Letterer siwe disease

Introduction

Langerhan cell histiocytosis (LCH) is the name proposed by Risdall et al,[1] in 1983, for a group of disorders previously called Histiocytosis X, where Langerhan cells play a central role in the pathogenesis. Letterer Siwe (LS) disease is an acute, disseminated, multisystem, often fatal type of LCH. The other types are Hand Schuller Christian (HSC) disease which is chronic and disseminated; eosinophilic granuloma which is chronic, benign and solitary and the mixed type.[2]

Case Report

A 1-year-old boy was admitted with history of recurrent pyoderma-like lesions especially over the scalp, face, palms and soles with purulent discharge from both ears since the age of 4 months. There was history of high fever with cough and dyspnoea at the age of 7 months, but there was never polyuria or polydypsia.

He was of moderate build and nourishment, was toxic, febrile and anaemic with pedal oedema. There was erythema, fissuring, scaling and crusting with vesicles and pustules over scalp; face especially over the forehead, periocular and retroauricuar areas [Figure - 1]; palms; soles; neck; groin folds and trunk. The palms and soles in addition had extensive haemorrhagic spots [Figure - 2] and there was a congenital haemangioma on the right palm. Multiple post inflammatory hypopigmented scars were present on the trunk; the teeth and nails were normal. There was also generalised lymphadenopathy, alopecia, blepharoc-onjunctivitis, oral candidiasis and bilateral purulent foul smelling ear discharge with a CSOM on the left side. There was gross hepatosplenomegaly but other systems were within normal limits.

He was born as the second child of a non-consanguinous marriage, was fully immunised for age and the developmental milestones were normal. The family history was noncontributory.

With a clinical diagnosis of Histiocytosis-X, the following investigations were done: Urine-normal; blood-Hb: 6gms%,TC: 7200/cmm, P-71 L-25 E-4, ESR: 104mm/first hour; Blood picture-normocytic hypochromic anaemia, platelets scattered and reduced in number. Gram stain of smear from intact pustule-sterile; culture of pus showed contaminant growth of Staph aureus. Elisa for HIV-negative, VDRL-nonreactive. X-rays of chest (PA) and skull (lateral) were normal. Skin biopsy showed extensive infiltration of dermis by histiocytes mainly in the upper zone and invading into the epidermis. A few atypical histiocytes with hyperchromatic nuclei and extravasated erythrocytes were also seen in the infiltrate.

The child was started on antipyretics, systemic antibiotics and topical agents. He developed acute abdominal distension and expired within a few days.

Discussion

In Letterer Siwe disease recurrent pyoderma -like lesions with crusting and scaling, vesicopustular and purpuric eruption occurs in crops over the face, scalpfand trunk, resembling seborrhoeic dermatitis. Nodular lesions in body flexures and intraoral haemorrhage, gingivitis, loose teeth, precocious eruption of teeth and ectopic teeth also may be seen.[3] The pustules are sterile and Tzanck smear of the vesicopustules shows pale histiocytes which can be used as a rapid screening test. Skin biopsy in LS disease shows a proliferative reaction with extensive upper dermal infiltration and epidermal invasion with histiocytes, some of which are atypical, along with erythrocytes.

This case had typical skin lesions and the biopsy confirmed LS disease. He also had recurrent otitis media which is the most frequent clinical symptom of HSC disease, thus suggesting that there can be an overlap of the two diseases, being manifestations of the same disease process. However, there was no polyuria or polydypsia characteristic of ′ HSC disease. Oral candidiasis may have been due to immune deficiency due to the repeated and long term therapy with antibiotics and steroids.

Chemotherapy coupled with radiation to localised bony lesions and supportive measures are useful in treatment, there being no unified therapeutic measure.[4] Thymic extracts have been successfully used to treat LS disease.[5]

Poor prognostic features include younger age; dissemination of lesions; involvement of lungs, liver, CNS and RE system; associated infection; anaemia, thrombocytopenia and purpura.[6] The child had extensive purpuric lesions on palms and soles which is a reputedly lethal sign,[6] as well as hepatosplenomegaly, thrombocytopenia and severe anaemia. He died within a few days proving again that the presence of purpura in LS disease is a lethal marker.