsone, which inhibits neutrophil infiltration, remains the standard treatment. The dosage can be adjusted by 25- or 50-mg increases weekly or biweekly in order to reach an optimal level of response.
Prior to starting dapsone, the patient must be screened for a glucose-6-phosphate dehydrogenase (G6PD) deficiency, which can increase the risk for hemolytic anemia or methemoglobinemia in patients receiving dapsone. For patients who cannot tolerate dapsone, a 500-mg, twice-a-day treatment of sulfapyridine, which can be adjusted by 1,000-mg increases weekly or biweekly, remains an adequate alternative.
Glucocorticoids such as prednisone, which can be used in conjunction with the above medications, provide an additional option if dapsone or sulfapyridine fail to completely control the disease.9
Colchicine, dicloxacillin, and sulfamethoxypyridazine are additional treatment alternatives, and intravenous immunoglobulin therapy has effectively treated LAD in a case of renal failure.3,10
Our patient responded quickly to dapsone 75 mg a day and clobetasol ointment. Currently, she is maintained on dapsone 25 mg a day, which keeps her in remission. Ophthalmologic evaluation revealed no ocular involvement. Additionally, a general medical work-up has identified no underlying disease.
CALL FOR CASES
If you have a case you’d like to see published, send a write-up (1,200 to 1,500 words) and an image of the patient’s condition to:
Dr. Amor Khachemoune
Ronald O. Perelman
Department of Dermatology
New York University School of Medicine
530 First Avenue, Ste. 7R
New York, NY 10016
Or, e-mail them to amorkh@pol.net.
Patient Presentation
This 85-year-old female presented with an extremely pruritic rash that was unresponsive to a 1-week prednisone taper. It began on her back and within days, new lesions were noted on her scalp, neck, chest, and legs. Calamine lotion temporarily alleviated the pruritus. The patient was otherwise asymptomatic and denied any visual problems. Her medications included an Albuterol inhaler for asthma and Lipitor for hypercholesterolemia.
What’s Your Diagnosis?
Diagnosis: Linear IgA Dermatosis
On physical examination, innumerable erythematous papules and plaques — some with white scale and some with erosions and hemorrhagic crust — were present in a bilateral and symmetrical distribution on the posterior neck, upper chest, back, buttocks, medial knees, and ankles. Of note, several intact vesicles were noted on the mid-back.
Biopsies for hematoxylin and eosin and immunofluorescence were done.Linear immunoglobulin A (IgA) dermatosis (LAD), which was first described as distinct from papillary IgA dermatitis herpetiformis in 1982 under the name adult linear IgA disease, is a rare autoimmune disorder in which IgA antibodies attack various indigenous antigens of the basement membrane zone (BMZ).1,2 Subsequent invasion of neutrophils and complement results in subepidermal separation. This translates clinically to vesicles erupting on the trunk and extremities in adults and on the lower abdomen and anogenital areas in children. Although this immunoglobulin disease can be distinguished by immunofluorescence studies demonstrating build-up of a linear band of IgA in the BMZ, it often presents with a clinical picture similar to that of other autoimmune blistering diseases.2,3
Clinical Features
The characteristic separation of the dermal-epidermal junction produces discrete, clustered or annular vesicles or bullae. The bullae variably rest on normal, erythematous or urticarial skin, and may secondarily become crusted, impetiginized or even necrotic.3 (See photo below.) These lesions are also occasionally engulfed by the presence of telangiectasia.4 In addition to the more common cutaneous distribution, LAD is also frequently associated with oral and ocular mucosal lesions.5
Pathogenesis and Histology
The IgA antibodies of LAD are most commonly confined to the basement membrane and only circulate in approximately 30% of patients.2 Bullous pemphigoid (BP) 180 antigen (BPAG2) and what have been postulated to be its breakdown products, 97 kDa (LABD97) and 120 kDa (LAD-1), appear to be the primary antigens implicated in the IgA autoantibody response.6
Not surprisingly, immunofluorescence provides an excellent tool for characterizing LAD. Indeed, a positive immunofluorescence finding will demonstrate a distinct accumulation of linear IgA along the BMZ. (See photo below.) Similarly, immunoelectron microscopy provides a second avenue for confirming and localizing the presence of an IgA autoimmune response in the BMZ. LAD also has a distinct histological pattern illustrated by an invasion of neutrophils to dermal papillary tips. Eosinophils, in addition to clustering with neutrophils inside the prominent subepidermal blisters, are found diffusely scattered throughout the papillary dermis.2,6,7
Etiology and Prevalence
Although the complete etiology of LAD is largely unknown, a number of autoimmune and inflammatory diseases, cancers, and drug reactions have demonstrated a relationship with the onset of the disease.
