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Diffuse Bullous Eruptions in an Elderly Woman: Late-Onset Bullous Systemic Lupus Erythematosus

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ABSTRACT

Vesiculobullous eruptions in the elderly represent a diverse range of varying pathophysiologies and can present a significant clinical dilemma to the diagnostician. Diagnosis requires a careful review of clinical history, attention to detail on physical and histomorphological examination, and appropriate immunofluorescence testing. We describe the case of a 73-year-old female who presented to our hospital with a painful blistering skin rash developed over 2 days. Examination of the skin was remarkable for numerous flaccid hemorrhagic bullae on a normal-appearing nonerythematous skin involving both the upper and lower extremities. Histopathology of the biopsy lesion showed interface change at the epidermo-dermal region with subepidermal blister formation, mild dermal fibrosis, and sparse interstitial neutrophilic infiltrate. Immunohistological analysis was significant for positive IgG basement membrane zone antibodies with a dermal pattern of localization on direct immunofluorescence and positive IgG antinuclear antibodies on indirect immunofluorescence. Evidence of antibodies to type VII collagen suggested the diagnosis of epidermolysis bullosa acquisita versus bullous systemic lupus erythematosus (BSLE). A diagnosis of BSLE was made based on positive American College of Rheumatology criteria, acquired vesiculo-bullous eruptions with compatible histopathological and immunofluorescence findings. This case illustrates one of many difficulties a physician encounters while arriving at a diagnosis from a myriad of immunobullous dermatoses. Also, it is important for internists and dermatologists alike to be aware of and differentiate this uncommon and nonspecific cutaneous SLE manifestation from a myriad of disorders presenting with vesiculobullous skin eruptions in the elderly.

No MeSH data available.


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Bullae replaced by patches of postinflammatory hypopigmentation.
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Figure 2: Bullae replaced by patches of postinflammatory hypopigmentation.

Mentions: This case illustrates one of many difficulties a physician encounters while arriving at a diagnosis from a myriad of immunobullous dermatoses. Histopathological and immunofluorescence testing play a key diagnostic role in narrowing an extensive differential diagnosis of vesiculobullous disorders. BSLE needs to be differentiated from pemphigoid, a much more common immunobullous disorder in elderly patients over 70 years old [2]. The negative pemphigoid serologies and incompatible immunofluorescence findings helped rule out pemphigoid. BSLE must also be differentiated from EBA, a disorder which shares many clinical and histopathological features of BSLE [3]. Immunopathological criteria of BSLE are similar to EBA and can present a significant diagnostic dilemma, especially in a patient with no known prior history of SLE. Differentiating these two disorders is important to inform the physician on optimum management and prognosis. A careful history taking focusing on characteristics of the lesions, relevant serologies, and response to dapsone help differentiate these two disorders [4]. Lesions of EBA are predominantly mechanobullous in nature and characteristically occur on trauma-exposed areas like the elbows, knees, ankles, and dorsum of the hands [5]. Also, EBA skin lesions are more resistant to treatment with steroids and other immunomodulatory agents, an important differentiating characteristic. Positive antinuclear antibody titers with fulfillment of ACR SLE criteria and response to steroids favored the diagnosis of BSLE in our patient [3, 6]. It was her first clinical manifestation of elderly-onset SLE, a specific SLE subset with significantly poor morbidity and mortality outcomes [7, 8]. BSLE is a very uncommon and nonspecific cutaneous manifestation of SLE occurring in less than 1% of patients [9]. It generally occurs in middle-aged adults but has also been rarely reported in the elderly [3]. BSLE may coexist with other immunobullous and nonimmunobullous diseases [5]. BSLE may occur as the initial manifestation of SLE and requires the diagnostician to maintain a high degree of clinical suspicion [10]. Secondly, SLE features are common in rheumatoid arthritis, and the two diseases can coexist in the same individual, as in our patient [11]. Our patient was initiated on empiric pulsed steroids, while diagnostic workup was in progress, and showed a good clinical response to treatment (fig 2). BSLE lesions have a dramatic response to dapsone, making it the therapeutic agent of choice with a significant diagnostic value [12]. Responses to corticosteroids are less definite, but case reports have documented effectiveness to steroid administration [13]. It is important for internists and dermatologists alike to be aware of and differentiate this uncommon and nonspecific cutaneous SLE manifestation from a myriad of disorders presenting with vesiculobullous skin eruptions in the elderly. At the time of this writing, the patient was asked to follow up with the dermatologist to discuss her diagnosis and institute definitive management.


