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Case Challenge:

A 6-Year-Old Girl with Extensive Bullous Skin Lesions Caroline Rassbach, MD; Neha Shah, MD; and Aisha Davis, MD

6-year-old previously healthy later, fluid-filled bullae developed at girl presented with extensive the centers of the target lesions and in- A bullous skin lesions involv- creased in number and size up to the ing approximately 25% of her total time of admission. body surface area (TBSA). She ini- Physical examination on admission tially presented 9 days earlier with revealed a nontoxic-appearing girl with low-grade , bilateral non-exu- extensive fluid-filled bullous lesions of dative conjunctival injection, cough, varying sizes behind her ears, around and . Her rash appeared as raised, her eyes and mouth, and on her neck, erythematous target lesions that began trunk, back, axillae, and perineum behind her ears and then progressed (Figures 1 and 2). Mucous membrane to involve her face and body. Her involvement included small bullae in symptoms at first presentation were her nares, hard palate, and perianal re- attributed to multiforme gion. She had scattered bullae on her All images courtesy of Neha Shah, MD, MPH. Reprinted with permission. and she was treated with hydroxyzine wrists and ankles, although her extrem- Figure 1. Extensive fluid-filled bullous lesions of and olopatadine eye drops. Five days ities were relatively spared. The skin at varying sizes covering the mouth, neck, and trunk the base of the bullae was erythema- of a 6-year-old girl. tous, slightly raised, and pruritic. Caroline Rassbach, MD, is a Pediatric Hospi- Initial work-up included a normal talist at Lucile Packard Children’s Hospital, Palo and , Alto, CA. Neha Shah, MD, is a Pediatric Hospital- clear chest radiograph, and negative her- ist at Children’s National Medical Center, Wash- pes simplex virus antigen swab from ington, DC. Aisha Davis, MD, is a Pediatric Hos- one of her lesions. She was admitted to pitalist at Children’s National Medical Center, the hospital for wound care, con- Washington, DC. trol, nutritional support, and diagnostic Address correspondence to: Caroline Rass- evaluation. She continued to develop bach, MD, 770 Welch Road #100, Palo Alto, CA bullae during her initial hospital days 94304; fax 650-498-5684; email: crassbac@stan- until approximately 60% of her TBSA ford.edu. was affected (Figure 3, see page 230). Disclosure: The authors have disclosed no rel- Her palms and soles also developed le- evant financial relationships. sions. Further testing and a skin biopsy Figure 2. Extensive fluid-filled bullous lesions of doi: 10.3928/00904481-20120525-05 varying sizes covering the trunk and perineum of revealed the diagnosis. a 6-year-old girl.

For diagnosis, see page 230 Editor’s note: Each month, Case Challenge features an unusual diagnosis. A description and images are presented, followed by the diagnosis and an explanation of how the diagnosis was determined. Your comments are welcome via email at [email protected]. To submit a case, visit: www.Healio.com/PedAnnals/Submit.

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Diagnosis: Bullous Systemic Erythematosus

The patient’s antinuclear was positive with a titer of 1:640. The skin biopsy revealed a dense, primarily neutrophilic infiltrate and separation at the dermal-epidermal junction. Direct revealed base- ment membrane deposits of immuno- globulin G (IgG), IgA, and IgM with- out complement deposits. This pattern is consistent with bullous , epidermolysis bullosa acquisita, or bullous systemic (BSLE). Additional serologic studies supported the diagnosis of BSLE, in- cluding positive anti-DNA , anti-Smith antibodies and anti-RNP antibodies, and low C3 and C4 levels. Figure 3. Lesions on the palm of the hand of a 6-year old girl. Serum testing for - specific antibodies was negative. ed patches. Patients with BSLE may biopsy in BSLE reveals subepidermal DISCUSSION develop chronic vesicobullous lesions neutrophilic infiltration in a concen- To our knowledge, our 6-year-old or may show spontaneous resolution trated or continuous pattern. Direct patient is the youngest reported case after 1 to 2 years. immunofluorescence testing reveals of BSLE in the literature. BSLE oc- Patients with BSLE represent fewer IgG in a linear or granular pattern at curs most commonly in females be- than 5% of all patients with systemic the dermo-epidermal junction. Anti- tween the ages of 10 and 40 years, lupus erythematosus (SLE) and may bodies to type VII collagen of the der- with the youngest previously reported or may not have other skin and sys- mal floor may also be present. This case in an 8-year-old girl. temic manifestations of SLE. Ne- infiltration leads to the characteristic Bullous systemic lupus erythema- phritis is the most common systemic separation of the and , tosus is a vesiculobullous eruption that manifestation in patients with BSLE. resulting in vesiculobullous lesions. may include vesicles or bullae on an BSLE has been reported as the ini- The for a child erythematous base filled with serous tial presentation of SLE, as coincid- with a bullous skin eruption includes or serosanguinous fluid. The lesions ing with an SLE exacerbation, and bullous , Ste- are generalized, often symmetric, and also following initiation of treatment vens Johnson syndrome/toxic epider- may involve the mucous membranes. for an SLE flare. Diagnostic criteria mal necrolysis, epidermolysis bullosa Commonly affected areas include the for BSLE include the presence of ac- acquisita, chronic bullous of skin over the joints, sun-exposed re- quired, non-scarring vesicles or bul- childhood (CBDB), bullous pemphi- gions, the trunk, and the vermilion lae, meeting the American College of goid, and vulgaris. These border. Lesions typically rupture and Rheumatology criteria for SLE, and diagnoses are made based on clinical then crust over, eventually healing as characteristic histopathology and im- presentation and skin biopsy results. non-scarred hyper- or hypo-pigment- munofluorescence testing. The skin Bullous SLE is often responsive

