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 Ayelet Shani-Adir, MD Anthony J. Mancini, MD |
Dermatology quiz Ayelet Shani-Adir, MD aSpring 2001  FIGURE 1 Appearance of scalp lesion at 3 months. A 14-MONTH-OLD WHITE MALE was referred to the dermatology clinic for evaluation of a pruritic acral rash. His mother said the rash had begun 8 months earlier with crops of pustules on his hands and feet which would last a few weeks and then disappear, only to recur. He was treated empirically on 2 occasions with permethrin 5% cream, but the rash continued to occur intermittently at 3–4 week intervals. Medical history was unremarkable and there was a positive family history for atopy. The patient had no known exposure to any infectious diseases. Physical examination revealed a well appearing infant male with multiple 1- to 2-mm vesiculopustules over the palms (Figure 1), soles (Figure 2), lateral surfaces of hands and feet, and wrists and ankles. The remainder of the physical examination was unremarkable. His oral mucosa was clear, and there was no lymphadenopathy or hepatosplenomegaly.  The most appropriate next step is to: Considering the test you chose was negative, the most likely
diagnosis is: The most appropriate therapy is:
A. A course of oral antibiotics. Answers: D, B, and C respectively. The patient presents with classic infantile acropustulosis (IA), or acropustulosis of infancy. This disease was initially described in 1979 by Kahn and Rywlin,1 and is characterized by recurrent crops of intensely pruritic vesiculopustules on the distal extremities. The initial cutaneous lesions can be erythematous papules which develop within 24 hours into vesicles and then into pustules, with no tendency to coalesce. The hands and feet are usually involved with lesions located on palms and soles and on lateral surfaces, and occasionally over the dorsal surfaces.2 IA is more common in males and in blacks and tends to be worse in summer than winter. Onset usually occurs between the ages of 6 to 12 months, but cases have been described at birth and can present later in childhood.3 Lesions typically last 2–4 weeks and then recur every few weeks to months, with eventual spontaneous resolution by 2 to 3 years of age. The etiology of IA is unknown. Several authors have noted that scabies infestation frequently precedes IA, suggesting a possible relationship between the two diseases.4–6 Additionally, the clinical presentation of scabies in children can often manifest as vesiculopustular lesions on hands and feet. However, the predominance of lesions on acral sites, the absence of other affected family members, and the lack of evidence of infestation by microscopic examination help to distinguish IA from scabies. Many patients who are diagnosed with IA have received antiscabitic treatment prior to the diagnosis, but the lesions persist or recur despite therapy. The true association between IA and scabies is difficult to ascertain, the difficulty compounded by the commonplace practice of treating patients for scabies without performing mineral oil examination to confirm infestation. In a recent study, 14 of 21 patients diagnosed with IA had a history of prior treatment for scabies, although only 2 had microscopic confirmation of mites, feces or ova.6 In any patient with a suspected diagnosis of IA, a mineral oil examination should be performed to exclude scabies infestation. The differential diagnosis of IA includes scabies, impetigo, cutaneous candidiasis, transient neonatal pustular melanosis, and pustular psoriasis. The diagnosis is rendered based on the clinical picture of recurrent attacks of pruritic, sterile palmar and plantar pustules and negative mineral oil examination for scabies. Lack of response to antiscabitic therapy is often confirmatory, as well. Histopathologic examination of lesions of infantile acropustulosis reveals large well-circumscribed subcorneal or intraepidermal pustules filled with polymorphonuclear leukocytes.7 The pustules may also contain eosinophils and pyknotic nuclear debris. The dermis shows papillary edema and a superficial perivascular infiltrate of mononuclear cells and a few eosinophils. Gram’s and potassium hydroxide (KOH) preparations reveal numerous polymorphonuclear leukocytes but no bacteria or yeast. Treatment for IA includes potent topical corticosteroids and antihistamines. Intermittent use of topical steroids can be used safely for flares, and the localization and distribution of involvement should alleviate concerns about toxicity of these preparations. In severe cases of infantile acropustulosis, dapsone 2 mg/kg/day has been used, although in most patients, the risk to benefit ratio does not justify its use for this disorder.1,8 While infantile acropustulosis is a benign, self-limited disorder, it can result in significant discomfort and irritability for the affected child. Adequate recognition, diagnosis and therapy are therefore vital. REFERENCES 1. Kahn G, Rywlin AM. Acropustulosis of infancy. Arch Dermatol. 1979;115:831–833. 2. Fine J, Resnick SD. Vesiculobullous and neonatal diseases. In: Schachner LA, Hansen RC, eds. Pediatric Dermatology. 2nd ed. New York, NY: Churchill Livingstone; 1995. 3. Hurwitz S. Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence. Philadelphia, Pa: WB Saunders; 1993. 4. Prendiville JS. Infantile acropustulosis—How often is it a sequela of scabies? Pediatr Dermatol. 1995;12:275–276. 5. Elpern DJ. Infantile acropustulosis and antecedent scabies. J Am Acad Dermatol. 1984;11:895. 6. Mancini AJ, Frieden IJ, Paller AS. Infantile acropustulosis revisited: history of scabies and response to topical corticosteroids. Pediatr Dermatol. 1998;15:337–341. 7. Vignon-Pennamen MD, Wallach D. Infantile acropustulosis. A clinicopathologic study of six cases. Arch Dermatol. 1986;122;1155–1160. 8. Findlay RF, Odom RB. Infantile acropustulosis. Am J Dis Child. 1983;137:455–457. |