Some conditions previously reported to be linked to LAD include systemic lupus erythematosus, rheumatoid arthritis, ulcerative colitis, dermatomyositis, multiple sclerosis, Crohn’s disease, typhoid, tuberculosis, brucella, respiratory infections, gynecological infections, and various cancers. In relation to adverse drug reactions, vancomycin, lithium carbonate, diclofenac, gliberamide, captopril, phenytonin sodium, ampicillin/sulbactam, and penicillin sodium, as well as others, have all been implicated in LAD’s development.3,4,8
LAD tends to affect women slightly more than men. It has a mean childhood onset of 3.3 to 4.5 years, a mean adult onset of 52 years, and has a reported age range of 6 months to 83 years.3 We now report a case of new-onset LAD in an 85-year-old woman.
Diagnosis and Management
LAD and many of the cutaneous autoimmune blistering disorders present with a similar heterogeneous clinical picture. BP and dermatitis herpetiformis are the most common diseases in the differential diagnosis. As mentioned previously, when presented with this differential diagnosis, histology, immunofluorescence, and immunoelectron microscopy allow for a near- definitive diagnosis of LAD.3
If the patient’s LAD is secondary to a drug reaction, the initial treatment involves discontinuing the culprit medication. In idiopathic and unresolved drug-induced LAD, a 50-mg daily treatment of dapsone, which inhibits neutrophil infiltration, remains the standard treatment. The dosage can be adjusted by 25- or 50-mg increases weekly or biweekly in order to reach an optimal level of response.
Prior to starting dapsone, the patient must be screened for a glucose-6-phosphate dehydrogenase (G6PD) deficiency, which can increase the risk for hemolytic anemia or methemoglobinemia in patients receiving dapsone. For patients who cannot tolerate dapsone, a 500-mg, twice-a-day treatment of sulfapyridine, which can be adjusted by 1,000-mg increases weekly or biweekly, remains an adequate alternative.
Glucocorticoids such as prednisone, which can be used in conjunction with the above medications, provide an additional option if dapsone or sulfapyridine fail to completely control the disease.9
Colchicine, dicloxacillin, and sulfamethoxypyridazine are additional treatment alternatives, and intravenous immunoglobulin therapy has effectively treated LAD in a case of renal failure.3,10
Our patient responded quickly to dapsone 75 mg a day and clobetasol ointment. Currently, she is maintained on dapsone 25 mg a day, which keeps her in remission. Ophthalmologic evaluation revealed no ocular involvement. Additionally, a general medical work-up has identified no underlying disease.
CALL FOR CASES
If you have a case you’d like to see published, send a write-up (1,200 to 1,500 words) and an image of the patient’s condition to:
Dr. Amor Khachemoune
Ronald O. Perelman
Department of Dermatology
New York University School of Medicine
530 First Avenue, Ste. 7R
New York, NY 10016
Or, e-mail them to amorkh@pol.net.
Patient Presentation
This 85-year-old female presented with an extremely pruritic rash that was unresponsive to a 1-week prednisone taper. It began on her back and within days, new lesions were noted on her scalp, neck, chest, and legs. Calamine lotion temporarily alleviated the pruritus. The patient was otherwise asymptomatic and denied any visual problems. Her medications included an Albuterol inhaler for asthma and Lipitor for hypercholesterolemia.
What’s Your Diagnosis?
Diagnosis: Linear IgA Dermatosis
On physical examination, innumerable erythematous papules and plaques — some with white scale and some with erosions and hemorrhagic crust — were present in a bilateral and symmetrical distribution on the posterior neck, upper chest, back, buttocks, medial knees, and ankles. Of note, several intact vesicles were noted on the mid-back.
