LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY

A 4½-month-old female patient presenting with an eythematopurpuric eruption underwent a skin biopsy for histopathology and was first diagnosed with isolated cutaneous Langerhans cell histiocytosis. Her lesions regressed within a few months and she was retrospectively diagnosed with late-onset self-healing Langerhans cell histiocytosis after being without skin or systemic involvement in a follow-up four years later.

Comments:

Self-healing Langerhans cell histiocytosis, which is characterized by clonal proliferation of Langerhans cells and presents with cutaneous lesions, is a rare self-limited variant of histiocytosis and can only be diagnosed retrospectively, after the patient remains free from systemic involvement for several years. Although it presents at birth or during the neonatal period, only a few cases of its late-onset type regarding the age of onset have been reported. Purpuric lesions that appear after the neonatal period serve as a clue for late-onset self-healing Langerhans cell histiocytosis and the patients should be monitored regularly for systemic involvement if the diagnosis is confirmed by a cutaneous biopsy.

Keywords:
Infant; Histiocytosis, Langerhans-cell; Self-healing

Reference	Number of cases	Skin involvement	Age at onset
Nakahigashi et al.¹¹11. Nakahigashi K, Ohta M, Sakai R, Sugimoto Y, Ikoma Y, Horiguchi Y. Late-onset self-healing reticulohistiocytosis: pediatric case of Hashimoto-Pritzker type Langerhans cell histiocytosis. J Dermatol. 2007;34:205-9.	1	Multiple	8 years
Belhadjali et al.¹³13. Belhadjali H, Mohamed M, Mahmoudi H, Youssef M, Moussa A, Chouchane S, et al. Self-healing Langerhans cell histiocytosis (Hashimoto-Pritzker disease): two Tunisian cases. Acta Dermatovenereol Alp Pannonica Adriat. 2008;17:188-92.	1	Multiple	20 days
Hashimoto et al.¹⁴14. Hashimoto K, Griffin D, Kohsbaki M. Self-healing reticulohistiocytosis: a clinical, histologic, and ultrastructural study of the fourth case in the literature. Cancer. 1982;49:331-7.	1	Multiple	17 days
Campourcy et al.¹⁵15. Campourcy M, Moreau-Cabarrot A, Gorguet B, Samalens G, Daste B, Eclache F, et al. Acquired regressive cutaneous non-Langerhans-cell histiocytosis in an infant. Ann Dermatol Venereol. 1997;124:167-70.	1	Multiple	15 months
Jang et al.¹⁶16. Jang KA, Ahn SJ, Choi JH, Sung KJ, Moon KC, Koh JK. Histiocytic disorders with spontaneous regression in infancy. Pediatr Dermatol. 2000;17:364-8.	3	Multiple	1, 2, 7 months
Murata et al.¹⁷17. Murata S, Yoshida Y, Adachi K, Morita E, Yamamoto O. Solitary, late-onset, self-healing Langerhans cell histiocytosis. Acta Derm Venereol. 2011;91:103-4.	1	Solitary	4 months
Nakahara et al.¹⁸18. Nakahara T, Kido-Nakahara M, Itoh E, Furue M. Late-onset self-healing Langerhans cell histiocytosis in a patient with atopic dermatitis. J Dermatol. 2014;41:450-1.	1	Multiple	10 months
Current patient	1	Multiple	3 months

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[1] *Corresponding author. E-mail: suleafsar@hotmail.com (F.S. Afsar).