|Year : 2016 | Volume
| Issue : 2 | Page : 112-113
Congenital self-healing reticulohistiocytosis in a newborn: A rare variant
Shailvi Banka1, Aparesh Patra1, Sandip Gupta2, SK Masud Hassan1
1 Department of Dermatology, Bankura Medical College and Hospital, Bankura, West Bengal, India
2 Department of Paediatrics, Bankura Medical College and Hospital, Bankura, West Bengal, India
|Date of Web Publication||30-Mar-2016|
Department of Dermatology, Bankura Medical College and Hospital, Bankura - 722 102, West Bengal
Source of Support: None, Conflict of Interest: None
Congenital self-healing reticulohistiocytosis or congenital self-healing Langerhans cell histiocytosis (LCH) or Hashimoto-Pritzker syndrome is a rare, benign, auto involuting form of LCH. It usually presents at birth or soon thereafter with spontaneously regressing multiple or solitary dusky papules or nodules. The actual incidence remains unknown probably because of its benign and self-limiting nature. Herein, we present a similar case present since birth because of its rarity in nature.
Keywords: Congenital, Hashimoto-Pritzker syndrome, reticulohistiocytosis
|How to cite this article:|
Banka S, Patra A, Gupta S, Masud Hassan S K. Congenital self-healing reticulohistiocytosis in a newborn: A rare variant. Indian J Paediatr Dermatol 2016;17:112-3
|How to cite this URL:|
Banka S, Patra A, Gupta S, Masud Hassan S K. Congenital self-healing reticulohistiocytosis in a newborn: A rare variant. Indian J Paediatr Dermatol [serial online] 2016 [cited 2020 Sep 20];17:112-3. Available from: http://www.ijpd.in/text.asp?2016/17/2/112/179413
| Introduction|| |
Langerhans cell histiocytosis (LCH) is a clonal disorder that is characterized by proliferation of Langerhans cell in various organs of the body. The annual incidence rate is 0.5–5.4 million children per year. Congenital self-healing reticulohistiocytosis (CSHR) is considered to be at one end of the spectrum of LCH. Although more than a 100 cases of LCH has been reported, the innocuous and unaggressive nature of CSHR differentiates it from the other variants of LCH. Unlike the other types of LCH, it is generally limited to the skin and is present since birth or shortly thereafter. We report a case of CSHR, who presented with multiple lesions over the whole body since birth.
| Case Report|| |
A 7-day-old male baby born out of nonconsanguineous marriage presented with multiple dusky, tan brown and hemorrhagic papules since birth. The baby was born at term after an uncomplicated vaginal delivery. On physical examination, multiple tan brown papules and nodules of size 0.1–1 cm were present on the whole body including the palm and sole [Figure 1]a,[Figure 1]b,[Figure 1]c. Mucous membrane was uninvolved. The general condition of the child was otherwise healthy. Histopathological examination of the skin lesion revealed dense histiocytic infiltrate involving the whole dermis. The cells have a distinct kidney-shaped nucleus, with abundant eosinophilic ground glass cytoplasm [Figure 2]a and [Figure 2]b. A provisional diagnosis of Hashimoto-Pritzker syndrome was made based on clinical and histopathological finding. Immunohistochemistry could not be done because of financial constraints. The child was kept on follow-up only on emollient and 6 months later, the lesions had subsided leaving only postinflammatory pigmentation.
|Figure 1: (a-c) Multiple dusky papules and nodules on whole body including face, palms, and soles |
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|Figure 2: (a) Dense infitration of histiocytes throughout the dermis. (b) High power view showing the kidney-shaped nucleus of histiocytes|
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| Discussion|| |
LCH is a benign clonal heterogeneous disorder involving the proliferation of Langerhans cell that are positive for immunohistochemical marker CD1a and S-100. They show the presence of birbeck granules on electron microscopy.
CSHR was first described by Hashimoto and Pritzker. It is a rare benign single-system LCH. The actual incidence, however, remains underreported because of its self-limiting nature. It has been described in two forms: Solitary and multinodular. Both forms can affect any part of the cutaneous surface. The lesion consists of aymptomatic papules, nodules, and vesicles with or without ulceration which are red, brown, pink, or dusky in color. Spontaneous resolution is the rule with absence of any systemic involvement. These two characteristics differentiate CSHR from other variants of LCH.
Histologically, skin lesion consists of Langerhans cell and no histological variant identifies this type of LCH.
CSRH although runs a benign course, there have been reports of pulmonary and ocular involvement that resolved along with skin lesion., There also has been reporting of CSRH presenting as a blueberry muffin lesion rising concern of it being a congenital infection. The exact nature of this disease remains debatable.
CSRH has an excellent survival rate with no deaths reported so far. The skin lesion subsides spontaneously in about 1–3 month although in recalcitrant cases, topical steroid, calcineurin inhibitor, and nitrogen mustard are advocated. The lesion heals with either hyperpigmentation, hypopigmentation or atrophic scar. CSRH should be differentiated from other variant of LCH and also from other histiocytic lesion such as juvenile xanthogranuloma. The treatment of LCH is “wait and watch” policy as the lesions are self-limiting. A long follow-up is, however, necessary due to the risk of multisystem involvement and recurrence of the lesion.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial Support and Sponsorship
Conflicts of Interest
There are no conflicts of interest.
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