J AM ACAD DERMATOL

VOLUME 62, NUMBER 5

Letters 895

Chronic bullous disease of childhood and pneumonia in a neonate with VATERL association and hypoplastic paranasal sinuses To the Editor: A 1-day-old Filipino girl was admitted with multiple congenital anomalies consisting of hypoplastic paranasal sinuses and VATERL association, a spectrum of birth defects which may include vertebral anomalies, anal atresia, tracheoesophageal fistula, renal defects, and limb abnormalities (OMIM # 192350). She was treated with intravenous antibiotics for suspected sepsis, including amikacin, ampicillin, metronidazole, and piperacillin-tazobactam. Fresh-frozen plasma transfusions were administered for the correction of prolonged prothrombin and partial thromboplastin times, and she received furosemide after the transfusions. On the tenth day of life, she developed multiple erythematous papules in the diaper area which evolved into vesicles and tense bullae. Similar lesions appeared on the scalp, around the mouth, and on the trunk and extremities (Fig 1). A diagnosis of chronic bullous disease of childhood (CBDC) was confirmed by histopathology and direct immunofluorescence (DIF) which showed a strong linear deposition of IgA and less intense deposition of IgG and C3 along the basement membrane zone (BMZ). The patient was treated with oral prednisone 0.5 mg/kg/day (1.0 mg daily) tapered over the course of 1 month. Other medications, including antibiotics and furosemide, were discontinued. There was healing of all bullae within 2 weeks of starting prednisone. No new lesions were observed thereafter except for a 2-mm erosion on the tip of the tongue (Fig 2). When the dose of prednisone had been tapered to 0.15 mg/kg/day, she developed pneumonia which resolved with antibiotic therapy. However, further episodes of respiratory distress led to her death 2 weeks later. To our knowledge, there are only three other reported cases of neonatal CBDC.1-3 All three infants had severe mucosal involvement complicated by respiratory failure. The first two cases had permanent sequelae; these included blindness from conjunctival scarring and dysphagia from pharyngeal scarring and sublingual ranulae, respectively. The second case had spontaneous resolution of skin lesions, but the oral mucosal involvement progressed, and the patient developed respiratory distress and pneumonia. All three infants survived the neonatal period with ventilatory support and the administration of systemic corticosteroids and/or sulfones. On reviewing the presentations of these three previously reported cases, it appears that significant mucosal involvement predisposing to

Fig 1. Neonatal chronic bullous disease of childhood, with tense blisters on the face, trunk, and extremities.

Fig 2. Erosion on the tip of the tongue in a neonate with chronic bullous disease of childhood.

respiratory difficulty and pneumonia may be a feature of neonatal CBDC. The blistering process could predispose to secondary infection, and the aspiration of material from sloughed blisters might increase the risk of pneumonia. Clinicians should have a high index of suspicion for respiratory involvement in neonates with CBDC. This patient had multiple congenital anomalies that may have contributed to the respiratory complications. The VATERL association may result in neonatal respiratory distress when tracheal or esophageal anomalies are present. Our patient did not have these anomalies. Hypoplastic paranasal sinuses and bilateral choanal atresia associated with hypogammaglobulinemia are reported in an adult with a history of repeated chest infections and pneumonia.4 An evaluation for immunodeficiency in our patient was not completed before she died. A drug-induced variant of CBDC was considered because of the infants exposure to various medications, including ampicillin and furosemide, before the appearance of the blisters.5,6 Consistent with other cases of drug-induced CBDC, our patients skin lesions responded immediately to treatment with oral prednisone. However, we found a deposition of multiple immunoreactants along the BMZ on DIF studies, whereas drug-induced CBDC often shows IgA alone.5,6

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J AM ACAD DERMATOL
MAY 2010

Charissa Mia D. Salud, MD,a and Marie Eleanore O. Nicolas, MDa,b Pediatric Dermatology Unit & Immunodermatology Unit,b Section of Dermatology,a University of the Philippines, Philippine General Hospital, Manila, Philippines Funding sources: None. Conicts of interest: None declared. Reprint requests: Marie Eleanore O. Nicolas, MD, FPDS, Pediatric Dermatology Unit & Immunodermatology Unit, Section of Dermatology, University of the Philippines, Philippine General Hospital, Room 101, Out-Patient Department, Philippine General Hospital, Padre Faura St, Manila 1000, Philippines E-mail: marielnicolas@hotmail.com
REFERENCES 1. Hruza LL, Mallory SB, Fitzgibbons J, Mallory GB Jr. Linear IgA bullous dermatosis in a neonate. Pediatr Dermatol 1993;10:171-6. 2. Kishida Y, Kameyama J, Nei M, Hashimoto T, Baba K. Linear IgA bullous dermatosis of neonatal onset: case report and review of the literature. Acta Paediatr 2004;93:850-2. 3. Lee SY, Leung CY, Leung CW, Chow CB, Leung KM, Lee QU. Linear IgA bullous dermatosis in a neonate. Arch Dis Child Fetal Neonatal Ed 2004;89:F280. 4. El-Sawy H, Siddiq M, Anbarasu A. Bilateral choanal atresia and paranasal sinus hypoplasia in an adult with hypogammaglobulinemia. Eur Arch Otorhinolaryngol 2006;263:1136-8. 5. Ho J, Ng P, Hoon TS. A retrospective study of adult linear IgA disease seen at the National Skin Centre. Dermatol Bull 2005;16:11-4. 6. Plunkett RW, Chiarello SE, Beutner EH. Linear IgA bullous dermatosis in one of two piroxicam-induced eruptions: a distinct direct immunofluorescence trend revealed by the literature. J Am Acad Dermatol 2001;45:691-6. doi:10.1016/j.jaad.2009.02.009

Fig 1. Shiny pink-yellow plaque in an infraabdominal skin fold with a small amount of vesiculation at the lateral margin of the plaque.

Fig 2. Basophilic laminated inclusion bodies (Michaelise Gutmann bodies). (Hematoxylineeosin stain; original magnication: 3400.)

Cutaneous malakoplakia in an abdominal skin fold To the Editor: Malakoplakia is a rare chronic inflammatory disease that is found primarily in the genitourinary tract. Cutaneous involvement is even rarer, and the lesions can be nonspecific, varying from papules to frank ulcers. The vulva and perianal region are usually involved, but other sites have been described.1,2 Lesions on the abdominal wall have been reported to occur within surgical scars or drainage sites after removal of an internal organ affected by malakoplakia.2 There is also often underlying immunosuppression from one cause or another. We report a case of cutaneous malakoplakia in an infraabdominal skin fold in a previously

healthy 60-year-old female with no history of immunosuppression or surgery. A 60-year-old female presented with a 2-year history of a rash in an infraabdominal skin fold, which had initially been itchy. The patient also suffered with mild hypertension and osteoarthritis for which she was taking indapamide and telmisartan. The rash had been treated 6 months before the consultation as a blistering intertrigo with 1% clotrimazole cream with moderate improvement. Examination revealed two discrete pink-yellow, waxy plaques in an infraabdominal skin fold (Fig 1). A small vesicle was present in the most lateral plaque. No vulval or inguinal lesions were seen. A histologic examination showed a loosely aggregated inltrate of histiocytes in the papillary and mid dermis with small numbers of admixed lymphocytes. No multinucleate giant cells were present. The histiocytes contained red blood cell fragments and rounded periodic acideSchiff (PAS)-positive calcied bodies, characteristic of MichaeliseGutman bodies (Fig 2). Von Kossa stain for calcium was positive (Fig 3), but Perl stain for ferric iron was