Dermatol Clin. Jul;11(3) Epidermolysis bullosa acquisita and bullous systemic lupus erythematosus. Diseases of autoimmunity to type VII collagen. Bullous Systemic Lupus Erythematosus (BSLE) is a rare manifestation of . (BP), inflammatory variant of epidermolysis bullosa acquisita (EBA), and linear IgA. However, unlike epidermolysis bullosa acquisita, bullous systemic lupus erythematosus tends to respond dramatically to treatment with.
|Published (Last):||23 April 2005|
|PDF File Size:||10.46 Mb|
|ePub File Size:||9.37 Mb|
|Price:||Free* [*Free Regsitration Required]|
Often associated is a heterogeneous profile of autoimmunity to components of type VII collagen. It needs to be differentiated from other subepidermal bullous diseases in view of potentially associated systemic manifestations of SLE. We report a year-old lady who presented with an extensive vesicobullous eruption on face, neck, trunk, and mucosa. She was also found to have photosensitivity, significant anemia, and high antibody titers confirming SLE.
Routine histopathology and immunoflorescence studies suggested BSLE and she responded favorably to dapsone. The importance of clinical and histopathological findings in confirming a diagnosis of BSLE along with the very encouraging response to dapsone therapy is highlighted.
Bullous systemic lupus erythematosus SLE is a clinicopathologically distinctive immunobullous eruption occurring during the course of SLE. The disease is very responsive to dapsone therapy. Subepidermal blister formation in the course of severe systemic lupus erythematosus SLE can occur due to extensive interface inflammation and basal cell vacuolation, presenting as polycyclic erosions with advancing blistering border predominantly on sun-exposed areas.
Occasionally other antigens such as laminin 5, laminin 6, and BP bullous pemphigoid antigen have also been described. A year-old lady, a homemaker and mother of three, presented with largely asymptomatic vesicobullous lesions 2 months durationinvolving head and neck, upper limbs, and trunk over both photo-exposed and unexposed areas.
The blisters would rupture over days; yielding clear sometimes hemorrhagic fluid; heal over weeks; leaving behind pigmentation and occasional scarring. Concurrent painful oral ulcers were reported; though, other mucosae were uninvolved. A 6-month history suggestive of Raynaud’s phenomenon; arthralgias involving small joints of hands and feet; photosensitivity; and intermittent episodes of mild to moderate fever was present.
The patient reported three previous, spontaneous abortions, 5months gestation the last one being 6 years back. On examination, there were multiple, tense, clear as well as hemorrhagic, vesicles and bullae, arising over an erythematous base and predominantly involving upper body [ Figure 1 ].
Bulla spread sign and Nikolsky sign were positive. In addition, superficial erosions, areas of postinflammatory hyperpigmentation, and small superficial scars were noted. Superficial erosions were also noted over the buccal mucosa, hard palate, and lips.
Her gulloso examination was essentially normal. Presence of tense vesicles marked with an arrow filled with cleat fluid along with superficial erosions with polycyclic margins.
Lesions are present on covered as well as hulloso areas. A provisional diagnosis of BSLE was considered and she was thoroughly investigated. She had a significant anemia 8. Other biochemical investigations were essentially normal, with no evidence bu,loso renal derangement.
Extensive serological workup revealed antinuclear antibody positivity of 1: Russell’s viper venom test revealed no lupus like anticoagulant. X-rays of chest and involved joints were normal. A lesional biopsy revealed subepidermal split with neutrophilic infiltrate in the papillary dermis [ Figure 2 ]. Direct immunofluorescence both perilesional and distant-covered skin revealed IgM and C3 deposition along the basement membrane zone BMZ as well as perivascular location.
Due to resource constraints, indirect immunofluorescence or salt-split skin could not be performed.
Based on clinical, histopathological, and serological evidence, a diagnosis of BSLE was confirmed. Biopsy taken from vesicular lesion revealed a subepidermal split, containing numerous red blood cells. Inset shows a positive lupus band IgG at the lupuus membrane zone. With a normal glucose 6-Phosphate dehydrogenase G6PD activity, we planned to start her on dapsone; however, her low hemoglobin prevented this.
Hematinics were also initiated. There was only partial improvement with new lesions developing even after 4 weeks; wherein, she was started on dapsone.
