Solitary Congenital Self-Healing Histiocytosis (Hashimoto-Pritzker Disease) Histiocitosis congénita autolimitada (Hashimoto-Pritzker) de presentación solitaria

Congenital self-healing Langerhans cell histiocytosis (CSHLCH), or Hashimoto-Pritzker disease, is a rare variant of Langerhans cell histiocytosis that was first described in 1973 by Hashimoto and Pritzker.1-5 It presents at birth or in the neonatal period.1-9 Few case series have been published in the literature.1,4,5 However, it is believed that the actual incidence is higher and that many cases go unreported due to spontaneous resolution or the lack of clinical recognition. We describe the case of a 2-month-old girl who presented a single lesion on the left thigh from birth. The lesion consisted of a desquamating indurated violaceous nodule with central ulceration covered by a serosanguineous scab (Figure 1). The biopsy showed a dermal and hypodermal proliferation of histiocytic cells with oval vesicular nuclei and abundant eosinophilic cytoplasm, accompanied by multinucleated giant cells (Figure 2). The histiocytic cells were positive for S-100, CD1a, and langerin (Figure 3). The laboratory workup and imaging tests ruled out systemic involvement, and the patient was diagnosed with CSHLCH. The lesion involuted spontaneously, and after 16 months of follow-up the patient is free of disease. Langerhans cell histiocytosis (LCH) refers to a group of disorders characterized by cell proliferation in various organs; the cells are positive for S-100 and CD1a and contain Birbeck granules in the cytoplasm.1,2,4,5 In 1987 the Writing Group of the Histiocyte Society grouped 4 conditions together under the heading of LCH, according to clinical presentation: an acute, disseminated form (Letterer-Siwe disease), a multifocal chronic and progressive form (HandSchüller-Christian disease), a chronic localized form or eosinophilic granuloma, and a congenital form (HashimotoPritzker disease). In 1997 the group published another classification in which they recommended that only the term LCH be used and that patient grading be based on the extension of the disease.9,10 CSHLCH is a rare disease characterized by painless reddish-brown papules or nodules that are present at birth or in the early months of life and involute within a few weeks or months.1-6 The lesions tend to be multiple and are most commonly distributed on the trunk, face, and scalp.4,5 Solitary lesions, first described by Berger et al. in 1986, are rare; since that initial description, only around 30 cases (25% of all reports) have been published.1,3,6 Lesions are rarely found in the oral mucosa or internal organs, although a few cases with systemic or mucosal involvement have been described, but these lesions regress along with the cutaneous one.1,5,7,9 Histologically, the lesions are characterized by a superficial and deep dermal infiltrate composed of histiocytic cells with kidney-shaped nuclei and abundant eosinophilic cytoplasm. Inflammatory cells such as lymphocytes or eosinophils and multinucleated giant cells can also be observed.2,4,5 2. O’Malley MP, Haake A, Goldsmith L, Berg D. Localized Darier disease. Implications for genetic studies. Arch Dermatol. 1997;133:1134-8. 3. Sakuntabhai A, Dhitavat J, Burge S, Hovnanian A. Mosaicism for ATP2A2 mutations causes segmental Darier’s disease. J Invest Dermatol. 2000;115:1144-7. 4. Mazereeuw-Hautier J, Thibaut I, Bonafé JL. Acantholytic dyskeratotic epidermal nevus: a rare histopathologic feature. J Cutan Pathol. 2002;29:52-4. 5. Martínez S, Vera A, Eloy-García C, Sanz A, Crespo V. Linear Darier disease. Actas Dermosifiliogr. 2006;97:139-41. 6. Gilaberte M, Puig L, Vidal D, Alomar A. Acantholytic dyskeratotic naevi following Blaschko’s lines: a mosaic form of Darier’s disease. J Eur Acad Dermatol Venereol. 2003;17:196-9. I. de la Hera,* R. Chico, R. Llamas, F. Vanaclocha