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to dapsone, which may be due to this followed by furosemide. She required wound care was performed daily un- ’s ability to impair neutro- a transfusion of packed red blood cells der the guidance of an advance prac- phils and prostaglandins. Additional and a peripherally inserted central tice wound care nurse. treatment options include immune catheter to maintain access. Due to Following an 8-week hospitalization, modulators such as colchicine, aza- the necessity for optimal sta- the patient demonstrated significant heal- thioprine, antimalarials, cyclophospha- tus for wound healing, nutrition was ing of her skin and a decrease in the de- mide, , and mycopheno- supported with nasogastric tube feeds. velopment of new bullous lesions. A slow late mofetil. The skin lesions of BSLE Her pain was managed with , steroid taper was begun, and she was dis- may be unresponsive to steroids, but gabapentin, and a low-dose ketamine charged home on mycophenolate mofetil, steroids are still used when other sys- , and dapsone. temic manifestations of SLE are pres- ent. Treatment of BSLE should be guid- ... bullous systemic lupus CONCLUSION ed by a rheumatologist. Although extremely rare in children, During our patient’s hospital erythematosus should be bullous systemic lupus erythematosus course, the bullae enlarged and then considered in the differential should be considered in the differential ruptured, leaving tender, denuded ar- diagnosis for any child with vesiculobul- eas that quickly epithelialized. They diagnosis for any child with lous disease, and appropriate serologic healed without scarring but with re- ... testing for autoimmune markers and skin sidual hypopigmentation. The patient biopsy should be considered in these pa- continued to develop new bullous le- tients. sions that involved her extremities, drip guided by pain management con- palms, and soles. She also developed sultants. were treated empiri- REFERENCES signs of , including protein- cally with clindamycin for coverage 1. Boguniewics M, Leung DYM. Adverse reac- tions to drugs. In Kliegman: Nelson Text- uria, , and . of skin flora, including possible meth- book of Pediatrics, 18th Ed. Philadelphia, As a result, she was started on a regi- icillin-resistant Staphylococcus aure- PA: Saunders; 2007. men of high-dose methylpredniso- us (MRSA). Blood, urine, and wound 2. Fujimoto W, Hamada T, Yamada J, Matsuura lone, mycophenolate mofetil, and hy- cultures failed to show a source of in- H, Iwatsuki K. Bullous systemic lupus ery- thematosus as an initial manifestation of droxychloroquine in order to suppress fection. She ultimately was treated for SLE. J Dermatol. 2005;32:1021-1027. her lupus activity. Despite these im- that developed on her foot 3. Gellis SE. Bullous of childhood. munosuppressives, she continued to near the site of several of her lesions. Dermatol Clin. 1986;4(1):89-98. 4. Habif TP. Vesicular and bullous diseases. In develop new bullae over a period of The most challenging aspect of her Clinical , 5th Ed. China: Mosby; many weeks, requiring a prolonged care was wound management, as there 2010. hospital stay and treatment with two were limitations in mobility and po- 5. Harris-Stith R, Erickson QL, Elston DM, David-Bajar K. Bullous eruption: a mani- doses of intravenous immunoglobulin sitioning given the extensive nature festation of lupus erythematosus. Cutis. (IVIG), dapsone, and . of her bullae. In general, small bullae 2003;72:31-37. The patient’s hospital course was were left intact to allow a natural ban- 6. Morelli JG. Vesiculobullous disorders. In complicated by fever, , and dage and sterile environment. These Kliegman: Nelson Textbook of Pediatrics, 18th Ed. Philadelphia, PA: Saunders; 2007. tachycardia. Her care required a mul- bullae eventually ruptured spontane- 7. Mutasim DF, Bilic M, Hawayek LH, Pipitone tidisciplinary approach including flu- ously, healed within 1 to 2 days, and MA, Sluzevich JC. Immunobullous diseases. id management, nutritional support, left hypopigmented skin behind. Larg- J Am Acad Dermatol. 2005;52:1029-1043. 8. Pilcher MF, Metzinger AR, Davis LS. Gen- pain control, wound care, and immu- er bullae were ruptured manually due eralized bullour eruption in a teenager. Ped nosuppressive therapy. The patient’s to pain and interference with position- Derm 2010;27:197-198. edema and tachycardia were managed ing and ambulation. Healing patterns 9. Vassileva J. Bullous systemic lupus erythe- matosus. Clin Dermatol. 2004;22:129-138. with multiple infusions of albumin were similar for these bullae. Her

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