She responded quite favorably with no new lesions and satisfactory healing of pre-existing ones [ Figure 3 ]. Over the next 6 months, steroids were completely withdrawn, and dapsone and hydroxychloroquine continued for further 1 year. The patient has remained in remission without any systemic manifestations as well, over the last 1 year. It generally affects young adult females and involves both sun-exposed and nonexposed areas. Clinically, it is characterized by subepidermal blisters; appearing on erythematous or urticated base; with a predilection for face, upper trunk, and proximal extremities.
Our understanding of the pathology of this interesting and unique dermatologic condition has evolved with improved diagnostic criteria and clinicohistologic profiling. Type I fulfilling all six criteria, while Type II undetermined antigen or dermal antigen other than type VII collagen satisfies criteria only.
As seen in our case, BSLE developing with or prior to features of SLE can present a diagnostic dilemma; hence, other subepidermal vesicobullous disorders need to be ruled out. Histopathologically, a predominant neutrophilic infiltrate lupux suggest Dermatitis herpetiformis DH and dramatic response to dapsone is also common to both diseases. Clinical, histopathological, and immunopathological overlap exists with BP as bjlloso.
EBA is histopathologically and immunopathologically identical, as it is also mediated by antibodies to type VII collagen; bullosl, a dramatic lipus to dapsone, as in our case, helps nulloso the two as EBA is notoriously resistant to treatment. Whether or not BSLE is associated with systemic activity is a hotly debated topic.
Lupus ampolloso: un verdadero reto diagnóstico y terapéutico
Most reported cases have dramatically responded to dapsone, with cessation of new lesions in days and healing within several days. Though, it may be difficult to introduce the drug initially, as in our case; every attempt should be made to use it, as relatively low doses mg have also been shown to be efficacious. Our patient showed only modest improvement on steroids and antimalarials, complete response being seen only with dapsone.
The case is presented for its typical and distinctive features which should prompt an astute physician to suspect and investigate underlying systemic manifestations of SLE. Also, the dramatic response to dapsone is highlighted which remains the drug of choice in the management of this disorder. Dapsone should be considered the drug of choice for bullous systemic lupus erythematosus as treatment response to other options is suboptimal. Even if one is not able to initiate dapsone due to relative contraindications, as in our case, every effort should be made to use it as early as possible.
The course of the disease is benign with remission reported in most of the cases within a year, including ours. National Center for Biotechnology InformationU. Journal List Indian J Dermatol v. Author information Article notes Copyright and License information Disclaimer.
Lupus Eritematoso Sistémico Bulloso en una niña de 10 años de edad. Reporte de un caso
Received Nov; Accepted Jan. This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3. This article has been cited by other articles in PMC.
Dapsonetype VII collagenimmunobullous diseases. Introduction What was known?
Lupus Eritematoso Sistémico Bulloso en una niña de 10 años de edad. Reporte de un caso
Case Report A year-old lady, a homemaker and mother of three, presented with largely asymptomatic vesicobullous lesions 2 months durationlkpus head and neck, upper limbs, and trunk over both photo-exposed and unexposed areas. Open in a separate window. Marked improvement seen 1 week after starting dapsone therapy.
Footnotes Source of Support: Nil Conflict of Interest: Yung A, Oakley A. Bullous systemic lupus erythematosus. Bullous systemic lupus erythematosus with antibodies recognizing multiple basement membrane components, bullous pemphigoid antigen1, lamini-5, laminin-6, and type VII collagen. Bullous eruption of systemic lupus erythematosus. Wojnarowska F, Briggaman RA, editors.
Management of Bullos Diseases. Chapman and Hall Ltd; A phenotypically distinctive but immunologically heterogeneous bullous disorder.
Immunodominant auto-epitopes of type VII collagen are short, paired peptide sequences within the fibronectin type III homology region of the noncollagenous NC1 domain.
Hermann Pinkus memorial Lecture Lupuss to collagen VII: Autoantibody mediated pathomechanisms regulate clinical-pathological phenotypes of acquired epidermolysisbullosa and bullous SLE. The carboxyl terminus of type VII collagen mediates antiparallel dimer formation and constitutes a new antigenic epitope for epidermolysisBullosaacquisita autoantibodies. Bullous systemic lupus erythematosus: Revised criteria for diagnosis. Vesiculobullous systemic lupus erythematosus.