Diffuse Bullous Eruptions in an Elderly Woman: Late-Onset Bullous Systemic Lupus Erythematosus
Bullae replaced by patches of postinflammatory hypopigmentation.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5121548&req=5

Figure 2: Bullae replaced by patches of postinflammatory hypopigmentation.
Mentions: This case illustrates one of many difficulties a physician encounters while arriving at a diagnosis from a myriad of immunobullous dermatoses. Histopathological and immunofluorescence testing play a key diagnostic role in narrowing an extensive differential diagnosis of vesiculobullous disorders. BSLE needs to be differentiated from pemphigoid, a much more common immunobullous disorder in elderly patients over 70 years old [2]. The negative pemphigoid serologies and incompatible immunofluorescence findings helped rule out pemphigoid. BSLE must also be differentiated from EBA, a disorder which shares many clinical and histopathological features of BSLE [3]. Immunopathological criteria of BSLE are similar to EBA and can present a significant diagnostic dilemma, especially in a patient with no known prior history of SLE. Differentiating these two disorders is important to inform the physician on optimum management and prognosis. A careful history taking focusing on characteristics of the lesions, relevant serologies, and response to dapsone help differentiate these two disorders [4]. Lesions of EBA are predominantly mechanobullous in nature and characteristically occur on trauma-exposed areas like the elbows, knees, ankles, and dorsum of the hands [5]. Also, EBA skin lesions are more resistant to treatment with steroids and other immunomodulatory agents, an important differentiating characteristic. Positive antinuclear antibody titers with fulfillment of ACR SLE criteria and response to steroids favored the diagnosis of BSLE in our patient [3, 6]. It was her first clinical manifestation of elderly-onset SLE, a specific SLE subset with significantly poor morbidity and mortality outcomes [7, 8]. BSLE is a very uncommon and nonspecific cutaneous manifestation of SLE occurring in less than 1% of patients [9]. It generally occurs in middle-aged adults but has also been rarely reported in the elderly [3]. BSLE may coexist with other immunobullous and nonimmunobullous diseases [5]. BSLE may occur as the initial manifestation of SLE and requires the diagnostician to maintain a high degree of clinical suspicion [10]. Secondly, SLE features are common in rheumatoid arthritis, and the two diseases can coexist in the same individual, as in our patient [11]. Our patient was initiated on empiric pulsed steroids, while diagnostic workup was in progress, and showed a good clinical response to treatment (fig 2). BSLE lesions have a dramatic response to dapsone, making it the therapeutic agent of choice with a significant diagnostic value [12]. Responses to corticosteroids are less definite, but case reports have documented effectiveness to steroid administration [13]. It is important for internists and dermatologists alike to be aware of and differentiate this uncommon and nonspecific cutaneous SLE manifestation from a myriad of disorders presenting with vesiculobullous skin eruptions in the elderly. At the time of this writing, the patient was asked to follow up with the dermatologist to discuss her diagnosis and institute definitive management.

View Article: PubMed Central - PubMed

ABSTRACT

Vesiculobullous eruptions in the elderly represent a diverse range of varying pathophysiologies and can present a significant clinical dilemma to the diagnostician. Diagnosis requires a careful review of clinical history, attention to detail on physical and histomorphological examination, and appropriate immunofluorescence testing. We describe the case of a 73-year-old female who presented to our hospital with a painful blistering skin rash developed over 2 days. Examination of the skin was remarkable for numerous flaccid hemorrhagic bullae on a normal-appearing nonerythematous skin involving both the upper and lower extremities. Histopathology of the biopsy lesion showed interface change at the epidermo-dermal region with subepidermal blister formation, mild dermal fibrosis, and sparse interstitial neutrophilic infiltrate. Immunohistological analysis was significant for positive IgG basement membrane zone antibodies with a dermal pattern of localization on direct immunofluorescence and positive IgG antinuclear antibodies on indirect immunofluorescence. Evidence of antibodies to type VII collagen suggested the diagnosis of epidermolysis bullosa acquisita versus bullous systemic lupus erythematosus (BSLE). A diagnosis of BSLE was made based on positive American College of Rheumatology criteria, acquired vesiculo-bullous eruptions with compatible histopathological and immunofluorescence findings. This case illustrates one of many difficulties a physician encounters while arriving at a diagnosis from a myriad of immunobullous dermatoses. Also, it is important for internists and dermatologists alike to be aware of and differentiate this uncommon and nonspecific cutaneous SLE manifestation from a myriad of disorders presenting with vesiculobullous skin eruptions in the elderly.

No MeSH data available.


Related in: MedlinePlus