Biopsies for hematoxylin and eosin and immunofluorescence were done.Linear immunoglobulin A (IgA) dermatosis (LAD), which was first described as distinct from papillary IgA dermatitis herpetiformis in 1982 under the name adult linear IgA disease, is a rare autoimmune disorder in which IgA antibodies attack various indigenous antigens of the basement membrane zone (BMZ).1,2 Subsequent invasion of neutrophils and complement results in subepidermal separation. This translates clinically to vesicles erupting on the trunk and extremities in adults and on the lower abdomen and anogenital areas in children. Although this immunoglobulin disease can be distinguished by immunofluorescence studies demonstrating build-up of a linear band of IgA in the BMZ, it often presents with a clinical picture similar to that of other autoimmune blistering diseases.2,3
Clinical Features
The characteristic separation of the dermal-epidermal junction produces discrete, clustered or annular vesicles or bullae. The bullae variably rest on normal, erythematous or urticarial skin, and may secondarily become crusted, impetiginized or even necrotic.3 (See photo below.) These lesions are also occasionally engulfed by the presence of telangiectasia.4 In addition to the more common cutaneous distribution, LAD is also frequently associated with oral and ocular mucosal lesions.5
Pathogenesis and Histology
The IgA antibodies of LAD are most commonly confined to the basement membrane and only circulate in approximately 30% of patients.2 Bullous pemphigoid (BP) 180 antigen (BPAG2) and what have been postulated to be its breakdown products, 97 kDa (LABD97) and 120 kDa (LAD-1), appear to be the primary antigens implicated in the IgA autoantibody response.6
Not surprisingly, immunofluorescence provides an excellent tool for characterizing LAD. Indeed, a positive immunofluorescence finding will demonstrate a distinct accumulation of linear IgA along the BMZ. (See photo below.) Similarly, immunoelectron microscopy provides a second avenue for confirming and localizing the presence of an IgA autoimmune response in the BMZ. LAD also has a distinct histological pattern illustrated by an invasion of neutrophils to dermal papillary tips. Eosinophils, in addition to clustering with neutrophils inside the prominent subepidermal blisters, are found diffusely scattered throughout the papillary dermis.2,6,7
Etiology and Prevalence
Although the complete etiology of LAD is largely unknown, a number of autoimmune and inflammatory diseases, cancers, and drug reactions have demonstrated a relationship with the onset of the disease.
Some conditions previously reported to be linked to LAD include systemic lupus erythematosus, rheumatoid arthritis, ulcerative colitis, dermatomyositis, multiple sclerosis, Crohn’s disease, typhoid, tuberculosis, brucella, respiratory infections, gynecological infections, and various cancers. In relation to adverse drug reactions, vancomycin, lithium carbonate, diclofenac, gliberamide, captopril, phenytonin sodium, ampicillin/sulbactam, and penicillin sodium, as well as others, have all been implicated in LAD’s development.3,4,8
LAD tends to affect women slightly more than men. It has a mean childhood onset of 3.3 to 4.5 years, a mean adult onset of 52 years, and has a reported age range of 6 months to 83 years.3 We now report a case of new-onset LAD in an 85-year-old woman.
Diagnosis and Management
LAD and many of the cutaneous autoimmune blistering disorders present with a similar heterogeneous clinical picture. BP and dermatitis herpetiformis are the most common diseases in the differential diagnosis. As mentioned previously, when presented with this differential diagnosis, histology, immunofluorescence, and immunoelectron microscopy allow for a near- definitive diagnosis of LAD.3
If the patient’s LAD is secondary to a drug reaction, the initial treatment involves discontinuing the culprit medication. In idiopathic and unresolved drug-induced LAD, a 50-mg daily treatment of dapsone, which inhibits neutrophil infiltration, remains the standard treatment. The dosage can be adjusted by 25- or 50-mg increases weekly or biweekly in order to reach an optimal level of response.
Prior to starting dapsone, the patient must be screened for a glucose-6-phosphate dehydrogenase (G6PD) deficiency, which can increase the risk for hemolytic anemia or methemoglobinemia in patients receiving dapsone. For patients who cannot tolerate dapsone, a 500-mg, twice-a-day treatment of sulfapyridine, which can be adjusted by 1,000-mg increases weekly or biweekly, remains an adequate alternative.
Glucocorticoids such as prednisone, which can be used in conjunction with the above medications, provide an additional option if dapsone or sulfapyridine fail to completely control the disease.9
Colchicine, dicloxacillin, and sulfamethoxypyridazine are additional treatment alternatives, and intravenous immunoglobulin therapy has effectively treated LAD in a case of renal failure.3,10
Our patient responded quickly to dapsone 75 mg a day and clobetasol ointment. Currently, she is maintained on dapsone 25 mg a day, which keeps her in remission. Ophthalmologic evaluation revealed no ocular involvement. Additionally, a general medical work-up has identified no underlying disease.
CALL FOR CASES
If you have a case you’d like to see published, send a write-up (1,200 to 1,500 words) and an image of the patient’s condition to:
Dr. Amor Khachemoune
Ronald O. Perelman
Department of Dermatology
New York University School of Medicine
530 First Avenue, Ste. 7R
New York, NY 10016
Or, e-mail them to amorkh@pol.net.
